Pub Date : 2022-11-06DOI: 10.17650/1818-8346-2022-17-4-94-105
O. Vinogradova, M. Pankrashkina, D. Shikhbabaeva, M. Chernikov, A. L. Neverova, V. Ivanova, E. Nikitin, E. Usikova, V. Ptushkin
Background. For many years the primary aim of treatment strategy for ph-negative myeloproliferative neoplasms has been to restrain disease progression, with lasting relief and management of symptoms to improve patients’ quality of life. Generally, this did not lead to a significant increase in life expectancy with primary myelofibrosis and didn’t decrease the risk of fibrosis in patients with polycythemia vera and essential thrombocythemia. To date a new class of targeted drugs has been developed, it is JAK2 inhibitors with pathogenetic effects. The results of clinical trials showed the high efficacy of the first registered drug of this its kind – ruxolitinib – that includes a faster reduction in the symptoms of tumor intoxication and in symptoms associated with the development of splenomegaly and increase in the overall survival rates. It is known that the data obtained during clinical trials of medicines may differ from the results obtained in routine clinical practice. In actual practice drugs are used in a much wider heterogeneous population of patients, less limited first of all by age and comorbid characteristics. It is possible to analyze cohorts of patients including a larger number of clinical cases with a longer follow-up period. In this regard of great interest is the actual clinical experience of long-term use of ruxolitinib in patients whose set is limited only by clinical contraindications for prescribing the drug.Aim. To present our own actual experience of targeted therapy of myelofibrosis and compare the results obtained with the data of clinical trials.Materials and methods. Our analysis includes data from 141 patients (67 (47.5 %) men and 74 (52.5 %) women) in a chronic phase myelofibrosis. All patients received ruxolitinib. Of these, 109 (69 %) patients had primary myelofibrosis, 26 (16 %) – postpolycythemia myelofibrosis, 6 (4 %) – postessential thrombocythemia myelofibrosis. The median age at the start of therapy was 62 (18–84) years. The median disease duration before ruxolitinib was prescribed – 79 (1–401) months. According to the dIpSS (dynamic International prognostic Scoring System) criteria, 13 % of patients were assigned to the low risk group, 38 % – to the intermediate-1, 36 % – to the intermediate-2, 13 % – to the high risk group. Most patients (52 %) had grade 3 bone marrow fibrosis.Results. The median duration of treatment was 18 (range from 1 to 115) months. Symptoms of intoxication were relieved 74 (81 %) of 91 patients, the spleen size decreased in 81 % of patients (the spleen size returned to normal in 25 % of patients). The increase in the median hemoglobin level was 15 %. The proportion of patients requiring blood transfusion decreased by 4 times (from 39 to 9 %). Mean platelet levels normalized in most patients with baseline high and low platelet levels. A complete clinical and hematological response was achieved in 16 % (n = 23) of cases, a partial response – in 26 % (n = 37) of cases, clinical improvement
{"title":"Possibilities of targeted therapy for myelofibrosis: Moscow experience","authors":"O. Vinogradova, M. Pankrashkina, D. Shikhbabaeva, M. Chernikov, A. L. Neverova, V. Ivanova, E. Nikitin, E. Usikova, V. Ptushkin","doi":"10.17650/1818-8346-2022-17-4-94-105","DOIUrl":"https://doi.org/10.17650/1818-8346-2022-17-4-94-105","url":null,"abstract":"Background. For many years the primary aim of treatment strategy for ph-negative myeloproliferative neoplasms has been to restrain disease progression, with lasting relief and management of symptoms to improve patients’ quality of life. Generally, this did not lead to a significant increase in life expectancy with primary myelofibrosis and didn’t decrease the risk of fibrosis in patients with polycythemia vera and essential thrombocythemia. To date a new class of targeted drugs has been developed, it is JAK2 inhibitors with pathogenetic effects. The results of clinical trials showed the high efficacy of the first