Hematology Reports最新文献_第4页

Morphological Clues of Acute Monocytic Leukemia in COVID-19-Induced Transient Leukoerythroblastic Reaction with Monocytosis. COVID-19 诱导的伴有单核细胞增多的一过性红细胞白血病反应中急性单核细胞白血病的形态学线索

IF 1.1

Hematology Reports Pub Date : 2024-05-28 DOI: 10.3390/hematolrep16020033

Ingrid S Tam, Mohamed Elemary, John DeCoteau, Anna Porwit, Emina E Torlakovic

{"title":"Morphological Clues of Acute Monocytic Leukemia in COVID-19-Induced Transient Leukoerythroblastic Reaction with Monocytosis.","authors":"Ingrid S Tam, Mohamed Elemary, John DeCoteau, Anna Porwit, Emina E Torlakovic","doi":"10.3390/hematolrep16020033","DOIUrl":"10.3390/hematolrep16020033","url":null,"abstract":"Viral infections, including those caused by COVID-19, can produce striking morphologic changes in peripheral blood. Distinguishing between reactive changes and abnormal morphology of monocytes remains particularly difficult, with low consensus rates reported amongst hematopathologists. Here, we report a patient who developed transient monocytosis of 11.06 × 109/L with 32% promonocytes and 1% blasts during hospitalization that was secondary to severe COVID-19 infection. Three days later, the clinical status of the patient improved and the WBC had decreased to 8.47 × 109/L with 2.2 × 109/L monocytes. Flow cytometry studies did not reveal immunophenotypic findings specific for an overt malignant population. At no time during admission did the patient develop cytopenia(s), and she was discharged upon clinical improvement. However, the peripheral blood sample containing promonocytes was sent for molecular testing with an extended next-generation sequencing myeloid panel and was positive for pathogenic NPM1 Type A and DNMT3A R882H mutations. Subsequently, despite an essentially normal complete blood count, the patient underwent a bone marrow assessment that showed acute myeloid leukemia with 77% promonocytes. This case emphasizes the critical importance of a full work up to exclude acute leukemia when classical promonocyte morphology is encountered in the peripheral blood. Promonocytes are not a part of the reactive changes associated with COVID-19 and remain specific to myeloid neoplasia.","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11203109/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141450324","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}

引用次数: 0

Phase II Trial of Romidepsin as Consolidation Therapy after Gemcitabine, Dexamethasone, and Cisplatin in Elderly Transplant-Ineligible Patients with Relapsed/Refractory Peripheral T-Cell Lymphoma. 罗米地平作为吉西他滨、地塞米松和顺铂治疗后的巩固疗法，用于不符合移植条件的老年复发性/难治性外周T细胞淋巴瘤患者的II期试验。

IF 1.1

Hematology Reports Pub Date : 2024-05-28 DOI: 10.3390/hematolrep16020034

Satoshi Yamasaki, Hiroatsu Iida, Akio Saito, Morio Matsumoto, Yoshiaki Kuroda, Tohru Izumi, Akiko M Saito, Hiroaki Miyoshi, Koichi Ohshima, Hirokazu Nagai, Hiromi Iwasaki

{"title":"Phase II Trial of Romidepsin as Consolidation Therapy after Gemcitabine, Dexamethasone, and Cisplatin in Elderly Transplant-Ineligible Patients with Relapsed/Refractory Peripheral T-Cell Lymphoma.","authors":"Satoshi Yamasaki, Hiroatsu Iida, Akio Saito, Morio Matsumoto, Yoshiaki Kuroda, Tohru Izumi, Akiko M Saito, Hiroaki Miyoshi, Koichi Ohshima, Hirokazu Nagai, Hiromi Iwasaki","doi":"10.3390/hematolrep16020034","DOIUrl":"10.3390/hematolrep16020034","url":null,"abstract":"Romidepsin is an important therapeutic option for patients with peripheral T-cell lymphoma (PTCL). However, the timing of romidepsin administration remains controversial. The objective of this study was to characterize the safety and efficacy of romidepsin as consolidation therapy after gemcitabine, dexamethasone, and cisplatin (GDP) therapy (GDPR). This study of patients treated between March 2019 and March 2021 was registered with the Japan Registry of Clinical Trials (registration number: jRCT0000000519). If complete response, partial response, or stable disease was confirmed after 2-4 GDP cycles, romidepsin was administered every 4 weeks for 1 year. Seven patients with relapsed/refractory (R/R) PTCL (T-follicular helper phenotype [n = 1] and angioimmunoblastic T-cell lymphoma [n = 6]) were included in this prospective study (PTCL-GDPR). After a median follow-up of 34 months of patients in PTCL-GDPR, the 2-year overall survival rate was 71%, and the overall response rate after treatment was 57%. Common adverse events in patients with PTCL-GDPR included hematological toxicities such as neutropenia, which improved with supportive treatment. There were no treatment-related mortalities. GDPR might be safe and effective in elderly transplant-ineligible patients with R/R PTCL; however, further investigation is required.","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11204088/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141450326","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}

