Philadelphia chromosome-negative myeloproliferative neoplasms (Ph- MPNs) are characterized by clonal myeloproliferation and somatic mutations. Major complications of Ph-MPNs are thrombosis, bleeding, transformation to myelofibrosis and leukemia. One important concern in the course of Ph-MPNs is risk of development of secondary solid cancers (SSC). In a large cohort of Turkish Ph-MPN patients, we aimed to determine the types and frequencies of SSC, to identify risk factors for SSC including role of cytoreductive therapies and to study impact of SSC on survival in Ph-MPNs.
Methodology
1013 patients diagnosed with Ph-MPN from 1995 and 2022 under follow up at adult hematology sections of Istanbul Bakırköy Dr Sadi Konuk Hospital and Istanbul University Medical Faculty were included in this retrospective study.
Results
Of the 1013 Ph- MPN patients enrolled in our study, 65, 46 and 37 patients were diagnosed with essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (PMF), respectively. Patient clinical and laboratory characteristics are summarized in Table 1. Sixty-seven patients (6.6%) developed SSC, predominantly carcinoma (64.2%), non-melanoma skin cancer (23.9%), sarcoma (4.5%), and melanoma (3%). Median time to SSC diagnosis was 80.03 ± 60.5 months with no significant difference among Ph- MPN subtypes. Compared to patients with no diagnosis of SSC, patients with SSC were older at time of Ph- MPN diagnosis (63 vs. 54 years; p<0.001) and included a higher proportion of males (p=0.025). Ph- MPN patients with SSC and without SSC showed no significant difference for complete blood count parameters, spleen size, Ph-MPN diagnosis groups, driver mutation frequencies and follow-up time. Arterial thrombosis frequency was higher in patients with SSC (37.3% vs. 25.3%; p=0.030). SSC rates were 5.7% in patients not exposed to cytoreductive treatment and 5.3%, 4% and 2.1% with exposure to ruxolitinib, anagrelide, and interferon (IFN), respectively. A trend toward lower SSC rates was noted with IFN therapy (3% vs. 97%; p=0.066). SSC incidence was significantly higher in patients exposed to hydroxyurea (HU) as first-line monotherapy compared to other treatment groups (7.8% vs. 4.6%; p=0.046). Median OS in patients with SSC and patients with no diagnosis of SSC group were 273 months and 195 months, respectively. PV patients, who developed SSC, had significantly worse median OS compared to PV patients without SSC (Figure-1).
Conclusion
The strengths of our study are that it enrolls a larger patient population, includes PV, ET and PMF subgroups, separately examines development of SSC after MPN, has a long follow-up period and has multicenter design. In MPN patients, malignancy screening gains more importance for those aged ≥65 and males. Our study evaluated with data from previous studies suggest that increased risk of developing SSC in M
{"title":"SECONDARY SOLID CANCER FREQUENCY AND RISK FACTORS IN PHILADELPHIA- NEGATIVE CHRONIC MYELOPROLIFERATIVE NEOPLASMS","authors":"Fehmi Hindilerden , Özge Nuran Akay , Elif Aksoy , Aynur Daglar Aday , Emine Gültürk , Meliha Nalçacı , İpek Yönal Hindilerden","doi":"10.1016/j.htct.2024.04.005","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.005","url":null,"abstract":"<div><h3>Objective</h3><p>Philadelphia chromosome-negative myeloproliferative neoplasms (Ph- MPNs) are characterized by clonal myeloproliferation and somatic mutations. Major complications of Ph-MPNs are thrombosis, bleeding, transformation to myelofibrosis and leukemia. One important concern in the course of Ph-MPNs is risk of development of secondary solid cancers (SSC). In a large cohort of Turkish Ph-MPN patients, we aimed to determine the types and frequencies of SSC, to identify risk factors for SSC including role of cytoreductive therapies and to study impact of SSC on survival in Ph-MPNs.</p></div><div><h3>Methodology</h3><p>1013 patients diagnosed with Ph-MPN from 1995 and 2022 under follow up at adult hematology sections of Istanbul Bakırköy Dr Sadi Konuk Hospital and Istanbul University Medical Faculty were included in this retrospective study.</p></div><div><h3>Results</h3><p>Of the 1013 Ph- MPN patients enrolled in our study, 65, 46 and 37 patients were diagnosed with essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (PMF), respectively. Patient clinical and laboratory characteristics are summarized in Table 1. Sixty-seven patients (6.6%) developed SSC, predominantly carcinoma (64.2%), non-melanoma skin cancer (23.9%), sarcoma (4.5%), and melanoma (3%). Median time to SSC diagnosis was 80.03 ± 60.5 months with no significant difference among Ph- MPN subtypes. Compared to patients with no diagnosis of SSC, patients with SSC were older at time of Ph- MPN diagnosis (63 vs. 54 years; p<0.001) and included a higher proportion of males (p=0.025). Ph- MPN patients with SSC and without SSC showed no significant difference for complete blood count parameters, spleen size, Ph-MPN diagnosis groups, driver mutation frequencies and follow-up time. Arterial thrombosis frequency was higher in patients with SSC (37.3% vs. 25.3%; p=0.030). SSC rates were 5.7% in patients not exposed to cytoreductive treatment and 5.3%, 4% and 2.1% with exposure to ruxolitinib, anagrelide, and interferon (IFN), respectively. A trend toward lower SSC rates was noted with IFN therapy (3% vs. 97%; p=0.066). SSC incidence was significantly higher in patients exposed to hydroxyurea (HU) as first-line monotherapy compared to other treatment groups (7.8% vs. 4.6%; p=0.046). Median OS in patients with SSC and patients with no diagnosis of SSC group were 273 months and 195 months, respectively. PV patients, who developed SSC, had significantly worse median OS compared to PV patients without SSC (Figure-1).</p></div><div><h3>Conclusion</h3><p>The strengths of our study are that it enrolls a larger patient population, includes PV, ET and PMF subgroups, separately examines development of SSC after MPN, has a long follow-up period and has multicenter design. In MPN patients, malignancy screening gains more importance for those aged ≥65 and males. Our study evaluated with data from previous studies suggest that increased risk of developing SSC in M","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924000877/pdfft?md5=0e6095a5a9bc0944605514aef0655acf&pid=1-s2.0-S2531137924000877-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878901","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
The incidence of extranodal presentation of the disease is less than 10% in follicular lymphomas. Follicular lymphoma with plasmacytic differentiation arising in an extranodal site like subcutaneous tissue and bone is uncommon and its natural history and treatment are poorly described in the literature.