registered drug of this its kind – ruxolitinib – that includes a faster reduction in the symptoms of tumor intoxication and in symptoms associated with the development of splenomegaly and increase in the overall survival rates. It is known that the data obtained during clinical trials of medicines may differ from the results obtained in routine clinical practice. In actual practice drugs are used in a much wider heterogeneous population of patients, less limited first of all by age and comorbid characteristics. It is possible to analyze cohorts of patients including a larger number of clinical cases with a longer follow-up period. In this regard of great interest is the actual clinical experience of long-term use of ruxolitinib in patients whose set is limited only by clinical contraindications for prescribing the drug.Aim. To present our own actual experience of targeted therapy of myelofibrosis and compare the results obtained with the data of clinical trials.Materials and methods. Our analysis includes data from 141 patients (67 (47.5 %) men and 74 (52.5 %) women) in a chronic phase myelofibrosis. All patients received ruxolitinib. Of these, 109 (69 %) patients had primary myelofibrosis, 26 (16 %) – postpolycythemia myelofibrosis, 6 (4 %) – postessential thrombocythemia myelofibrosis. The median age at the start of therapy was 62 (18–84) years. The median disease duration before ruxolitinib was prescribed – 79 (1–401) months. According to the dIpSS (dynamic International prognostic Scoring System) criteria, 13 % of patients were assigned to the low risk group, 38 % – to the intermediate-1, 36 % – to the intermediate-2, 13 % – to the high risk group. Most patients (52 %) had grade 3 bone marrow fibrosis.Results. The median duration of treatment was 18 (range from 1 to 115) months. Symptoms of intoxication were relieved 74 (81 %) of 91 patients, the spleen size decreased in 81 % of patients (the spleen size returned to normal in 25 % of patients). The increase in the median hemoglobin level was 15 %. The proportion of patients requiring blood transfusion decreased by 4 times (from 39 to 9 %). Mean platelet levels normalized in most patients with baseline high and low platelet levels. A complete clinical and hematological response was achieved in 16 % (n = 23) of cases, a partial response – in 26 % (n = 37) of cases, clinical improvement","PeriodicalId":36905,"journal":{"name":"Klinicheskaya Onkogematologiya/Clinical Oncohematology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87844319","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-11-06DOI: 10.17650/1818-8346-2022-17-4-38-47
O. Rukavitsyn, V. Pop, M. V. Drozd, Yu. E. Ryabukhina
Understanding the molecular biological basis of chronic lymphocytic leukemia (CLL) pathogenesis and stratification of patients into risk groups has now led to significant advances in treatment. New targeted drugs with different mechanisms of action (bruton’s tyrosine kinase inhibitors, bCL-2 inhibitors, pI3K inhibitors) have significantly improved the prognosis of high-risk CLL patients. In some CLL cases the nodular tumor component can change to a more aggressive subtype of lymphoma (often diffuse large b-cell) with preservation of the small-cell leukemic component with the CLL phenotype (Richter’s syndrome), usually characterized by rapid progression and poor prognosis. The issue of treatment efficacy in patients with Richter’s syndrome still remains unresolved. The results of new drugs clinical trials are often contradictory and cannot yet be recommended for routine use in clinical practice. The low incidence of Richter’s syndrome, the lack of a unified view of the pathogenesis and therapy approaches make the search for effective drugs an urgent task, so each clinical observation is of undoubted interest.A clinical case of CLL patient with unfavorable molecular cytogenetic risk and transformation into diffuse large b-cell lymphoma (Richter’s syndrome) is presented. The combined use of bCL-2 inhibitors (venetoclax) and pI3K (duvelisib) led to the achievement of partial remission followed by a gradual increase in the positive antitumor effect.
