Pub Date : 2022-11-14DOI: 10.3389/frhem.2022.967156
Shan He, X. Mao, Zhaoting Cheng, Xiaojian Zhu, M. Xiao, J. Zhou
Relapsed/refractory (R/R) mantle cell lymphoma (MCL) with primary drug resistance to Bruton tyrosine kinase inhibitor and mutated TP53 responds poorly to conventional treatments. Chimeric antigen receptor (CAR) T cell therapy has emerged as one of the most effective treatments for R/R B cell lymphoma. However, no reports of CD5 CAR T cell treatment for MCL have been reported. In this paper, we report a R/R MCL patient with primary drug resistance to BTK inhibitors and TP53 mutation enrolled in a human CD5 CAR T cell trial. Remission of the primary disease was observed half a month after CAR T cell infusion. However, ascites was observed 2 weeks later. Flow cytometry suggested disease progression and immunophenotypic transformation. CD5 in CAR T cells turned negative and the expression of CD38 was enhanced. The patient was treated with a combination of daratumumab and Gemox (gemcitabine + oxaliplatin), abdominal distension and pain were markedly reduced, and ascites disappeared. We report the first case of human CD5 CAR T cell treatment for a patient with R/R MCL, providing insight on treatment strategies for such patients.
复发/难治性(R/R)套细胞淋巴瘤(MCL)对布鲁顿酪氨酸激酶抑制剂和突变TP53的原发性耐药对常规治疗反应不佳。嵌合抗原受体(CAR) T细胞疗法已成为R/R B细胞淋巴瘤最有效的治疗方法之一。然而，目前还没有关于CD5 CAR - T细胞治疗MCL的报道。在这篇论文中，我们报道了一名原发性BTK抑制剂耐药和TP53突变的R/R MCL患者参加了一项人类CD5 CAR - T细胞试验。CAR - T细胞输注后半个月观察原发疾病的缓解。2周后出现腹水。流式细胞术提示疾病进展和免疫表型转化。CAR - T细胞中CD5转为阴性，CD38表达增强。患者经达拉单抗联合Gemox(吉西他滨+奥沙利铂)治疗，腹胀、疼痛明显减轻，腹水消失。我们报告了首例人类CD5 CAR - T细胞治疗R/R MCL患者的病例，为此类患者的治疗策略提供了见解。
{"title":"Immunophenotypic transformation in relapsed/refractory mantle cell lymphoma treated with human anti-CD5 chimeric antigen receptor T cells: A Case Report","authors":"Shan He, X. Mao, Zhaoting Cheng, Xiaojian Zhu, M. Xiao, J. Zhou","doi":"10.3389/frhem.2022.967156","DOIUrl":"https://doi.org/10.3389/frhem.2022.967156","url":null,"abstract":"Relapsed/refractory (R/R) mantle cell lymphoma (MCL) with primary drug resistance to Bruton tyrosine kinase inhibitor and mutated TP53 responds poorly to conventional treatments. Chimeric antigen receptor (CAR) T cell therapy has emerged as one of the most effective treatments for R/R B cell lymphoma. However, no reports of CD5 CAR T cell treatment for MCL have been reported. In this paper, we report a R/R MCL patient with primary drug resistance to BTK inhibitors and TP53 mutation enrolled in a human CD5 CAR T cell trial. Remission of the primary disease was observed half a month after CAR T cell infusion. However, ascites was observed 2 weeks later. Flow cytometry suggested disease progression and immunophenotypic transformation. CD5 in CAR T cells turned negative and the expression of CD38 was enhanced. The patient was treated with a combination of daratumumab and Gemox (gemcitabine + oxaliplatin), abdominal distension and pain were markedly reduced, and ascites disappeared. We report the first case of human CD5 CAR T cell treatment for a patient with R/R MCL, providing insight on treatment strategies for such patients.","PeriodicalId":101407,"journal":{"name":"Frontiers in hematology","volume":"92 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114711268","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: 10.3389/frhem.2022.1020852
Susu Duan, Yifan Dang, Gaurav Manohar Rajani, K. Kis‐Tóth, Joe Salas
Efmoroctocog alfa, a recombinant factor VIII Fc fusion protein referred to herein as rFVIIIFc, is an extended half-life factor replacement therapy approved for use in patients with hemophilia A. Previous studies have shown that rFVIIIFc has an immunoregulatory effect on monocyte-derived macrophages. This study provides novel findings and an understanding of how rFVIIIFc modulates monocyte differentiation into osteoclasts. rFVIIIFc was found to engage with Fc-gamma receptors (FcγR) on the monocyte surface, leading to increased inhibitory FcγR signaling in cells. Monocyte differentiation into osteoclasts in vitro was inhibited in a concentration-dependent manner following rFVIIIFc treatment, with the interaction between the Fc domain of rFVIIIFc and FcγRII on monocytes playing a role in this effect. The C1 and C2 domains of rFVIIIFc were also found to play a role in inhibiting osteoclast formation. rFVIIIFc treatment of monocytes skewed their differentiation from osteoclasts into a group of less differentiated monocytes with unique myeloid cell phenotypes. The results of this study suggest that rFVIIIFc has a unique immune-regulatory effect on monocyte differentiation, inhibiting osteoclast formation. We propose a “double touchpoint” model for rFVIIIFc interaction with monocytes, with both the Fc domain and domains of FVIII binding to the monocyte surface. Further study is needed to determine if this immune-regulatory effect has any potential benefit on the bone and joint health of patients with hemophilia A receiving rFVIIIFc.