Objective
Sharing an unusual case report of a resistant extranodal follicular lymphoma with plasmacytic differentiation transformed in Diffuse Large B Cell lymphoma ABC subtype undergoing successful treatment with bone marrow transplantation.
Case report
In November 2012 a 48-year-old woman was complaining about knee pain during movements. A CT done at that time demonstrated an osteolytic lesion in her right knee in the lateral condyle. The biopsy of the lesion was consistent with the diagnosis of follicular lymphoma with plasmocytic differentiation. Bone marrow aspiration and total body CT were normal without evidence of other tumor masses. The patient underwent radiation therapy and was in perfect condition until late 2017 when she was presented to the hematology consultation because of some subcutaneous masses on her body. PET CT scan revealed several subcutaneous masses with high FDG uptake, one in her right shoulder (3.5 × 1.8 cm), two on her right breast (6.0 × 3.4 cm and 2.1 × 1.3 cm), one on the left side of her neck (1.5 × 0.6 cm), and one on her left inguinal region (4.0 × 2.3 cm). A biopsy of the mass in her inguinal region revealed the diagnosis of follicular lymphoma with plasmacytic differentiation (CD10, CD20, CD138, and MUM1 positive). She was referred to the hematology department for further evaluation and treatment. On admission, the bone marrow aspiration and biopsy showed no malignant diseases. Due to the perfect clinical condition of the patient, we decided to go with Rituximab monotherapy. But after 4 courses no improvement was seen. So, we decided to go with RCVP therapy but still, the disease was refractory, and the PET CT showed other than the subcutaneous masses, a lytic bone lesion in her left talus. We went with 2 RCHOP therapies and 4 RCHOEP plus Bortezomib and only after that, the patient went into total remission. One year later, the masses started to grow in the same location. A second biopsy revealed high-grade follicular lymphoma. We continued with R-lenalidomide but the disease was still refractory. A third biopsy performed showed a high-grade DLBCL ABC subtype. In this condition, we started salvage therapy with 2 cycles of R-BEGEV protocol and referred the patient to a clinic abroad for autologous bone marrow transplantation. The patient underwent total remission after the protocol and autologous bone marrow transplant. She has been in remission since July 2022.
Discussion
The transformation of follicular lymphoma with plasmacytic differentiation, positive for MUM1 has a high probability according to l
{"title":"A RARE CASE OF A RESISTANT EXTRANODAL FOLLICULAR LYMPHOMA WITH PLASMACYTIC DIFFERENTIATION TRANSFORMED IN DIFFUSE LARGE B CELL LYMPHOMA TREATED SUCCESSFULLY WITH AUTOLOGOUS BONE MARROW TRANSPLANTATION.","authors":"Viola CAVOLLI , Aferdita UKIMERAJ , Suzana KRASNIQI","doi":"10.1016/j.htct.2024.04.030","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.030","url":null,"abstract":"<div><h3>Case report: Context</h3><p>The incidence of extranodal presentation of the disease is less than 10% in follicular lymphomas. Follicular lymphoma with plasmacytic differentiation arising in an extranodal site like subcutaneous tissue and bone is uncommon and its natural history and treatment are poorly described in the literature.</p></div><div><h3>Objective</h3><p>Sharing an unusual case report of a resistant extranodal follicular lymphoma with plasmacytic differentiation transformed in Diffuse Large B Cell lymphoma ABC subtype undergoing successful treatment with bone marrow transplantation.</p></div><div><h3>Case report</h3><p>In November 2012 a 48-year-old woman was complaining about knee pain during movements. A CT done at that time demonstrated an osteolytic lesion in her right knee in the lateral condyle. The biopsy of the lesion was consistent with the diagnosis of follicular lymphoma with plasmocytic differentiation. Bone marrow aspiration and total body CT were normal without evidence of other tumor masses. The patient underwent radiation therapy and was in perfect condition until late 2017 when she was presented to the hematology consultation because of some subcutaneous masses on her body. PET CT scan revealed several subcutaneous masses with high FDG uptake, one in her right shoulder (3.5 × 1.8 cm), two on her right breast (6.0 × 3.4 cm and 2.1 × 1.3 cm), one on the left side of her neck (1.5 × 0.6 cm), and one on her left inguinal region (4.0 × 2.3 cm). A biopsy of the mass in her inguinal region revealed the diagnosis of follicular lymphoma with plasmacytic differentiation (CD10, CD20, CD138, and MUM1 positive). She was referred to the hematology department for further evaluation and treatment. On admission, the bone marrow aspiration and biopsy showed no malignant diseases. Due to the perfect clinical condition of the patient, we decided to go with Rituximab monotherapy. But after 4 courses no improvement was seen. So, we decided to go with RCVP therapy but still, the disease was refractory, and the PET CT showed other than the subcutaneous masses, a lytic bone lesion in her left talus. We went with 2 RCHOP therapies and 4 RCHOEP plus Bortezomib and only after that, the patient went into total remission. One year later, the masses started to grow in the same location. A second biopsy revealed high-grade follicular lymphoma. We continued with R-lenalidomide but the disease was still refractory. A third biopsy performed showed a high-grade DLBCL ABC subtype. In this condition, we started salvage therapy with 2 cycles of R-BEGEV protocol and referred the patient to a clinic abroad for autologous bone marrow transplantation. The patient underwent total remission after the protocol and autologous bone marrow transplant. She has been in remission since July 2022.