{"title":"A clinical case of the effective combined use of BCL-2 and PI3K inhibitors in the treatment of a patient with an unfavorable chronic lymphocytic leukemia with transformation into diffuse large B-cell lymphoma (Richter’s syndrome)","authors":"O. Rukavitsyn, V. Pop, M. V. Drozd, Yu. E. Ryabukhina","doi":"10.17650/1818-8346-2022-17-4-38-47","DOIUrl":"https://doi.org/10.17650/1818-8346-2022-17-4-38-47","url":null,"abstract":"Understanding the molecular biological basis of chronic lymphocytic leukemia (CLL) pathogenesis and stratification of patients into risk groups has now led to significant advances in treatment. New targeted drugs with different mechanisms of action (bruton’s tyrosine kinase inhibitors, bCL-2 inhibitors, pI3K inhibitors) have significantly improved the prognosis of high-risk CLL patients. In some CLL cases the nodular tumor component can change to a more aggressive subtype of lymphoma (often diffuse large b-cell) with preservation of the small-cell leukemic component with the CLL phenotype (Richter’s syndrome), usually characterized by rapid progression and poor prognosis. The issue of treatment efficacy in patients with Richter’s syndrome still remains unresolved. The results of new drugs clinical trials are often contradictory and cannot yet be recommended for routine use in clinical practice. The low incidence of Richter’s syndrome, the lack of a unified view of the pathogenesis and therapy approaches make the search for effective drugs an urgent task, so each clinical observation is of undoubted interest.A clinical case of CLL patient with unfavorable molecular cytogenetic risk and transformation into diffuse large b-cell lymphoma (Richter’s syndrome) is presented. The combined use of bCL-2 inhibitors (venetoclax) and pI3K (duvelisib) led to the achievement of partial remission followed by a gradual increase in the positive antitumor effect.","PeriodicalId":36905,"journal":{"name":"Klinicheskaya Onkogematologiya/Clinical Oncohematology","volume":"40 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86835009","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-11-06DOI: 10.17650/1818-8346-2022-17-4-60-66
A. Melikyan, I. Subortseva
.
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{"title":"Resolution of the Expert Council on the issues of diagnosis and treatment of myeloproliferative neoplasms, existing standards and the possibility of their implementation in real clinical practice in Russia","authors":"A. Melikyan, I. Subortseva","doi":"10.17650/1818-8346-2022-17-4-60-66","DOIUrl":"https://doi.org/10.17650/1818-8346-2022-17-4-60-66","url":null,"abstract":"<jats:p>.</jats:p>","PeriodicalId":36905,"journal":{"name":"Klinicheskaya Onkogematologiya/Clinical Oncohematology","volume":"21 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86938094","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-11-06DOI: 10.17650/1818-8346-2022-17-4-81-87
N. Guskova, O. Selyutina, I. Lysenko, E. A. Guskova, A. Donskaya, N. Samaneva, E. Kapuza, T. F. Pushkareva
A complex clinical case of acute myelomonocytic leukemia with extramedullary lesion of the testis is presented. patient yu., born in 1968, applied to the National Medical Research Centre for Oncology (Rostov-on-don) after an injury to the inguinal region. ultrasound was performed: in the right testicle in the middle third, a mass of 30 × 23 × 16 mm was revealed. A biopsy was performed: the morphological picture is characteristic of a typical seminoma. Orchofuniculectomy was performed on the right. Histopathological conclusion: the morphological picture is more characteristic of a typical seminoma, but does not allow excluding lymphoma. In order to differentiate between a germ cell tumor and a lymphoproliferative disease, an immunohistochemical study of the tumor tissue, a morphological and immunophenotypic study of the bone marrow were performed. According to the immunohistochemical data, the morphological picture and immunophenotype of tumor cells are characteristic of extranodal NK/T-cell lymphoma of the testis with Cd4 co-expression. However, according to the myelogram data, 20 % of morphologically heterogeneous blast cells were found: with round or bean-shaped nuclei, delicate mesh structure of chromatin, 1–2 nucleoli and a monocytoid form of nuclei with indistinct nucleoli. The cytoplasm of varying basophilia degrees, vacuolized, with delicate azurophilic granularity. The content of the monocytoid population was increased (19 %), represented mainly by promonocytes, which corresponds to acute myelomonocytic leukemia. According to flow cytometry, the immunophenotype of blast cells corresponds to acute myeloid leukemia with Cd56 co-expression. In connection with the new data obtained, the histological preparation was revised again with the expansion of the immunohistochemical study. Result: morphological picture and immunophenotype of tumor cells are characteristic of acute myelomonocytic leukemia with extramedullary lesions of the right testicular tissue. final diagnosis: acute myelomonocytic leukemia with extramedullary lesion of the right testicle, with Cd56 co-expression. The presented clinical case showed the need to use a wide range of diagnostic techniques to determine the nature of the disease. The results of morphological and cytometric studies of the bone marrow were decisive in establishing the diagnosis of M4 acute myeloid leukemia with extramedullary lesions of the right testicle in this patient.
