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Hb Thessaloniki, a Novel, Hyperunstable, Alpha Globin Variant Detected in Northern Greece. Hb Thessaloniki,在希腊北部发现的一种新的,高度不稳定的α -球蛋白变体。
IF 1.9
Hematology Reports Pub Date : 2026-02-26 DOI: 10.3390/hematolrep18020017
Effrossyni Boutou, Nikos Papandreou, Genovefa Mantzou, Efthymia Vlachaki, Athanasios Vyzantiadis, Christos Chassanidis, Maria Dimopoulou, Angeliki Balassopoulou, Stamatia Theodoridou
{"title":"Hb Thessaloniki, a Novel, Hyperunstable, Alpha Globin Variant Detected in Northern Greece.","authors":"Effrossyni Boutou, Nikos Papandreou, Genovefa Mantzou, Efthymia Vlachaki, Athanasios Vyzantiadis, Christos Chassanidis, Maria Dimopoulou, Angeliki Balassopoulou, Stamatia Theodoridou","doi":"10.3390/hematolrep18020017","DOIUrl":"10.3390/hematolrep18020017","url":null,"abstract":"<p><p><b>Background:</b> Haemoglobinopathies are the most common monogenic disorders both in Greece and worldwide. The most effective strategies against them are carrier detection and prenatal testing following genetic risk assessment consultation for couples on the likelihood of their offspring being affected. <b>Case Presentation:</b> A novel alpha globin chain variant, named Hb Thessaloniki, was detected in Northern Greece. The underlying point variation HBA1:c.260T>C (ref. seq. NM_000558.5) was detected in the HBA1 gene, in heterozygosity, during a routinely performed population screening for haemoglobinopathies. The amino-acid residue Leu86 was replaced by a structure disrupting Pro residue, resulting in a hyperunstable product as shown by the isopropanol test and predicted by the Dynamut2 and Alphafold3 algorithms. The haematological phenotype, due to which genetic analysis was performed, presented with mild microcytosis and hypochromia and was also indicative of the presence of an unstable haemoglobin produced in small quantities (variant encoded by HBA1). Since the proband's partner presented with a normal haematological phenotype, there is no risk of the couple giving birth to an affected offspring. Expanded analysis of the proband's relatives identified biallelic variants (α<sup>Parma</sup>α/αα<sup>Τhessaloniki</sup>) in the proband's mother, who presented with no apparent clinical findings, expect for slightly reduced haematological indices. <b>Conclusions</b>: The novel Hb Thessaloniki identified, although theoretically hyperunstable, seems to have minor effects on erythrocyte function, as indicated by haematological findings on the proband and his close relatives. Future identification of co-inheritance with HBA pathogenic point variations or deletions may provide further information regarding genetic counselling. In parallel, the usage of structure-function relation-calculating algorithms may enhance our prediction capability for novel variants.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 2","pages":""},"PeriodicalIF":1.9,"publicationDate":"2026-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13010653/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147503731","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
Inherited Platelet Disorders During Pregnancy and Delivery: Overview of Management Strategies and Emerging Therapeutic Considerations. 妊娠和分娩期间的遗传性血小板疾病:管理策略概述和新出现的治疗考虑。
IF 1.9
Hematology Reports Pub Date : 2026-02-26 DOI: 10.3390/hematolrep18020016
Victor Zibara, Nicoletta Machin
{"title":"Inherited Platelet Disorders During Pregnancy and Delivery: Overview of Management Strategies and Emerging Therapeutic Considerations.","authors":"Victor Zibara, Nicoletta Machin","doi":"10.3390/hematolrep18020016","DOIUrl":"10.3390/hematolrep18020016","url":null,"abstract":"<p><p>Inherited platelet disorders (IPDs) comprise a heterogeneous group of rare conditions that present particular challenges during pregnancy, with bleeding risk increasing during labor and the immediate postpartum period. These disorders require coordinated, multidisciplinary management to mitigate maternal and neonatal bleeding risk. Although data remains limited, individuals with IPD, including Bernard-Soulier syndrome, Glanzmann thrombasthenia, MYH9-related disorders, Hermansky-Pudlak syndrome, and platelet storage pool disorders, are at an increased risk for obstetrical bleeding, with the degree of risk varying by underlying diagnosis. In severe inherited platelet disorders such as Glanzmann thrombasthenia, peripartum hemorrhage is common, with up to half of the deliveries in some series requiring red cell or platelet transfusion. Because these conditions are congenital, the fetus may also be affected, placing neonates at risk for serious bleeding complications, including intracranial hemorrhage, although available data is limited. Despite the considerable morbidity and mortality risk associated with inherited platelet disorders, management strategies during pregnancy and delivery remain poorly defined. This stands in contrast to other bleeding disorders, such as factor deficiencies, for which multiple therapeutic approaches have been evaluated in the peripartum setting. In this review, we summarize the available evidence and current management strategies for individuals with inherited platelet disorders during pregnancy and delivery.