</p></div><div><h3>Discussion</h3><p>The transformation of follicular lymphoma with plasmacytic differentiation, positive for MUM1 has a high probability according to l","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924001123/pdfft?md5=22c64bd06c3cbe63459a9c57988f9b96&pid=1-s2.0-S2531137924001123-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878830","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-05-01DOI: 10.1016/j.htct.2024.04.013
orhan Ayyildiz, Songul Beskisiz, Abdullah Karakus
Case report
Multiple myeloma is a hematological malignancy that develops as a result of clonal proliferation of plasma cells and progresses with remissions and relapses. It is clinically characterized by many symptoms and signs such as osteolytic bone lesions, hypercalcemia, renal dysfunction, hypergammaglobulinemia and anemia. However, involvement of the central nervous system, especially the leptomeningeal/cranial region, is a rare and prognostically important form of relapse of the disease. Nervous syste
{"title":"A VERY RARE RELAPS TYPE IN MULTIPLE MYELOMA: LEPTOMENGEAL AND CRANIAL INVOLVEMENT","authors":"orhan Ayyildiz, Songul Beskisiz, Abdullah Karakus","doi":"10.1016/j.htct.2024.04.013","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.013","url":null,"abstract":"<div><h3>Case report</h3><p>Multiple myeloma is a hematological malignancy that develops as a result of clonal proliferation of plasma cells and progresses with remissions and relapses. It is clinically characterized by many symptoms and signs such as osteolytic bone lesions, hypercalcemia, renal dysfunction, hypergammaglobulinemia and anemia. However, involvement of the central nervous system, especially the leptomeningeal/cranial region, is a rare and prognostically important form of relapse of the disease. Nervous syste</p></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924000956/pdfft?md5=e35c00bf1a2d230779b854b0b1c3514c&pid=1-s2.0-S2531137924000956-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878987","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Primary plasma cell leukemia is a rare and aggressive variant of plasma cell neoplasm, and its diagnosis is based on the percentage (≥ 20%) of circulating plasma cells in the peripheral blood. It accounts for about 0.5-2% of all plasma cell dyscrasias and the median age of presentation is 55 years. In young adults, it is even rarer, and only a few isolated case reports have been reported.
Objective
In this case, we are representing an aggressive form of plasma cell leukemia that was successfully treated with daratumumab therapy and autologous bone marrow transplantation.
Case report
In October 2021 a 38-year-old man was admitted to the emergency room with extreme sweating and fatigue, problems with urination, and pain in the lower abdomen. The complete blood count showed anemia (Hb=8.0 g/dl) and biochemistry showed high levels of urea (26.55 mmol/l) and creatinine (1142 µmol/l). He was admitted to the nephrology department when he started immediate dialysis. Abdominal ultrasound showed splenomegaly (180mm). Because of anemia and splenomegaly, a hematologist consultation was requested. Immune electrophoresis revealed low levels of IgG, IgM, IgA, and kappa chains (4.9 mg/l) and normal levels of lambda chains (26.3 mg/l). Lambda/kappa ratio was 5.36. The sedimentation rate was 150 mm/h, there were no osteolytic bone lesions according to standard X-rays and calcium levels were normal. Peripheral blood smear showed plasma cells up to 22 percent. Bone marrow aspiration and biopsy showed full infiltration with plasma cells with lambda expression that were CD56 negative and CD38 and CD138 positive. The diagnosis of plasma cell leukemia was made, and he was transferred to the hematology union for further therapy. We started chemotherapy with the VTD PACE protocol. After 2 cycles bone marrow aspiration was performed and still the presence of more than 90% of plasma cells was detected. The patient was still in dialysis and in critical condition with a Lambda/kappa ratio of 100 (1200/11.9 mg/l). Because the disease was refractory, he was referred to a clinic outside of Kosovo for further therapy and bone marrow transplantation. He received triple therapy with Daratumumab, Thalidomide, and Bortezomib. After two cycles he underwent remission, and an Autologous bone marrow transplant was successful. The patient has been in remission since July 2022. He is taking subcutaneous Bortezomib every two weeks and is no longer on dialysis.