引用次数: 0

Appropriate Treatment Intensity for Diffuse Large B-Cell Lymphoma in the Older Population: A Review of the Literature. 老年弥漫性大 B 细胞淋巴瘤的适当治疗强度：文献综述。

IF 1.1

Hematology Reports Pub Date : 2024-05-24 DOI: 10.3390/hematolrep16020032

Satoshi Yamasaki

{"title":"Appropriate Treatment Intensity for Diffuse Large B-Cell Lymphoma in the Older Population: A Review of the Literature.","authors":"Satoshi Yamasaki","doi":"10.3390/hematolrep16020032","DOIUrl":"10.3390/hematolrep16020032","url":null,"abstract":"Most patients with diffuse large B-cell lymphoma (DLBCL) are >65 years of age, with the number of patients expected to increase in the coming years. A comprehensive geriatric assessment that carefully evaluates fitness status and comorbidities is essential for selecting the appropriate treatment intensity. Although generally healthy patients or those <80 years of age may benefit from standard immunochemotherapy, unfit/frail patients or patients >80 years old may require reduced-intensity chemotherapy or less-toxic drugs. Some new drugs are currently being tested as single or combined agents for first-line treatment, aiming to improve the outcomes of conventional chemotherapy. This review systematically collates and discusses the outcomes associated with the use of immunochemotherapy in older patients with DLBCL, as well as considering the impact of full-dose immunochemotherapy on quality of life in older and frail patients, summarizing the rationale for reduced dosing in the older population, and presenting recommendations for selecting patients likely to benefit from reduced dosing. If preliminary efficacy and safety data are confirmed in future clinical trials, non-chemotherapy-based immunotherapy approaches could become an alternative potentially curative option in frail patients and those >80 years of age with DLBCL.","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11204029/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141450323","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}

引用次数: 0

EBV-Positive Nodal T- and NK-Cell Lymphoma Mimicking Anaplastic Large Cell Lymphoma: A Case Report EBV阳性结节性T细胞和NK细胞淋巴瘤模仿无弹性大细胞淋巴瘤：病例报告

IF 0.9

Hematology Reports Pub Date : 2024-05-23 DOI: 10.3390/hematolrep16020031

B. Abro, Pamela Allen, Saja Asakrah, Kyle Bradley, Linsheng Zhang

{"title":"EBV-Positive Nodal T- and NK-Cell Lymphoma Mimicking Anaplastic Large Cell Lymphoma: A Case Report","authors":"B. Abro, Pamela Allen, Saja Asakrah, Kyle Bradley, Linsheng Zhang","doi":"10.3390/hematolrep16020031","DOIUrl":"https://doi.org/10.3390/hematolrep16020031","url":null,"abstract":"EBV-positive nodal T- and NK-cell lymphoma (EBV+ NT/NKCL) is a recently recognized entity in the 5th edition of the WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues. Notably, CD30 positivity is frequently observed in (EBV+ NT/NKCL), creating diagnostic challenges to distinguish it from ALK-negative anaplastic large cell lymphoma (ALCL). Furthermore, cases of EBV+ ALCL have been documented in the literature, predating the inclusion of EBV+ nodal cytotoxic T-cell lymphoma as a variant of peripheral T-cell lymphoma. We present a case of a 47-year-old male presenting with multiple lymphadenopathies. The histomorphologic and immunophenotypic features of the lymph node closely resemble ALK-negative ALCL, characterized by uniform CD30 expression and a subcapsular distribution of lymphoma cells. However, the lymphoma cells exhibit diffuse positivity for EBV, consistent with EBV+ NT/NKCL. A case of ALK-negative ALCL with an immunophenotype identical to the EBV-positive case is included for comparison. Given that EBV+ NT/NKCL represents an aggressive neoplasm requiring unique clinical management compared to ALK-negative ALCL, it is critical to accurately differentiate EBV+ NT/NKCL from ALK-negative ALCL with a cytotoxic T-cell immunophenotype.","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141104152","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}