{"title":"Recombinant factor VIII Fc fusion protein engages monocytes via Fc and FVIII domains to reduce monocyte differentiation into osteoclasts","authors":"Susu Duan, Yifan Dang, Gaurav Manohar Rajani, K. Kis‐Tóth, Joe Salas","doi":"10.3389/frhem.2022.1020852","DOIUrl":"https://doi.org/10.3389/frhem.2022.1020852","url":null,"abstract":"Efmoroctocog alfa, a recombinant factor VIII Fc fusion protein referred to herein as rFVIIIFc, is an extended half-life factor replacement therapy approved for use in patients with hemophilia A. Previous studies have shown that rFVIIIFc has an immunoregulatory effect on monocyte-derived macrophages. This study provides novel findings and an understanding of how rFVIIIFc modulates monocyte differentiation into osteoclasts. rFVIIIFc was found to engage with Fc-gamma receptors (FcγR) on the monocyte surface, leading to increased inhibitory FcγR signaling in cells. Monocyte differentiation into osteoclasts in vitro was inhibited in a concentration-dependent manner following rFVIIIFc treatment, with the interaction between the Fc domain of rFVIIIFc and FcγRII on monocytes playing a role in this effect. The C1 and C2 domains of rFVIIIFc were also found to play a role in inhibiting osteoclast formation. rFVIIIFc treatment of monocytes skewed their differentiation from osteoclasts into a group of less differentiated monocytes with unique myeloid cell phenotypes. The results of this study suggest that rFVIIIFc has a unique immune-regulatory effect on monocyte differentiation, inhibiting osteoclast formation. We propose a “double touchpoint” model for rFVIIIFc interaction with monocytes, with both the Fc domain and domains of FVIII binding to the monocyte surface. Further study is needed to determine if this immune-regulatory effect has any potential benefit on the bone and joint health of patients with hemophilia A receiving rFVIIIFc.","PeriodicalId":101407,"journal":{"name":"Frontiers in hematology","volume":"9 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127830947","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 : 2022-10-26DOI: 10.3389/frhem.2022.1005666
M. Egyed, B. Kajtár, C. Foldesi, V. Skov, L. Kjær, H. Hasselbalch
The JAK2V617F mutation is an acquired somatic mutation, which is prevalent in patients with the Philadelphia-chromosome negative myeloproliferative neoplasms (MPNs). In these diseases the mutation gives rise to constitutive JAK-STAT signaling with increased blood cell counts and in vivo activation of neutrophils and platelets as well, which altogether contribute to a chronic inflammatory and thrombogenic state with a 12-fold increased risk of coronary disease. Treatment with recombinant interferon-alpha2 (rIFN) reduces the JAK2V617F allelic burden in a large number of MPN-patients. Long-term treatment with rIFN associates with low-burden JAK2V617F in a subset of patients and a decreased thrombosis risk as well. In the general population the JAK2V617F mutation has been shown to associate with ischemic heart disease and thrombosis. Based upon the above observations we herein report the first patient with CHIP-JAK2V617F, in whom treatment with rIFN resolved severe angina pectoris. During a short period off rIFN the symptoms reappeared to resolve in concert with reduction of JAK2V617F allele burden, when rIFN was reinstituted. The JAK2V617F mutation may be a novel therapeutic target to prohibit the development of cardiovascular diseases using rIFN either as monotherapy or in combination with potent anti-inflammatory agents.