Discussion
In this case, the patient demonstrated an aggressive clinical course with typical features of plasma cell leukemia i.e. severe anemia, renal failure, lack of bone involvement, more than 20% plasma cell on peripheral blood smear, splenomegaly and bone marrow infiltration of plasma cells negative for CD56. Daratumumab therapy followed by autologous bone marrow transplantation was successful and was the best treatmen
{"title":"A SUCCESSFUL CASE OF PRIMARY PLASMA CELL LEUKEMIA TREATED WITH DARATUMUMAB-BASED THERAPY FOLLOWED BY AUTOLOGOUS BONE MARROW TRANSPLANTATION","authors":"Aferdita UKIMERAJ , Viola Cavolli , Suzana KRASNIQI","doi":"10.1016/j.htct.2024.04.035","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.035","url":null,"abstract":"<div><h3>Case report: Context</h3><p>Primary plasma cell leukemia is a rare and aggressive variant of plasma cell neoplasm, and its diagnosis is based on the percentage (≥ 20%) of circulating plasma cells in the peripheral blood. It accounts for about 0.5-2% of all plasma cell dyscrasias and the median age of presentation is 55 years. In young adults, it is even rarer, and only a few isolated case reports have been reported.</p></div><div><h3>Objective</h3><p>In this case, we are representing an aggressive form of plasma cell leukemia that was successfully treated with daratumumab therapy and autologous bone marrow transplantation.</p></div><div><h3>Case report</h3><p>In October 2021 a 38-year-old man was admitted to the emergency room with extreme sweating and fatigue, problems with urination, and pain in the lower abdomen. The complete blood count showed anemia (Hb=8.0 g/dl) and biochemistry showed high levels of urea (26.55 mmol/l) and creatinine (1142 µmol/l). He was admitted to the nephrology department when he started immediate dialysis. Abdominal ultrasound showed splenomegaly (180mm). Because of anemia and splenomegaly, a hematologist consultation was requested. Immune electrophoresis revealed low levels of IgG, IgM, IgA, and kappa chains (4.9 mg/l) and normal levels of lambda chains (26.3 mg/l). Lambda/kappa ratio was 5.36. The sedimentation rate was 150 mm/h, there were no osteolytic bone lesions according to standard X-rays and calcium levels were normal. Peripheral blood smear showed plasma cells up to 22 percent. Bone marrow aspiration and biopsy showed full infiltration with plasma cells with lambda expression that were CD56 negative and CD38 and CD138 positive. The diagnosis of plasma cell leukemia was made, and he was transferred to the hematology union for further therapy. We started chemotherapy with the VTD PACE protocol. After 2 cycles bone marrow aspiration was performed and still the presence of more than 90% of plasma cells was detected. The patient was still in dialysis and in critical condition with a Lambda/kappa ratio of 100 (1200/11.9 mg/l). Because the disease was refractory, he was referred to a clinic outside of Kosovo for further therapy and bone marrow transplantation. He received triple therapy with Daratumumab, Thalidomide, and Bortezomib. After two cycles he underwent remission, and an Autologous bone marrow transplant was successful. The patient has been in remission since July 2022. He is taking subcutaneous Bortezomib every two weeks and is no longer on dialysis.</p></div><div><h3>Discussion</h3><p>In this case, the patient demonstrated an aggressive clinical course with typical features of plasma cell leukemia i.e. severe anemia, renal failure, lack of bone involvement, more than 20% plasma cell on peripheral blood smear, splenomegaly and bone marrow infiltration of plasma cells negative for CD56. Daratumumab therapy followed by autologous bone marrow transplantation was successful and was the best treatmen","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924001172/pdfft?md5=923f9918f147f6de2f22109d898a6e4a&pid=1-s2.0-S2531137924001172-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878503","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-05-01DOI: 10.1016/j.htct.2024.04.039
Suad Enaami , Ebtihaj Hassan , Jalal Eltabib
Objective
Carcinoma ex pleomorphic adenoma CXPA, a rare epithelial malignancy arising from a primary or recurrent benign pleomorphic adenoma, accounts for 11.% of all malignant salivary gland neoplasms. It is difficult to diagnose preoperatively. often poses a diagnostic challenge to clinicians and pathologists Treatment involves an ablative surgical procedure with neck dissection followed by radiotherapy. We aim to investigate the impact of postoperative radiotherapy on improving disease-free survival.
Case report
A 39-year-old Libyan male presented with painless swelling near the angle of the right mandible four months ago. FNA Cytology showed a benign pleomorphic adenoma. A total parotidectomy with VII CN preservation was done in September 2022. The histopathological features were consistent with carcinoma EX pleomorphic adenoma, a widely invasive salivary duct carcinoma grade III with < 1mm(close)margins staged PT1 N0 M0. The immunohistochemistry revealed the negative expression of ER and PR assays.
Methodology
In December 2022, he received adjuvant radiation to the tumor bed (66 GY) in 33 fractions over 6 weeks based on the VMAT technique. 12-month follow-up, the patient showed no evidence of local or regional disease recurrence or distant metastasis.
Results
Radical surgery, followed by adjuvant radiotherapy, should be considered the standard of care for a patient, with significant improvement in 5-year locoregional control. and in general, salivary gland neoplasms respond poorly to chemotherapy and are currently indicated only for palliative sitting. More prospective data is needed to establish a role for hormonal therapy and molecularly targeted therapies.
Conclusion
CXPA is an uncommonly aggressive malignancy that, if associated with regional metastasis, invariably leads to mortality. Total resection of the tumor, followed by adjuvant radiotherapy, should be considered the standard of care for a patient with significantly improved 5-year locoregional control. Early and prompt diagnosis, followed by aggressive surgical intervention and adjuvant radiotherapy for patients with carcinoma ex pleomorphic adenoma, can enhance their survival rates.