引用次数: 0

Complications of Brentuximab Therapy in Patients with Hodgkin’s Lymphoma and Concurrent Autoimmune Pathology—A Case Series 霍奇金淋巴瘤合并自身免疫病理患者接受布伦妥昔单抗治疗的并发症--病例系列

IF 0.9

Hematology Reports Pub Date : 2024-05-20 DOI: 10.3390/hematolrep16020030

Oana Diana Preda, S. Badelita, I. Ursuleac, R. Irimia, Sonia Balanica, Monica Cojocaru, Cristina Cotruta, C. Dobrea, Daniel Coriu

{"title":"Complications of Brentuximab Therapy in Patients with Hodgkin’s Lymphoma and Concurrent Autoimmune Pathology—A Case Series","authors":"Oana Diana Preda, S. Badelita, I. Ursuleac, R. Irimia, Sonia Balanica, Monica Cojocaru, Cristina Cotruta, C. Dobrea, Daniel Coriu","doi":"10.3390/hematolrep16020030","DOIUrl":"https://doi.org/10.3390/hematolrep16020030","url":null,"abstract":"Background: Brentuximab Vedotin (BV) has revolutionized the treatment landscape for Hodgkin’s lymphoma, yet its effects on pre-existing autoimmune disorders remain elusive. Methods: Here, we present four cases of patients with concurrent autoimmune conditions—Crohn’s disease, vitiligo, type I diabetes, and minimal change disease—undergoing BV therapy for Hodgkin’s lymphoma. The patients were treated with A-AVD instead of ABVD due to advanced-stage disease with high IPI scores. Results: Our findings reveal the surprising and complex interplay between BV exposure and autoimmune manifestations, highlighting the need for multidisciplinary collaboration in patient management. Notably, the exacerbation of autoimmune symptoms was observed in the first three cases where T-cell-mediated autoimmunity predominated. Additionally, BV exposure precipitated autoimmune thrombocytopenia in the vitiligo patient, underscoring the profound disruptions in immune regulation. Conversely, in the minimal change disease case, a disease characterized by a blend of B- and T-cell-mediated immunity, the outcome was favorable. Conclusions: This paper underscores the critical importance of vigilance toward autoimmune flare-ups induced by BV in patients with concurrent autoimmune conditions, offering insights for tailored patient care.","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141120132","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}

引用次数: 0

Anti-PF4 ELISA-Negative, SRA-Positive Heparin-Induced Thrombocytopenia 抗 PF4 酶联免疫吸附试验阴性、SRA 阳性肝素诱导的血小板减少症

IF 0.9

Hematology Reports Pub Date : 2024-05-09 DOI: 10.3390/hematolrep16020029

A. Attah, Chelsea Peterson, Max W. Jacobs, Rama Bhagavatula, Deep Shah, Robert Kaplan, Y. Samhouri

引用次数: 0

Treatment Patterns and Clinical Outcomes of Patients with Moderate to Severe Acute Graft-Versus-Host Disease: A Multicenter Chart Review Study 中重度急性移植物抗宿主疾病患者的治疗模式和临床结果：多中心病历回顾研究

IF 0.9

Hematology Reports Pub Date : 2024-05-06 DOI: 10.3390/hematolrep16020028

D. Michonneau, Raynier Devillier, Mikko Keränen, M. Rubio, Malin Nicklasson, H. Labussière-Wallet, Martin Carre, A. Huynh, Elisabet Viayna, Montserrat Roset, Jonathan Finzi, Minja Pfeiffer, D. Thunström, Núria Lara, Lorenzo Sabatelli, P. Chevallier, M. Itälä-Remes