{"title":"Ropeginterferon-alfa2b resolves angina pectoris and reduces JAK2V617F in a patient with clonal hematopoiesis of indeterminate potential: A case report","authors":"M. Egyed, B. Kajtár, C. Foldesi, V. Skov, L. Kjær, H. Hasselbalch","doi":"10.3389/frhem.2022.1005666","DOIUrl":"https://doi.org/10.3389/frhem.2022.1005666","url":null,"abstract":"The JAK2V617F mutation is an acquired somatic mutation, which is prevalent in patients with the Philadelphia-chromosome negative myeloproliferative neoplasms (MPNs). In these diseases the mutation gives rise to constitutive JAK-STAT signaling with increased blood cell counts and in vivo activation of neutrophils and platelets as well, which altogether contribute to a chronic inflammatory and thrombogenic state with a 12-fold increased risk of coronary disease. Treatment with recombinant interferon-alpha2 (rIFN) reduces the JAK2V617F allelic burden in a large number of MPN-patients. Long-term treatment with rIFN associates with low-burden JAK2V617F in a subset of patients and a decreased thrombosis risk as well. In the general population the JAK2V617F mutation has been shown to associate with ischemic heart disease and thrombosis. Based upon the above observations we herein report the first patient with CHIP-JAK2V617F, in whom treatment with rIFN resolved severe angina pectoris. During a short period off rIFN the symptoms reappeared to resolve in concert with reduction of JAK2V617F allele burden, when rIFN was reinstituted. The JAK2V617F mutation may be a novel therapeutic target to prohibit the development of cardiovascular diseases using rIFN either as monotherapy or in combination with potent anti-inflammatory agents.","PeriodicalId":101407,"journal":{"name":"Frontiers in hematology","volume":"144 11 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129525152","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 : 2022-10-20DOI: 10.3389/frhem.2022.1035281
Marianna Palazzo, Gaia Ciolli, S. Pilerci, I. Attucci, L. Pengue, A. Vannucchi, E. Antonioli
Multiple myeloma patients have an increased risk of infections due to both the inherent nature of the disease and ongoing treatment. We describe the case of a patient who was treated with daratumumab-lenalidomide-dexamethasone regimen for two years and developed a parvovirus B19 infection. The clinical picture, characterized by trilinear cytopenia, was initially attributed to anti-neoplastic treatment. Later on, when the patient’s condition worsened, an extensive diagnostic workup was applied and parvovirus B19 infection was detected by PCR. Due to the lack of effective antiviral drugs, the patient received IV immunoglobulins and it took 10 days to achieve a decrease in viral copies. Physicians should be aware that recent changes in the therapeutic scenario of multiple myeloma would make patients more susceptible to atypical infections in this patient setting.
{"title":"Case report: Unexpected parvovirus B19 infection in a myeloma patient treated with daratumumab","authors":"Marianna Palazzo, Gaia Ciolli, S. Pilerci, I. Attucci, L. Pengue, A. Vannucchi, E. Antonioli","doi":"10.3389/frhem.2022.1035281","DOIUrl":"https://doi.org/10.3389/frhem.2022.1035281","url":null,"abstract":"Multiple myeloma patients have an increased risk of infections due to both the inherent nature of the disease and ongoing treatment. We describe the case of a patient who was treated with daratumumab-lenalidomide-dexamethasone regimen for two years and developed a parvovirus B19 infection. The clinical picture, characterized by trilinear cytopenia, was initially attributed to anti-neoplastic treatment. Later on, when the patient’s condition worsened, an extensive diagnostic workup was applied and parvovirus B19 infection was detected by PCR. Due to the lack of effective antiviral drugs, the patient received IV immunoglobulins and it took 10 days to achieve a decrease in viral copies. Physicians should be aware that recent changes in the therapeutic scenario of multiple myeloma would make patients more susceptible to atypical infections in this patient setting.","PeriodicalId":101407,"journal":{"name":"Frontiers in hematology","volume":"13 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127742937","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 : 2022-10-19DOI: 10.3389/frhem.2022.1004717
Morgane Canonne, Fabienne George, C. Graux
Mature B cell malignancies constitute a wide range of biologically and clinically heterogeneous hematological diseases. Despite an increasingly thorough understanding of the pathophysiology of these pathologies and significant improvements in therapies, a dismal outcome still affects a large number of patients. Therefore, further investigations into new treatment perspectives are highly needed and they depend entirely on the ex vivo culture of patient cells. Primary cells usually demand superior culture models, as they are notoriously difficult to cultivate. The literature is not devoid of approaches ranging from two- to three-dimensional systems for culturing mature malignant primary B cells. However, they display substantial protocol inter-variation. This imposes a high risk of failures, repeats, and inconsistent results, which are neither compatible with the rare value of primary cells nor the efficiency of the drug discovery process. In this review, we provide a thorough overview of the different approaches that have been implemented in the literature for the culture of mature malignant primary B cells, and we discuss associated considerations and limitations to assist researchers in determining a fit-for-purpose culture system, thereby attempting to reduce the number of trials and errors as well as associated biomaterial expenditure.