{"title":"CARCINOMA EX PLEOMORPHIC ADENOMA: DIAGNOSTIC CHALLENGE AND TREATMENT PROTOCOL","authors":"Suad Enaami , Ebtihaj Hassan , Jalal Eltabib","doi":"10.1016/j.htct.2024.04.039","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.039","url":null,"abstract":"<div><h3>Objective</h3><p>Carcinoma ex pleomorphic adenoma CXPA, a rare epithelial malignancy arising from a primary or recurrent benign pleomorphic adenoma, accounts for 11.% of all malignant salivary gland neoplasms. It is difficult to diagnose preoperatively. often poses a diagnostic challenge to clinicians and pathologists Treatment involves an ablative surgical procedure with neck dissection followed by radiotherapy. We aim to investigate the impact of postoperative radiotherapy on improving disease-free survival.</p></div><div><h3>Case report</h3><p>A 39-year-old Libyan male presented with painless swelling near the angle of the right mandible four months ago. FNA Cytology showed a benign pleomorphic adenoma. A total parotidectomy with VII CN preservation was done in September 2022. The histopathological features were consistent with carcinoma EX pleomorphic adenoma, a widely invasive salivary duct carcinoma grade III with < 1mm(close)margins staged PT1 N0 M0. The immunohistochemistry revealed the negative expression of ER and PR assays.</p></div><div><h3>Methodology</h3><p>In December 2022, he received adjuvant radiation to the tumor bed (66 GY) in 33 fractions over 6 weeks based on the VMAT technique. 12-month follow-up, the patient showed no evidence of local or regional disease recurrence or distant metastasis.</p></div><div><h3>Results</h3><p>Radical surgery, followed by adjuvant radiotherapy, should be considered the standard of care for a patient, with significant improvement in 5-year locoregional control. and in general, salivary gland neoplasms respond poorly to chemotherapy and are currently indicated only for palliative sitting. More prospective data is needed to establish a role for hormonal therapy and molecularly targeted therapies.</p></div><div><h3>Conclusion</h3><p>CXPA is an uncommonly aggressive malignancy that, if associated with regional metastasis, invariably leads to mortality. Total resection of the tumor, followed by adjuvant radiotherapy, should be considered the standard of care for a patient with significantly improved 5-year locoregional control. Early and prompt diagnosis, followed by aggressive surgical intervention and adjuvant radiotherapy for patients with carcinoma ex pleomorphic adenoma, can enhance their survival rates.</p></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924001214/pdfft?md5=75b657d0a63955ad496d482026200de2&pid=1-s2.0-S2531137924001214-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878504","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Peripheral T-cell lymphomas (PTCLs) are a heterogeneous group of aggressive non-Hodgkin lymphomas with a rare occurrence, representing less than 15% of all adult non-Hodgkin lymphomas. The diagnosis and treatment of PTCLs pose significant challenges due to their diverse presentations and the aggressive nature of the disease. This case report discusses a 58-year-old male with a long-standing history of diabetes mellitus and previous bypass surgery, who presented with jaundice, hepatosplenomegaly, and ascites. Laboratory findings showed anemia, elevated liver enzymes, and hyponatremia. Imaging and biopsy results revealed nodular lung lesions, hepatosplenomegaly, liver mass lesions, bile duct dilatation, abdominopelvic lymphadenopathies, and T-cell lymphoma infiltration. The patient's treatment protocol included the CHOEP + BV regimen, alongside interventions for hyperbilirubinemia and renal failure. This case underscores the atypical presentation of PTCL with jaundice and the complexities involved in diagnosing and managing such cases, highlighting the need for a thorough and multidisciplinary approach.
{"title":"Peripheral T-cell Lymphoma with Jaundice: Insights from a Complex Case","authors":"Candas MUMCU , Bengisu Ece DUMAN , Berra Nur ISCI , Emre BAL , Irem KABALCI KADIOGLU , Bulut SAT , Meryem SENER , Hayriye TEZCAN , Arbil ACIKALIN , Birol GUVENC","doi":"10.1016/j.htct.2024.04.020","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.020","url":null,"abstract":"<div><h3>CASE</h3><p>Peripheral T-cell lymphomas (PTCLs) are a heterogeneous group of aggressive non-Hodgkin lymphomas with a rare occurrence, representing less than 15% of all adult non-Hodgkin lymphomas. The diagnosis and treatment of PTCLs pose significant challenges due to their diverse presentations and the aggressive nature of the disease. This case report discusses a 58-year-old male with a long-standing history of diabetes mellitus and previous bypass surgery, who presented with jaundice, hepatosplenomegaly, and ascites. Laboratory findings showed anemia, elevated liver enzymes, and hyponatremia. Imaging and biopsy results revealed nodular lung lesions, hepatosplenomegaly, liver mass lesions, bile duct dilatation, abdominopelvic lymphadenopathies, and T-cell lymphoma infiltration. The patient's treatment protocol included the CHOEP + BV regimen, alongside interventions for hyperbilirubinemia and renal failure. This case underscores the atypical presentation of PTCL with jaundice and the complexities involved in diagnosing and managing such cases, highlighting the need for a thorough and multidisciplinary approach.</p></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924001020/pdfft?md5=17e9a7dfc4ffdf28039f945573456e29&pid=1-s2.0-S2531137924001020-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878984","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-05-01DOI: 10.1016/j.htct.2024.04.028
asli odabasi giden , duzgun ozatli , piltan buyukkaya , ozgur meletli
Objective
Mantle cell lymphoma (MCL) is a rare subtype of B-cell lymphoma characterized by clinical and biological heterogeneity. Lymph nodes are the most commonly involved sites. Other important regions affected by the disease are bone marrow and spleen. However, skin involvement is rare in MCL, and most cases occur due to secondary cutaneous spread of disseminated disease. In this report, a case of relapsed, refractory (R/R) MCL with skin lesions is discussed.
Case report
A 43-year-old male patient was admitted to our clinic with the complaint of palpable cervical and axillary diffuse lymph nodes. The patient was diagnosed with MCL as a result of lymph node biopsy, and was evaluated as stage 4 and a high-risk disease according to the MIPI scoring system, After chemoimmunotherapy, autologous bone marrow transplantation was performed. The patient who was followed up as a complete response, macular lesions raised from the skin appeared on the lower extremities 4 years after the initial diagnosis (Figure 1), and a skin biopsy was performed; MCL was evaluated as R/R disease. In the immunohistochemical study, CD5, CyclinD1 were positive, Sox-11 was weakly positive, and Ki 67 were evaluated as 100%. The patient was delivered rituximab + ibrutinib (R+I) treatments. After treatment, skin lesions disappeared. After 3 cycles of treatment, the patient underwent an allogeneic bone marrow transplant from his fully compatible sibling. During this period, skin lesions appeared on the trunk, and a skin biopsy was performed; It was evaluated as GVHD (graft versus host disease) and prednol treatment was delivered. The patient, who was evaluated as prednol refractory during the follow-up, was delivered JAK-2 inhibitor and his complaints regressed. The patient was evaluated as a complete metabolic response at the 3rd month post-transplant follow-up.