{"title":"Diagnosis of acute myelomonocytic leukemia in a patient with extramedullary testicular lesion","authors":"N. Guskova, O. Selyutina, I. Lysenko, E. A. Guskova, A. Donskaya, N. Samaneva, E. Kapuza, T. F. Pushkareva","doi":"10.17650/1818-8346-2022-17-4-81-87","DOIUrl":"https://doi.org/10.17650/1818-8346-2022-17-4-81-87","url":null,"abstract":"A complex clinical case of acute myelomonocytic leukemia with extramedullary lesion of the testis is presented. patient yu., born in 1968, applied to the National Medical Research Centre for Oncology (Rostov-on-don) after an injury to the inguinal region. ultrasound was performed: in the right testicle in the middle third, a mass of 30 × 23 × 16 mm was revealed. A biopsy was performed: the morphological picture is characteristic of a typical seminoma. Orchofuniculectomy was performed on the right. Histopathological conclusion: the morphological picture is more characteristic of a typical seminoma, but does not allow excluding lymphoma. In order to differentiate between a germ cell tumor and a lymphoproliferative disease, an immunohistochemical study of the tumor tissue, a morphological and immunophenotypic study of the bone marrow were performed. According to the immunohistochemical data, the morphological picture and immunophenotype of tumor cells are characteristic of extranodal NK/T-cell lymphoma of the testis with Cd4 co-expression. However, according to the myelogram data, 20 % of morphologically heterogeneous blast cells were found: with round or bean-shaped nuclei, delicate mesh structure of chromatin, 1–2 nucleoli and a monocytoid form of nuclei with indistinct nucleoli. The cytoplasm of varying basophilia degrees, vacuolized, with delicate azurophilic granularity. The content of the monocytoid population was increased (19 %), represented mainly by promonocytes, which corresponds to acute myelomonocytic leukemia. According to flow cytometry, the immunophenotype of blast cells corresponds to acute myeloid leukemia with Cd56 co-expression. In connection with the new data obtained, the histological preparation was revised again with the expansion of the immunohistochemical study. Result: morphological picture and immunophenotype of tumor cells are characteristic of acute myelomonocytic leukemia with extramedullary lesions of the right testicular tissue. final diagnosis: acute myelomonocytic leukemia with extramedullary lesion of the right testicle, with Cd56 co-expression. The presented clinical case showed the need to use a wide range of diagnostic techniques to determine the nature of the disease. The results of morphological and cytometric studies of the bone marrow were decisive in establishing the diagnosis of M4 acute myeloid leukemia with extramedullary lesions of the right testicle in this patient.","PeriodicalId":36905,"journal":{"name":"Klinicheskaya Onkogematologiya/Clinical Oncohematology","volume":"27 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78951364","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-11-06DOI: 10.17650/1818-8346-2022-17-4-67-80
M. V. Firsova, N. Risinskaya, M. Solovev, T. Obukhova, M. Kislitsyna, E. Nikulina, I. Yakutik, T. Abramova, A. Sudarikov, A. Kovrigina, L. Mendeleeva
Background. Multiple myeloma complicated by extramedullary plasmacytoma is an unfavorable variant of the disease. It remains unknown what triggers tumor transformation. The review presents literature data on the pathogenesis of extramedullary disease, as well as a clinical example of a comprehensive study of the tumor substrate.Aim. To study the molecular and biological characteristics of the tumor substrate of the bone marrow and extramedullary plasmacytoma using various research methods.Materials and methods. A 55-year-old patient was admitted to National Medical Research Center for Hematology with a diagnosis of multiple myeloma occurring with extramedullary plasmacytoma of the retroperitoneal space. dNA was isolated from samples of different localization (blood plasma, Cd138+ bone marrow cells, plasmacytoma and buccal epithelial cells). The profile of short tandem dNA repeats (STR) from the obtained samples was studied by multiplex polymerase chain reaction followed by fragment analysis. fluorescent in situ hybridization (fISH) of bone marrow Cd138+ cells was performed using various dNA probes. Comparative genomic hybridization on a microarray (arrayCGH) plasmacytoma dNA was also performed. The mutation profile of the KRAS, NRAS, BRAF genes was studied by Sanger sequencing in tumor samples of various localizations.Results. The induction therapy (vCd (bortezomib + cyclophosphamide + dexamethasone), vRd (bortezomib + lenalidomide + dexamethasone), daratumumab therapy) was ineffective, death occurred 4 months after the first clinical manifestations appeared. Comparison of STR markers of circulating cell-free tumor dNA (cfdNA), Cd138+ bone marrow cells, and plasmacytoma revealed the largest number of involved loci exactly in plasmacytoma’ dNA. A mutation in the NRAS gene was found only in plasmacytoma’ dNA. This indicates the presence of another clone of tumor cells in the extra-medullary plasmacytoma. Molecular karyotyping of plasmacytoma using the arrayCGH method revealed rearrangements of many chromosomes. 1p32.3 bi-allelic deletion, amplification of 1q21, 8q24/MyC rearrangements and del17p13 were confirmed by arrayCGH molecular karyotyping and fISH studies in bone marrow and plasmacytoma.Conclusion. A comprehensive molecular genetic study of the extramedullary plasmacytoma’ substrate is necessary to understand the pathogenesis mechanisms and, on this basis, to develop differentiated therapeutic approaches.