{"title":"Treatment Patterns and Clinical Outcomes of Patients with Moderate to Severe Acute Graft-Versus-Host Disease: A Multicenter Chart Review Study","authors":"D. Michonneau, Raynier Devillier, Mikko Keränen, M. Rubio, Malin Nicklasson, H. Labussière-Wallet, Martin Carre, A. Huynh, Elisabet Viayna, Montserrat Roset, Jonathan Finzi, Minja Pfeiffer, D. Thunström, Núria Lara, Lorenzo Sabatelli, P. Chevallier, M. Itälä-Remes","doi":"10.3390/hematolrep16020028","DOIUrl":"https://doi.org/10.3390/hematolrep16020028","url":null,"abstract":"Acute graft-versus-host disease (aGVHD) remains a barrier to successful allogeneic hematopoietic stem cell transplantation (HSCT) outcomes. Contemporary comprehensive analyses of real-world clinical outcomes among patients who develop aGVHD post-HSCT are needed to better understand the unmet needs of this patient population. This multicenter, retrospective chart review describes treatment patterns and clinical outcomes among patients (≥18 years old) from Finland, Sweden, and France who developed grades II–IV aGVHD after their first HSCT (January 2016–June 2017). From 13 participating centers, 151 patients were included. The median (Q1, Q3) age at HSCT was 56 (45, 62) years old. One line of aGVHD treatment was received by 47.7%, and the most common first-line treatment was methylprednisolone (alone or in a combination regimen, 74.2%; monotherapy, 25.8%). Among patients treated with methylprednisolone, 79.5% achieved a complete or partial response. The median (Q1, Q3) number of treatment lines was 2.0 (1.0, 3.0). The median (Q1, Q3) time to obtain an aGVHD diagnosis from transplant was 29.5 (21.0, 44.0) days, and 14.5 (7.0, 34.0) days to achieve the best response for 110 evaluable patients. At 6 and 12 months, 53.6% and 49.0%, respectively, achieved a complete response. Chronic GVHD occurred in 37.7% of patients, and aGVHD reoccurred in 26.5%. Following aGVHD diagnosis, mortality rates were 30.0% at 6 months and 37.3% at 12 months. Findings from this study demonstrate a continuing unmet need for new therapies that control aGVHD and improve mortality.","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141006796","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}

引用次数: 0

Current Therapeutic Sequencing in Chronic Lymphocytic Leukemia. 当前慢性淋巴细胞白血病的治疗排序。

IF 0.9

Hematology Reports Pub Date : 2024-04-30 DOI: 10.3390/hematolrep16020027

Samir Mouhssine, Nawar Maher, Sreekar Kogila, Claudio Cerchione, Giovanni Martinelli, Gianluca Gaidano

{"title":"Current Therapeutic Sequencing in Chronic Lymphocytic Leukemia.","authors":"Samir Mouhssine, Nawar Maher, Sreekar Kogila, Claudio Cerchione, Giovanni Martinelli, Gianluca Gaidano","doi":"10.3390/hematolrep16020027","DOIUrl":"10.3390/hematolrep16020027","url":null,"abstract":"The treatment landscape of chronic lymphocytic leukemia (CLL), the most frequent leukemia in adults, is constantly changing. CLL patients can be divided into three risk categories, based on their IGHV mutational status and the occurrence of TP53 disruption and/or complex karyotype. For the first-line treatment of low- and intermediate-risk CLL, both the BCL2 inhibitor venetoclax plus obinutuzumab and the second generation BTK inhibitors (BTKi), namely acalabrutinib and zanubrutinib, are valuable and effective options. Conversely, venetoclax-based fixed duration therapies have not shown remarkable results in high-risk CLL patients, while continuous treatment with acalabrutinib and zanubrutinib displayed favorable outcomes, similar to those obtained in TP53 wild-type patients. The development of acquired resistance to pathway inhibitors is still a clinical challenge, and the optimal treatment sequencing of relapsed/refractory CLL is not completely established. Covalent BTKi-refractory patients should be treated with venetoclax plus rituximab, whereas venetoclax-refractory CLL may be treated with second generation BTKi in the case of early relapse, while venetoclax plus rituximab might be used if late relapse has occurred. On these grounds, here we provide an overview of the current state-of-the-art therapeutic algorithms for treatment-naïve patients, as well as for relapsed/refractory disease.","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11130833/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141157963","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}