Figure-1 Lower extremity skin involvement
Methodology
Conclusion
MCL is a different type of non-Hodgkin lymphoma that usually affects extranodal sites. The most commonly affected areas are the bone marrow, gastrointestinal tract, and Waldeyer's ring, but the skin is rarely affected. The disease can present with a wide variety of lesions, ranging from petechial erythematous macules to subcutaneous nodules, and very atypical presentations, such as acneiform lesions, have also been reported. Since extremity and trunk involvement is more common, skin involvement can be seen anywhere in the body. Most often, skin lesions are accompanied by systemic symptoms, but a few cases of only cutaneous lesions without systemic involvement have been described. Skin lesions may develop before clinical symptoms appear. In one report describing five cases of MCL involving the skin; 3 patients initially presented with skin lesions but there was evidence of extensive disease at diagnosis. MCL can often involve the skin as a manifestation of disseminated disease and
{"title":"A CASE OF RELAPSED REFRACTORY MANTLE CELL LYMPHOMA PRESENTING WITH SKIN LESIONS","authors":"asli odabasi giden , duzgun ozatli , piltan buyukkaya , ozgur meletli","doi":"10.1016/j.htct.2024.04.028","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.028","url":null,"abstract":"<div><h3>Objective</h3><p>Mantle cell lymphoma (MCL) is a rare subtype of B-cell lymphoma characterized by clinical and biological heterogeneity. Lymph nodes are the most commonly involved sites. Other important regions affected by the disease are bone marrow and spleen. However, skin involvement is rare in MCL, and most cases occur due to secondary cutaneous spread of disseminated disease. In this report, a case of relapsed, refractory (R/R) MCL with skin lesions is discussed.</p></div><div><h3>Case report</h3><p>A 43-year-old male patient was admitted to our clinic with the complaint of palpable cervical and axillary diffuse lymph nodes. The patient was diagnosed with MCL as a result of lymph node biopsy, and was evaluated as stage 4 and a high-risk disease according to the MIPI scoring system, After chemoimmunotherapy, autologous bone marrow transplantation was performed. The patient who was followed up as a complete response, macular lesions raised from the skin appeared on the lower extremities 4 years after the initial diagnosis (Figure 1), and a skin biopsy was performed; MCL was evaluated as R/R disease. In the immunohistochemical study, CD5, CyclinD1 were positive, Sox-11 was weakly positive, and Ki 67 were evaluated as 100%. The patient was delivered rituximab + ibrutinib (R+I) treatments. After treatment, skin lesions disappeared. After 3 cycles of treatment, the patient underwent an allogeneic bone marrow transplant from his fully compatible sibling. During this period, skin lesions appeared on the trunk, and a skin biopsy was performed; It was evaluated as GVHD (graft versus host disease) and prednol treatment was delivered. The patient, who was evaluated as prednol refractory during the follow-up, was delivered JAK-2 inhibitor and his complaints regressed. The patient was evaluated as a complete metabolic response at the 3rd month post-transplant follow-up.</p><p>Figure-1 Lower extremity skin involvement</p><p>Methodology</p></div><div><h3>Conclusion</h3><p>MCL is a different type of non-Hodgkin lymphoma that usually affects extranodal sites. The most commonly affected areas are the bone marrow, gastrointestinal tract, and Waldeyer's ring, but the skin is rarely affected. The disease can present with a wide variety of lesions, ranging from petechial erythematous macules to subcutaneous nodules, and very atypical presentations, such as acneiform lesions, have also been reported. Since extremity and trunk involvement is more common, skin involvement can be seen anywhere in the body. Most often, skin lesions are accompanied by systemic symptoms, but a few cases of only cutaneous lesions without systemic involvement have been described. Skin lesions may develop before clinical symptoms appear. In one report describing five cases of MCL involving the skin; 3 patients initially presented with skin lesions but there was evidence of extensive disease at diagnosis. MCL can often involve the skin as a manifestation of disseminated disease and","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S253113792400110X/pdfft?md5=0cd62e4e6459fa71b53b23afa57b467d&pid=1-s2.0-S253113792400110X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878828","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-05-01DOI: 10.1016/j.htct.2024.04.004
Weronika Lebowa , Jakub Dybaś , Stefan Chłopicki , Tomasz Sacha
Objective
Polycythemia vera (PV) is a chronic myeloproliferative neoplasm characterized by an increase in red blood cell mass. Thrombotic complications are the main cause of morbidity and mortality in PV. Elevated hematocrit and increased blood viscosity are crucial risk factors for thrombus formation. The aim of our analysis is to evaluate the biochemical alterations in red blood cells (RBCs) and the hemoglobin structure in patients with PV that may be associated with thrombotic complications.
Methodology
Blood samples were taken from 20 PV patients and 16 healthy individuals. The isolated RBCs were examined using Raman spectroscopy.
Results
We found a larger contribution of ferrous heme iron, which is a molecular state typical for deoxyhemoglobin in PV samples compared to the control samples. Furthermore, a significant increase in the Fe II/Fe III ratio in PV samples was correlated with a higher hematocrit (Hct) to hemoglobin (Hgb) ratio. A positive trend between a higher Fe II/ Fe III ratio and a higher RDW-SD and RDW-CV was observed in PV samples. In RBCs collected from PV patients we observed a less stable hemoglobin structure.
Conclusion
Higher values of RDW-SD and RDW-CV may reflect a higher Fe II/ Fe III and be a simple indicator of biochemical alterations in RBCs. A higher Hct/ Hgb ratio could indicate higher clonal myeloproliferative potential and be associated with shorter time to thrombosis in patients with PV. Our future analysis will focus on correlating the above observations with the prothrombotic activity to demonstrate a possible link between the biochemical alterations of RBCs and the thrombotic complications in PV.