{"title":"Multiple myeloma with extramedullary plasmacytoma: pathogenesis and clinical case","authors":"M. V. Firsova, N. Risinskaya, M. Solovev, T. Obukhova, M. Kislitsyna, E. Nikulina, I. Yakutik, T. Abramova, A. Sudarikov, A. Kovrigina, L. Mendeleeva","doi":"10.17650/1818-8346-2022-17-4-67-80","DOIUrl":"https://doi.org/10.17650/1818-8346-2022-17-4-67-80","url":null,"abstract":"Background. Multiple myeloma complicated by extramedullary plasmacytoma is an unfavorable variant of the disease. It remains unknown what triggers tumor transformation. The review presents literature data on the pathogenesis of extramedullary disease, as well as a clinical example of a comprehensive study of the tumor substrate.Aim. To study the molecular and biological characteristics of the tumor substrate of the bone marrow and extramedullary plasmacytoma using various research methods.Materials and methods. A 55-year-old patient was admitted to National Medical Research Center for Hematology with a diagnosis of multiple myeloma occurring with extramedullary plasmacytoma of the retroperitoneal space. dNA was isolated from samples of different localization (blood plasma, Cd138+ bone marrow cells, plasmacytoma and buccal epithelial cells). The profile of short tandem dNA repeats (STR) from the obtained samples was studied by multiplex polymerase chain reaction followed by fragment analysis. fluorescent in situ hybridization (fISH) of bone marrow Cd138+ cells was performed using various dNA probes. Comparative genomic hybridization on a microarray (arrayCGH) plasmacytoma dNA was also performed. The mutation profile of the KRAS, NRAS, BRAF genes was studied by Sanger sequencing in tumor samples of various localizations.Results. The induction therapy (vCd (bortezomib + cyclophosphamide + dexamethasone), vRd (bortezomib + lenalidomide + dexamethasone), daratumumab therapy) was ineffective, death occurred 4 months after the first clinical manifestations appeared. Comparison of STR markers of circulating cell-free tumor dNA (cfdNA), Cd138+ bone marrow cells, and plasmacytoma revealed the largest number of involved loci exactly in plasmacytoma’ dNA. A mutation in the NRAS gene was found only in plasmacytoma’ dNA. This indicates the presence of another clone of tumor cells in the extra-medullary plasmacytoma. Molecular karyotyping of plasmacytoma using the arrayCGH method revealed rearrangements of many chromosomes. 1p32.3 bi-allelic deletion, amplification of 1q21, 8q24/MyC rearrangements and del17p13 were confirmed by arrayCGH molecular karyotyping and fISH studies in bone marrow and plasmacytoma.Conclusion. A comprehensive molecular genetic study of the extramedullary plasmacytoma’ substrate is necessary to understand the pathogenesis mechanisms and, on this basis, to develop differentiated therapeutic approaches.","PeriodicalId":36905,"journal":{"name":"Klinicheskaya Onkogematologiya/Clinical Oncohematology","volume":"12 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91176186","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-11-06DOI: 10.17650/1818-8346-2022-17-4-48-59
V. Potapenko, V. Baykov, А. Y. Markova, N. Mikhailova, A. Ter-Grigoryan, Y. Krivolapov
Kikuchi–Fujimoto disease, or necrotizing histiocytic lymphadenitis, is one of the rare causes of benign lymphadeno-pathy. The diagnosis is based on histological and immunohistochemical analysis of the lymph node biopsy. The article presents four clinical cases of Kikuchi–Fujimoto disease. According to the results of the primary analysis of lymph node tissue three patients were misdiagnosed with lymphoma. due to the unusual for lymphoid malignancy course the primary material was reviewed. The diagnosis of Kikuchi–Fujimoto disease was put. In three patients the disease has a re-current course. during the observation period, the course of the disease in all the presented patients is benign with normal quality of life.