{"title":"Ex vivo culture of malignant primary B cells","authors":"Morgane Canonne, Fabienne George, C. Graux","doi":"10.3389/frhem.2022.1004717","DOIUrl":"https://doi.org/10.3389/frhem.2022.1004717","url":null,"abstract":"Mature B cell malignancies constitute a wide range of biologically and clinically heterogeneous hematological diseases. Despite an increasingly thorough understanding of the pathophysiology of these pathologies and significant improvements in therapies, a dismal outcome still affects a large number of patients. Therefore, further investigations into new treatment perspectives are highly needed and they depend entirely on the ex vivo culture of patient cells. Primary cells usually demand superior culture models, as they are notoriously difficult to cultivate. The literature is not devoid of approaches ranging from two- to three-dimensional systems for culturing mature malignant primary B cells. However, they display substantial protocol inter-variation. This imposes a high risk of failures, repeats, and inconsistent results, which are neither compatible with the rare value of primary cells nor the efficiency of the drug discovery process. In this review, we provide a thorough overview of the different approaches that have been implemented in the literature for the culture of mature malignant primary B cells, and we discuss associated considerations and limitations to assist researchers in determining a fit-for-purpose culture system, thereby attempting to reduce the number of trials and errors as well as associated biomaterial expenditure.","PeriodicalId":101407,"journal":{"name":"Frontiers in hematology","volume":"72 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124253456","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 : 2022-10-11DOI: 10.3389/frhem.2022.997262
Borja Puertas, Beatriz Rey-Búa, R. Eiros, Alberto Martín-Arribas, E. Montero-Mateos, A. Hernandez-Sanchez, S. Gómez-Úbeda, Elena Alejo-Alonso, Ana A. Martín-López, P. Antunez, Eduardo Villacorta-Argüelles, M. Gallego-Delgado, N. Puig, M. Mateos, V. González-Calle
There is no recommendation on what salvage therapy is optimal in the solid organ recipient with AL amyloidosis, such as a heart transplant. With this case, we illustrate how treatment with daratumumab may be effective and safe in a patient with AL amyloidosis with renal involvement at the relapse after heart transplantation and autologous stem cell transplantation.
{"title":"Case Report: Renal relapse after heart transplantation, induction, and autologous stem cell transplantation in a patient with AL amyloidosis with exclusive cardiac involvement","authors":"Borja Puertas, Beatriz Rey-Búa, R. Eiros, Alberto Martín-Arribas, E. Montero-Mateos, A. Hernandez-Sanchez, S. Gómez-Úbeda, Elena Alejo-Alonso, Ana A. Martín-López, P. Antunez, Eduardo Villacorta-Argüelles, M. Gallego-Delgado, N. Puig, M. Mateos, V. González-Calle","doi":"10.3389/frhem.2022.997262","DOIUrl":"https://doi.org/10.3389/frhem.2022.997262","url":null,"abstract":"There is no recommendation on what salvage therapy is optimal in the solid organ recipient with AL amyloidosis, such as a heart transplant. With this case, we illustrate how treatment with daratumumab may be effective and safe in a patient with AL amyloidosis with renal involvement at the relapse after heart transplantation and autologous stem cell transplantation.","PeriodicalId":101407,"journal":{"name":"Frontiers in hematology","volume":"14 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129291282","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 : 2022-10-10DOI: 10.3389/frhem.2022.1008363
Marwa Elsayed, Haitham Abdelhakim, L. Shune, S. Abhyankar, Anurag K. Singh, Da Zhang, J. McGuirk, O. Aljitawi
The utilization of hematopoietic stem cell transplantation (HSCT) has been rapidly growing due to multiple factors, including better availability of donors and improved supportive care. Hyperbaric oxygen has been associated with the improvement of hematopoietic stem cell (HSC) homing at the time of transplant through lowering erythropoietin levels in preclinical studies. We studied the role of hyperbaric oxygen (HBO) in the enhancement of engraftment of HSC when utilized prior to umbilical cord HSCT and autologous HSCT in two pilot clinical trials with excellent safety profiles. In these two pilot studies, we observed an uncommon phenomenon post-transplant, particularly a significant peripheral blood lymphocytosis and lymphocyte infiltration of different tissues in 3/34 of HBO-treated patients. This peripheral blood lymphocyte expansion was associated with various clinical manifestations that can be confused with infections, inflammatory conditions, or disease relapse. We hypothesize that this observation is related to different immune reconstitution dynamics related to the use of HBO. While the incidence is ~9%, this may have implications as HBO is being investigated in larger clinical trials. This case series highlights the clinical presentation, course, outcome, and potential implications of this significant rise in lymphocytes when utilizing HBO before HSCT.