{"title":"BIOCHEMICAL PROPERTIES OF RED BLOOD CELLS IN POLYCYTHEMIA VERA","authors":"Weronika Lebowa , Jakub Dybaś , Stefan Chłopicki , Tomasz Sacha","doi":"10.1016/j.htct.2024.04.004","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.004","url":null,"abstract":"<div><h3>Objective</h3><p>Polycythemia vera (PV) is a chronic myeloproliferative neoplasm characterized by an increase in red blood cell mass. Thrombotic complications are the main cause of morbidity and mortality in PV. Elevated hematocrit and increased blood viscosity are crucial risk factors for thrombus formation. The aim of our analysis is to evaluate the biochemical alterations in red blood cells (RBCs) and the hemoglobin structure in patients with PV that may be associated with thrombotic complications.</p></div><div><h3>Methodology</h3><p>Blood samples were taken from 20 PV patients and 16 healthy individuals. The isolated RBCs were examined using Raman spectroscopy.</p></div><div><h3>Results</h3><p>We found a larger contribution of ferrous heme iron, which is a molecular state typical for deoxyhemoglobin in PV samples compared to the control samples. Furthermore, a significant increase in the Fe II/Fe III ratio in PV samples was correlated with a higher hematocrit (Hct) to hemoglobin (Hgb) ratio. A positive trend between a higher Fe II/ Fe III ratio and a higher RDW-SD and RDW-CV was observed in PV samples. In RBCs collected from PV patients we observed a less stable hemoglobin structure.</p></div><div><h3>Conclusion</h3><p>Higher values of RDW-SD and RDW-CV may reflect a higher Fe II/ Fe III and be a simple indicator of biochemical alterations in RBCs. A higher Hct/ Hgb ratio could indicate higher clonal myeloproliferative potential and be associated with shorter time to thrombosis in patients with PV. Our future analysis will focus on correlating the above observations with the prothrombotic activity to demonstrate a possible link between the biochemical alterations of RBCs and the thrombotic complications in PV.</p></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924000865/pdfft?md5=81949c8b4f05ac0477b8e58a21f34bc3&pid=1-s2.0-S2531137924000865-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878899","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-05-01DOI: 10.1016/j.htct.2024.04.026
orhan Ayyildiz , sengul baran , hazal sahin , abdullah karakus
Case report: INTRODUCTION
Adrenal glands are one of the organs where malignancies frequently metastasize. However, primary malignancies of the adrenal glands are rare. Primary adrenal lymphomas (PAL) account for less than 1% of extranodal lymphomas. It is seen bilaterally in 75% of cases. The most common subtype is diffuse large B-cell lymphoma.
CASE
A 57-year-old male patient with no known history of disease applied to an external center with complaints of weight loss (13 kg, more than 10% of body weight), tremors, loss of appetite and lower back pain. As a result of the evaluations made at an external center, thorax CT showed bilateral adrenal masses. PET CT was taken with the preliminary diagnosis of malignancy; In the right adrenal gland, approximately 11.6 × 8.1cm (SUVmax: 25.6) and in the left adrenal gland, approximately 10.1 × 7.2cm (SUVmax: 24.4) in size, heterogeneous dense hypermetabolic solid mass sections with necrotic areas were seen and left paraaortic (SUVmax: 11.7) lymph node with dimensions of 1.6 × 1.2 cm and a few mildly-intensely hypermetabolic lymph nodes were observed in the interaortocaval chain. Primary malignancy storage was evaluated in the foreground of dense hypermetabolic mass regions of heterogeneous structure defined in both adrenal glands. After pheochromocytoma was diagnosed, the patient was referred to our clinic after the tru-cut pathology performed on the mass lesions in the right adrenal gland revealed that the morphological and histochemical findings were consistent with diffuse large B-cell lymphoma.
The patient's vital signs were stable upon admission. In the hemogram, Hgb: 9.7g/dL Hct: 31.2% MCV: 96 fL Platlet: 126.000 / mm3. In biochemistry, creatinine:1.83mg/dL urea:80 mg/dL Na:139 mmol/L K:4.36 mmol/L Ca:10.2mg/dL uric acid:9.4mg/dL LD:343U/L. There was no sign of adrenal insufficiency other than dehydration. The patient was started on hydration and allopurinol treatments. During follow-up, urea and creatinine levels decreased to normal limits. The patient's basal cortisol was 12.02 µg/dL and ACTH was 83.8ng/L. Low-dose (1µg) ACTH test was performed on the patient, for whom chemotherapy was planned for his primary disease, with the preliminary diagnosis of adrenal insufficiency. The patient's cortisol levels were detected as 8.23-10.35-8.93-9.75 µg/dL at 30-60-90 and 120 minutes, respectively, and hydrocortisone treatment was started with the diagnosis of adrenal insufficiency. During the follow-up of the patient, R-CHOP chemotherapy was started, and since the patient had prednisolone in the chemotherapy course, hydrocortisone was discontinued during chemotherapy and isolated prednisolone treatment was given. Central Nervous System involvement was detected in the cerebrospinal fluid during intrathecal (IT) chemotherapy (Mtx, Dexamethasone, Cytosine Arabinosine). Intrathecal therapy was initially administered 3 times a week and subsequently twice a week. Si