{"title":"Kikuchi–Fujimoto disease: literature review and report of four cases","authors":"V. Potapenko, V. Baykov, А. Y. Markova, N. Mikhailova, A. Ter-Grigoryan, Y. Krivolapov","doi":"10.17650/1818-8346-2022-17-4-48-59","DOIUrl":"https://doi.org/10.17650/1818-8346-2022-17-4-48-59","url":null,"abstract":"Kikuchi–Fujimoto disease, or necrotizing histiocytic lymphadenitis, is one of the rare causes of benign lymphadeno-pathy. The diagnosis is based on histological and immunohistochemical analysis of the lymph node biopsy. The article presents four clinical cases of Kikuchi–Fujimoto disease. According to the results of the primary analysis of lymph node tissue three patients were misdiagnosed with lymphoma. due to the unusual for lymphoid malignancy course the primary material was reviewed. The diagnosis of Kikuchi–Fujimoto disease was put. In three patients the disease has a re-current course. during the observation period, the course of the disease in all the presented patients is benign with normal quality of life.","PeriodicalId":36905,"journal":{"name":"Klinicheskaya Onkogematologiya/Clinical Oncohematology","volume":"64 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80330882","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-11-06DOI: 10.17650/1818-8346-2022-17-4-88-93
T. Pavlova, T. Valiev
Chronic myeloid leukemia is a ph-positive myeloproliferative disease, which is usually manifested by hyperleukocytosis and massive splenomegaly. Chronic myeloid leukemia is rare in childhood and adolescence, it accounts for 2 to 3 % of all leukemias cases. priapism is a rare manifestation of chronic myeloid leukemia and is an urgent urological condition that requires timely treatment to prevent long-term complications, in particular, erectile dysfunction.This review presents the literature information about priapism as the first sign of chronic myeloid leukemia, as well as the first description in the Russian literature of a clinical case of priapism in a 9-year-old patient with chronic myeloid leukemia.
{"title":"Priapism as the first symptom of chronic myeloid leukemia: literature review and own clinical case report","authors":"T. Pavlova, T. Valiev","doi":"10.17650/1818-8346-2022-17-4-88-93","DOIUrl":"https://doi.org/10.17650/1818-8346-2022-17-4-88-93","url":null,"abstract":"Chronic myeloid leukemia is a ph-positive myeloproliferative disease, which is usually manifested by hyperleukocytosis and massive splenomegaly. Chronic myeloid leukemia is rare in childhood and adolescence, it accounts for 2 to 3 % of all leukemias cases. priapism is a rare manifestation of chronic myeloid leukemia and is an urgent urological condition that requires timely treatment to prevent long-term complications, in particular, erectile dysfunction.This review presents the literature information about priapism as the first sign of chronic myeloid leukemia, as well as the first description in the Russian literature of a clinical case of priapism in a 9-year-old patient with chronic myeloid leukemia.","PeriodicalId":36905,"journal":{"name":"Klinicheskaya Onkogematologiya/Clinical Oncohematology","volume":"467 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78560182","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-11-03DOI: 10.17650/1818-8346-2022-17-4-33-37
S. Zakharov, T. Mitina, R. Vardanyan, I. N. Kontievskiy, A. Faenko, Z. Tekeeva
Background. Idiopathic thrombocytopenic purpura (ITp) is an autoimmune disease characterized by antibody-mediated platelets destruction and impairment of their production, which manifests itself as: isolated thrombocytopenia, risk of spontaneous hemorrhage and bleeding of varying severity. ITp is a hematological, orphan disease with an incidence of 1–4 cases per 100,000 population. In modern literature, primary and secondary immune thrombocytopenias are distinguished. primary immune thrombocytopenia is a diagnosis of exclusion. To verify it, a certain diagnostic search is required.Aim. To evaluate clinical characteristics and treatment efficacy in patients with a confirmed primary immune thrombocytopenia in the Moscow region.Materials and methods. This article presents the results of an analysis of more than 2,400 outpatient records of patients diagnosed with thrombocytopenia (for the period from 2010 to 2022). Of these, about 400 confirmed clinical cases of various ITp forms were included in the ITp registry of the Moscow Region. All patients