引用次数: 0

Is There an Association between a Tonsillar Diffuse Large B-Cell Lymphoma Arising after a Neck Squamous Cell Carcinoma of Occult Primary? A Case Report and Extensive Literature Review. 颈部隐匿原发鳞状细胞癌后出现扁桃体弥漫大 B 细胞淋巴瘤之间有关联吗？病例报告和广泛的文献综述。

IF 0.9

Hematology Reports Pub Date : 2024-04-29 DOI: 10.3390/hematolrep16020026

Dimitris Tatsis, Athena Niakou, Konstantinos Paraskevopoulos, Stavroula Papadopoulou, Konstantinos Vahtsevanos

{"title":"Is There an Association between a Tonsillar Diffuse Large B-Cell Lymphoma Arising after a Neck Squamous Cell Carcinoma of Occult Primary? A Case Report and Extensive Literature Review.","authors":"Dimitris Tatsis, Athena Niakou, Konstantinos Paraskevopoulos, Stavroula Papadopoulou, Konstantinos Vahtsevanos","doi":"10.3390/hematolrep16020026","DOIUrl":"10.3390/hematolrep16020026","url":null,"abstract":"Objectives: The aim of this review is to focus on the possibility of patients with squamous cell carcinoma to develop a second primary disease such as DLBCL, perhaps because of the irradiation of the head and neck area.Materials and methods: A case of an 89-year-old man is reported, who initially underwent surgical and complementary treatment for neck squamous cell carcinoma of occult primary and later for tonsillar diffuse large B-cell non-Hodgkin lymphoma.Results: The second primary was considered a recurrence in the neck of the original cancer of unknown primary, so a new surgical management was decided. The final pathology report described a diffuse large B-cell non-Hodgkin lymphoma.Conclusions: The importance of maintaining follow-ups for patients with occult primary cancers who are at an elevated risk of developing a metastasis or a second primary carcinoma outbreak is highlighted.","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11130887/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141157992","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}

引用次数: 0

Eculizumab Treatment of Massive Hemolysis Occurring in a Rare Co-Existence of Paroxysmal Nocturnal Hemoglobinuria and Myasthenia Gravis 依库珠单抗治疗阵发性夜间血红蛋白尿和重症肌无力罕见并发症中发生的大量溶血

IF 0.9

Hematology Reports Pub Date : 2024-04-19 DOI: 10.3390/hematolrep16020025

Ráhel Réka Bicskó, Árpád Illés, Zsuzsanna Hevessy, G. Ivády, György Kerekes, Gábor Méhes, T. Csépány, L. Gergely

{"title":"Eculizumab Treatment of Massive Hemolysis Occurring in a Rare Co-Existence of Paroxysmal Nocturnal Hemoglobinuria and Myasthenia Gravis","authors":"Ráhel Réka Bicskó, Árpád Illés, Zsuzsanna Hevessy, G. Ivády, György Kerekes, Gábor Méhes, T. Csépány, L. Gergely","doi":"10.3390/hematolrep16020025","DOIUrl":"https://doi.org/10.3390/hematolrep16020025","url":null,"abstract":"The co-occurrence of myasthenia gravis (MG) and paroxysmal nocturnal hemoglobinuria (PNH) is rare; only one case has been published so far. We report a 63-year-old Caucasian female patient who was diagnosed with MG at the age of 43. Thymoma was also detected, and so it was surgically resected, which resulted in reasonable disease control for nearly 20 years. Slight hemolysis began to emerge, and then myasthenia symptoms progressed, so immunosuppressive therapy was started. Due to progressive disease and respiratory failure, the patient underwent plasmapheresis, and ventilatory support was stopped. Marked hemolysis was present, and diagnostic tests confirmed PNH with type III PNH cells. Her myasthenia symptoms aggravated, mechanical ventilation had to be started again, and due to the respiratory acidosis, massive hemolysis occurred. After two plasmapheresis sessions, the patient received eculizumab at 600 mg, resulting in prompt hemolysis control. After the second dose of the treatment, the patient was extubated. Still, due to their inability to cough, she developed another respiratory failure and pneumonia–sepsis, resulting in the patient’s death. This case highlights the rare association between these two serious diseases and similar immune-mediated pathophysiology mechanisms involving the complement system.","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-04-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140683192","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}

引用次数: 0