{"title":"ADRENAL INSUFFICIENCY DETECTED BEFORE TREATMENT IN A PATIENT DIAGNOSED WITH BILATERALLY PRIMARY ADRENAL DIFFUSE LARGE B CELL LYMPHOMA:A CASE REPORT","authors":"orhan Ayyildiz , sengul baran , hazal sahin , abdullah karakus","doi":"10.1016/j.htct.2024.04.026","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.026","url":null,"abstract":"<div><h3>Case report: INTRODUCTION</h3><p>Adrenal glands are one of the organs where malignancies frequently metastasize. However, primary malignancies of the adrenal glands are rare. Primary adrenal lymphomas (PAL) account for less than 1% of extranodal lymphomas. It is seen bilaterally in 75% of cases. The most common subtype is diffuse large B-cell lymphoma.</p></div><div><h3>CASE</h3><p>A 57-year-old male patient with no known history of disease applied to an external center with complaints of weight loss (13 kg, more than 10% of body weight), tremors, loss of appetite and lower back pain. As a result of the evaluations made at an external center, thorax CT showed bilateral adrenal masses. PET CT was taken with the preliminary diagnosis of malignancy; In the right adrenal gland, approximately 11.6 × 8.1cm (SUVmax: 25.6) and in the left adrenal gland, approximately 10.1 × 7.2cm (SUVmax: 24.4) in size, heterogeneous dense hypermetabolic solid mass sections with necrotic areas were seen and left paraaortic (SUVmax: 11.7) lymph node with dimensions of 1.6 × 1.2 cm and a few mildly-intensely hypermetabolic lymph nodes were observed in the interaortocaval chain. Primary malignancy storage was evaluated in the foreground of dense hypermetabolic mass regions of heterogeneous structure defined in both adrenal glands. After pheochromocytoma was diagnosed, the patient was referred to our clinic after the tru-cut pathology performed on the mass lesions in the right adrenal gland revealed that the morphological and histochemical findings were consistent with diffuse large B-cell lymphoma.</p><p>The patient's vital signs were stable upon admission. In the hemogram, Hgb: 9.7g/dL Hct: 31.2% MCV: 96 fL Platlet: 126.000 / mm3. In biochemistry, creatinine:1.83mg/dL urea:80 mg/dL Na:139 mmol/L K:4.36 mmol/L Ca:10.2mg/dL uric acid:9.4mg/dL LD:343U/L. There was no sign of adrenal insufficiency other than dehydration. The patient was started on hydration and allopurinol treatments. During follow-up, urea and creatinine levels decreased to normal limits. The patient's basal cortisol was 12.02 µg/dL and ACTH was 83.8ng/L. Low-dose (1µg) ACTH test was performed on the patient, for whom chemotherapy was planned for his primary disease, with the preliminary diagnosis of adrenal insufficiency. The patient's cortisol levels were detected as 8.23-10.35-8.93-9.75 µg/dL at 30-60-90 and 120 minutes, respectively, and hydrocortisone treatment was started with the diagnosis of adrenal insufficiency. During the follow-up of the patient, R-CHOP chemotherapy was started, and since the patient had prednisolone in the chemotherapy course, hydrocortisone was discontinued during chemotherapy and isolated prednisolone treatment was given. Central Nervous System involvement was detected in the cerebrospinal fluid during intrathecal (IT) chemotherapy (Mtx, Dexamethasone, Cytosine Arabinosine). Intrathecal therapy was initially administered 3 times a week and subsequently twice a week. Si","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924001081/pdfft?md5=b246bc191c1e5c28f0a32a5b0ba27940&pid=1-s2.0-S2531137924001081-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878834","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
This case report illustrates the unconventional progression of multiple myeloma (MM) in a 57-year-old male, primarily highlighted by a cutaneous manifestation on the right cheek malar region, indicative of disease recurrence. Initially diagnosed following back pain and dyspnea, the patient's journey took a distinctive path from standard multiple myeloma treatments to the innovative application of daratumumab + CYBORG therapy. The biopsy from the lesion confirmed the recurrence of plasma cell neoplasia, leading to the adoption of daratumumab + CYBORG therapy. This innovative treatment strategy underscores the evolving landscape of MM management, particularly in cases presenting with atypical symptoms such as cutaneous involvement. The implementation of daratumumab + CYBORG therapy in this context not only highlights its potential as a significant advancement in MM treatment.
本病例报告展示了一名 57 岁男性多发性骨髓瘤（MM）的非常规进展，主要突出表现为右颊颊区的皮肤表现，表明疾病复发。患者最初因背部疼痛和呼吸困难而被确诊，从标准的多发性骨髓瘤治疗到达拉单抗+CYBORG疗法的创新应用，患者的病程经历了一条与众不同的道路。病灶活检证实浆细胞瘤复发，因此采用了达拉单抗+CYBORG疗法。这一创新治疗策略凸显了 MM 治疗的不断发展，尤其是在出现皮肤受累等非典型症状的病例中。在这种情况下采用达拉单抗+CYBORG疗法，不仅凸显了其作为MM治疗领域重大进展的潜力，而且还为MM患者提供了一种新的治疗方法。
{"title":"A Case of Multiple Myeloma with Atypical Cutaneous Presentation Treated by Daratumumab + CYBORG","authors":"Bengisu Ece DUMAN , Candas MUMCU , Berra Nur ISCI , Emre BAL , Meryem SENER , Hande Yanar , Arbil ACIKALIN , Birol GUVENC","doi":"10.1016/j.htct.2024.04.017","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.017","url":null,"abstract":"<div><p>This case report illustrates the unconventional progression of multiple myeloma (MM) in a 57-year-old male, primarily highlighted by a cutaneous manifestation on the right cheek malar region, indicative of disease recurrence. Initially diagnosed following back pain and dyspnea, the patient's journey took a distinctive path from standard multiple myeloma treatments to the innovative application of daratumumab + CYBORG therapy. The biopsy from the lesion confirmed the recurrence of plasma cell neoplasia, leading to the adoption of daratumumab + CYBORG therapy. This innovative treatment strategy underscores the evolving landscape of MM management, particularly in cases presenting with atypical symptoms such as cutaneous involvement. The implementation of daratumumab + CYBORG therapy in this context not only highlights its potential as a significant advancement in MM treatment.</p></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924000993/pdfft?md5=db9e70153ef4ae198bceed14bdd02f75&pid=1-s2.0-S2531137924000993-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878981","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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