live in the Moscow region, receive treatment and are observed at the Center for Orphan diseases of the M.f. vladimirskiy Moscow Regional Research Clinical Institute.Results. There are 415 patients with a verified diagnosis of ITp in the register of the Moscow Region Center for Orphan diseases of the M.f. vladimirskiy Moscow Regional Research Clinical Institute (71 % (n = 294) are female). In 69.8 % (n = 290) of patients at the time of disease manifestation, hemorrhagic syndrome was recorded. As a first-line therapy, 92.8 % (n = 385) of patients received corticosteroids (prednisolone, methylprednisolone, dexamethasone), in the second-line therapy, 82 % (n = 340) of patients were recommended therapy with thrombopoietin receptor agonists (romiplostim, eltrombopag). The options for third-line therapy in patients with ITp are rituximab monotherapy, splenectomy, and intravenous immunoglobulin. Splenectomy was performed in 3.37 % (n = 14) of patients.Conclusion. when evaluating this register, the highest efficiency of thrombopoietin receptor agonists (romiplostim, eltrombopag) is observed – 84.1 % of the objective response.
{"title":"Management of patients with immune thrombocytopenia in the Moscow region","authors":"S. Zakharov, T. Mitina, R. Vardanyan, I. N. Kontievskiy, A. Faenko, Z. Tekeeva","doi":"10.17650/1818-8346-2022-17-4-33-37","DOIUrl":"https://doi.org/10.17650/1818-8346-2022-17-4-33-37","url":null,"abstract":"Background. Idiopathic thrombocytopenic purpura (ITp) is an autoimmune disease characterized by antibody-mediated platelets destruction and impairment of their production, which manifests itself as: isolated thrombocytopenia, risk of spontaneous hemorrhage and bleeding of varying severity. ITp is a hematological, orphan disease with an incidence of 1–4 cases per 100,000 population. In modern literature, primary and secondary immune thrombocytopenias are distinguished. primary immune thrombocytopenia is a diagnosis of exclusion. To verify it, a certain diagnostic search is required.Aim. To evaluate clinical characteristics and treatment efficacy in patients with a confirmed primary immune thrombocytopenia in the Moscow region.Materials and methods. This article presents the results of an analysis of more than 2,400 outpatient records of patients diagnosed with thrombocytopenia (for the period from 2010 to 2022). Of these, about 400 confirmed clinical cases of various ITp forms were included in the ITp registry of the Moscow Region. All patients live in the Moscow region, receive treatment and are observed at the Center for Orphan diseases of the M.f. vladimirskiy Moscow Regional Research Clinical Institute.Results. There are 415 patients with a verified diagnosis of ITp in the register of the Moscow Region Center for Orphan diseases of the M.f. vladimirskiy Moscow Regional Research Clinical Institute (71 % (n = 294) are female). In 69.8 % (n = 290) of patients at the time of disease manifestation, hemorrhagic syndrome was recorded. As a first-line therapy, 92.8 % (n = 385) of patients received corticosteroids (prednisolone, methylprednisolone, dexamethasone), in the second-line therapy, 82 % (n = 340) of patients were recommended therapy with thrombopoietin receptor agonists (romiplostim, eltrombopag). The options for third-line therapy in patients with ITp are rituximab monotherapy, splenectomy, and intravenous immunoglobulin. Splenectomy was performed in 3.37 % (n = 14) of patients.Conclusion. when evaluating this register, the highest efficiency of thrombopoietin receptor agonists (romiplostim, eltrombopag) is observed – 84.1 % of the objective response.","PeriodicalId":36905,"journal":{"name":"Klinicheskaya Onkogematologiya/Clinical Oncohematology","volume":"4 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75909580","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-11-03DOI: 10.17650/1818-8346-2022-17-4-16-32
V. Potapenko, V. Baykov, A. V. Zinchenko, N. Potikhonova
Langerhans cells histiocytosis is a variant of malignant histiocytosis. The course and symptoms vary. patients with localized forms have a better prognosis, because local therapy is effective. patients with multifocal forms of histiocytosis receive systemic drug therapy, which cures some of the patients. This review provides up-to-date data about typical presentation of the organ involvement, diagnosis, course and therapy of various forms of Langerhans cells histiocytosis.