{"title":"Case Report: Unique patterns of lymphocyte recovery post-hematopoietic stem cell transplant associated with hyperbaric oxygen therapy: A case series","authors":"Marwa Elsayed, Haitham Abdelhakim, L. Shune, S. Abhyankar, Anurag K. Singh, Da Zhang, J. McGuirk, O. Aljitawi","doi":"10.3389/frhem.2022.1008363","DOIUrl":"https://doi.org/10.3389/frhem.2022.1008363","url":null,"abstract":"The utilization of hematopoietic stem cell transplantation (HSCT) has been rapidly growing due to multiple factors, including better availability of donors and improved supportive care. Hyperbaric oxygen has been associated with the improvement of hematopoietic stem cell (HSC) homing at the time of transplant through lowering erythropoietin levels in preclinical studies. We studied the role of hyperbaric oxygen (HBO) in the enhancement of engraftment of HSC when utilized prior to umbilical cord HSCT and autologous HSCT in two pilot clinical trials with excellent safety profiles. In these two pilot studies, we observed an uncommon phenomenon post-transplant, particularly a significant peripheral blood lymphocytosis and lymphocyte infiltration of different tissues in 3/34 of HBO-treated patients. This peripheral blood lymphocyte expansion was associated with various clinical manifestations that can be confused with infections, inflammatory conditions, or disease relapse. We hypothesize that this observation is related to different immune reconstitution dynamics related to the use of HBO. While the incidence is ~9%, this may have implications as HBO is being investigated in larger clinical trials. This case series highlights the clinical presentation, course, outcome, and potential implications of this significant rise in lymphocytes when utilizing HBO before HSCT.","PeriodicalId":101407,"journal":{"name":"Frontiers in hematology","volume":"57 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134302936","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 : 2022-10-06DOI: 10.3389/frhem.2022.983424
Yueh-Shih Chang, Jung-Jr Ye, Tzu-Chien Cheng, Yingfen Wen, Chi-Ying F. Huang, K. Yeh
Viral reactivation was previously reported after severe acute respiratory syndrome coronavirus‐2 (SARS-CoV-2) infection but was seldom documented after SARS-CoV-2 vaccination, except varicella-zoster virus and cytomegalovirus. Here, we present a case of reactive Epstein–Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) and thrombosis with thrombocytopenia syndrome after receiving SARS-CoV-2 mRNA vaccination. Antiplatelet factor 4 antibody was detected, and the bone marrow study showed hemophagocytosis and was positive in the immunohistochemistry staining for EBV-encoded small nuclear RNAs and negative staining for CD3 and CD56 markers of small lymphocytes. The high percentage of CD38 high/HLA-DR+ cells among CD8+ T cells further confirmed HLH. After intravenous administration of immunoglobulin, the clinical symptoms, D-dimer level, fibrinogen, platelet count, EBV-DNA titer, and anti-PF4 level were all improved. Further investigation into the pathogenesis of vaccine-associated EBV reactivation, such as TNF-α, interleukin-1β (IL-1β), and interleukin-6 (IL-6), is warranted.