{"title":"Langerhans cell histiocytosis in adults: literature review","authors":"V. Potapenko, V. Baykov, A. V. Zinchenko, N. Potikhonova","doi":"10.17650/1818-8346-2022-17-4-16-32","DOIUrl":"https://doi.org/10.17650/1818-8346-2022-17-4-16-32","url":null,"abstract":"Langerhans cells histiocytosis is a variant of malignant histiocytosis. The course and symptoms vary. patients with localized forms have a better prognosis, because local therapy is effective. patients with multifocal forms of histiocytosis receive systemic drug therapy, which cures some of the patients. This review provides up-to-date data about typical presentation of the organ involvement, diagnosis, course and therapy of various forms of Langerhans cells histiocytosis.","PeriodicalId":36905,"journal":{"name":"Klinicheskaya Onkogematologiya/Clinical Oncohematology","volume":"40 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73968124","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-07-20DOI: 10.17650/1818-8346-2022-17-3-127-136
L. Kogoniya, M. O. Rusanov, V. Shikina
Cardioncology has emerged as a new field at the intersection of cardiology and oncology. Despite the fact that improving efficiency of antitumor treatment increased the survival of oncological hematological patients, the long-term cardiovascular consequences of this treatment have become more clinically significant.Despite the effectiveness of modern methods of treatment, some drugs, such as Bcr-Abl kinase inhibitors, anthracyclines, HER2/Erbb2 inhibitors, vascular endothelial growth factor inhibitors, fluoropyrimidines, as well as radiation therapy can have a pronounced effect on the cardiovascular system. These toxic effects lead to cardiac arrhythmia, heart failure, vascular toxicity and even death. It is important for hematologists, oncologists and cardiologists to understand the basic diagnostic and treatment strategies that should be used in the event of toxicity of this kind. At a time when, due to the developed cardiotoxicity, antitumor therapy should be discontinued, in some cases, it is possible to consider continuing treatment with caution and careful monitoring.
{"title":"Cardiotoxicity of anticancer drugs and radiotherapy in patients with hematologic malignancies and solid tumors","authors":"L. Kogoniya, M. O. Rusanov, V. Shikina","doi":"10.17650/1818-8346-2022-17-3-127-136","DOIUrl":"https://doi.org/10.17650/1818-8346-2022-17-3-127-136","url":null,"abstract":"Cardioncology has emerged as a new field at the intersection of cardiology and oncology. Despite the fact that improving efficiency of antitumor treatment increased the survival of oncological hematological patients, the long-term cardiovascular consequences of this treatment have become more clinically significant.Despite the effectiveness of modern methods of treatment, some drugs, such as Bcr-Abl kinase inhibitors, anthracyclines, HER2/Erbb2 inhibitors, vascular endothelial growth factor inhibitors, fluoropyrimidines, as well as radiation therapy can have a pronounced effect on the cardiovascular system. These toxic effects lead to cardiac arrhythmia, heart failure, vascular toxicity and even death. It is important for hematologists, oncologists and cardiologists to understand the basic diagnostic and treatment strategies that should be used in the event of toxicity of this kind. At a time when, due to the developed cardiotoxicity, antitumor therapy should be discontinued, in some cases, it is possible to consider continuing treatment with caution and careful monitoring.","PeriodicalId":36905,"journal":{"name":"Klinicheskaya Onkogematologiya/Clinical Oncohematology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87042065","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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