{"title":"Case report: Reactive Epstein–Barr virus-associated hemophagocytic lymphohistiocytosis and thrombosis with thrombocytopenia syndrome following SARS-CoV-2 vaccination and treated with intravenous immunoglobulin","authors":"Yueh-Shih Chang, Jung-Jr Ye, Tzu-Chien Cheng, Yingfen Wen, Chi-Ying F. Huang, K. Yeh","doi":"10.3389/frhem.2022.983424","DOIUrl":"https://doi.org/10.3389/frhem.2022.983424","url":null,"abstract":"Viral reactivation was previously reported after severe acute respiratory syndrome coronavirus‐2 (SARS-CoV-2) infection but was seldom documented after SARS-CoV-2 vaccination, except varicella-zoster virus and cytomegalovirus. Here, we present a case of reactive Epstein–Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) and thrombosis with thrombocytopenia syndrome after receiving SARS-CoV-2 mRNA vaccination. Antiplatelet factor 4 antibody was detected, and the bone marrow study showed hemophagocytosis and was positive in the immunohistochemistry staining for EBV-encoded small nuclear RNAs and negative staining for CD3 and CD56 markers of small lymphocytes. The high percentage of CD38 high/HLA-DR+ cells among CD8+ T cells further confirmed HLH. After intravenous administration of immunoglobulin, the clinical symptoms, D-dimer level, fibrinogen, platelet count, EBV-DNA titer, and anti-PF4 level were all improved. Further investigation into the pathogenesis of vaccine-associated EBV reactivation, such as TNF-α, interleukin-1β (IL-1β), and interleukin-6 (IL-6), is warranted.","PeriodicalId":101407,"journal":{"name":"Frontiers in hematology","volume":"70 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123223490","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 : 2022-09-16DOI: 10.3389/frhem.2022.974392
T. Troiano, V. Brescia, Lucia De Marinis, A. Marinaccio, R. Lovero, R. Rizzi, F. Di Serio
Background IgD Multiple Myeloma is a rare form of plasma cell dyscrasia and accounts for approximately 1-2% of all cases of Multiple Myeloma. It mainly affects young, male subjects; it is characterized by an aggressive course, a high production of Bence Jones protein, acute renal failure and an often unfortunate outcome compared to the other isotypes of MM. A distinctive feature is the lack of a monoclonal peak on serum protein electrophoresis (SPE). Case report a 57-year-old man with pain in his left lower limb and weight loss goes to the Emergency Department (Emergency Department). Laboratory tests performed showed normocytic normochromic anemia (Hemoglobin 9.4 g/dL), acute renal failure (s-creatinine 2.85 mg/dL, e-GFR 23 mL/min/1.73 m². serum protein electrophoresis (SPE) detected only mild polyclonal in the gamma zone with no evidence of any monoclonal peak. Results serum immunofixation (s-IFE) showed a monoclonal IgD λ band and a monoclonal λ band. The Free Light Chains (s-FLC) measurement showed a ratio of 0.04. The bone marrow biopsy confirmed an infiltration of> 20% of clonal plasma cells; renal biopsy diagnosed “cast nephropathy”. Conclusion IgD λ/λ Multiple Myeloma is a rare form of this disease with a poor prognosis; an early and correct laboratory diagnosis is crucial for appropriate treatment and effective monitoring in order to improve patient outcome.
{"title":"Case Report: A case of IgD lambda/lambda Multiple Myeloma in patient with acute renal failure and without monoclonal spike in serum electrophoresis","authors":"T. Troiano, V. Brescia, Lucia De Marinis, A. Marinaccio, R. Lovero, R. Rizzi, F. Di Serio","doi":"10.3389/frhem.2022.974392","DOIUrl":"https://doi.org/10.3389/frhem.2022.974392","url":null,"abstract":"Background IgD Multiple Myeloma is a rare form of plasma cell dyscrasia and accounts for approximately 1-2% of all cases of Multiple Myeloma. It mainly affects young, male subjects; it is characterized by an aggressive course, a high production of Bence Jones protein, acute renal failure and an often unfortunate outcome compared to the other isotypes of MM. A distinctive feature is the lack of a monoclonal peak on serum protein electrophoresis (SPE). Case report a 57-year-old man with pain in his left lower limb and weight loss goes to the Emergency Department (Emergency Department). Laboratory tests performed showed normocytic normochromic anemia (Hemoglobin 9.4 g/dL), acute renal failure (s-creatinine 2.85 mg/dL, e-GFR 23 mL/min/1.73 m². serum protein electrophoresis (SPE) detected only mild polyclonal in the gamma zone with no evidence of any monoclonal peak. Results serum immunofixation (s-IFE) showed a monoclonal IgD λ band and a monoclonal λ band. The Free Light Chains (s-FLC) measurement showed a ratio of 0.04. The bone marrow biopsy confirmed an infiltration of> 20% of clonal plasma cells; renal biopsy diagnosed “cast nephropathy”. Conclusion IgD λ/λ Multiple Myeloma is a rare form of this disease with a poor prognosis; an early and correct laboratory diagnosis is crucial for appropriate treatment and effective monitoring in order to improve patient outcome.","PeriodicalId":101407,"journal":{"name":"Frontiers in hematology","volume":"30 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127684241","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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