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 2","pages":""},"PeriodicalIF":1.9,"publicationDate":"2026-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13010773/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147503827","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
A Review of Patient-Reported Outcomes and Clinical Outcomes in Acute and Chronic Myeloid and Lymphoid Leukemias. 急性和慢性髓性和淋巴性白血病患者报告的结果和临床结果综述。
IF 1.9
Hematology Reports Pub Date : 2026-02-06 DOI: 10.3390/hematolrep18010015
Bryan Chan, Eesha Balar, Seiichi Villalona, Judith Karp, Allison Leahy, Catherine Lai
{"title":"A Review of Patient-Reported Outcomes and Clinical Outcomes in Acute and Chronic Myeloid and Lymphoid Leukemias.","authors":"Bryan Chan, Eesha Balar, Seiichi Villalona, Judith Karp, Allison Leahy, Catherine Lai","doi":"10.3390/hematolrep18010015","DOIUrl":"10.3390/hematolrep18010015","url":null,"abstract":"<p><p><b>Introduction:</b> This review specifically focuses on interventional clinical trials in leukemias and myelodysplastic syndromes (MDS), summarizing how patient-reported outcome measures (PROMs) have been implemented to evaluate treatment effects rather than to directly influence clinical outcomes. <b>Objective:</b> Clinical outcomes of interest typically include response rates, disease-free survival (DFS), and overall survival (OS). Patient-reported outcome measures (PROMs) are standardized questionnaires that collect information regarding health outcomes directly from the patient and are used to evaluate new treatments and healthcare quality. In addition, the use of PROMs in cancer care has been shown to improve patient-provider communication and patient satisfaction. <b>Material and Methods:</b> This is a qualitative, narrative synthesis and review structured around PROMs focused on six critical themes: symptoms/symptom burden, physical, emotional, social/role, and functional status, and global health status measurement. <b>Results:</b> PROMs that are assessed in oncologic research include the EORTC QLQ-C30, FACT-Leu, QLQ-CLL16, and EQ-5D. PROs are associated with clinical outcomes such as DFS and OS, and the FACT-Leu scales, HRQOL and physical functioning scores were independent prognosticators of OS in patients with AML. <b>Conclusions:</b> Through our review, notable trends were identified that further highlight the importance of greater incorporation of PRO measures in future clinical trials, particularly in the understudied realm of hematologic malignancies, in order to better delineate the link between survival and HRQOL.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 1","pages":""},"PeriodicalIF":1.9,"publicationDate":"2026-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12921762/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146257805","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
Factors Influencing the Use of G-CSF in Drug-Induced Agranulocytosis. 影响G-CSF在药物性粒细胞缺乏症中应用的因素。
IF 1.9
Hematology Reports Pub Date : 2026-02-03 DOI: 10.3390/hematolrep18010014
Emmanuel Andrès, Jean-Edouard Terrade, Xavier Jannot, Noel Lorenzo-Villalba
{"title":"Factors Influencing the Use of G-CSF in Drug-Induced Agranulocytosis.","authors":"Emmanuel Andrès, Jean-Edouard Terrade, Xavier Jannot, Noel Lorenzo-Villalba","doi":"10.3390/hematolrep18010014","DOIUrl":"10.3390/hematolrep18010014","url":null,"abstract":"<p><p>Drug-induced agranulocytosis is a rare but life-threatening adverse reaction associated with numerous non-chemotherapy drugs. Management relies on immediate drug withdrawal, infection control, and, in selected patients, administration of granulocyte-colony stimulating factor (G-CSF). This review summarizes current knowledge on the determinants of epidemiology, clinical presentation, hematologic and biologic features, comorbidities, and outcomes influencing the decision to introduce G-CSF in drug-induced agranulocytosis. Evidence from observational studies and meta-analyses suggests that G-CSF shortens neutropenia duration and hospitalization, although its impact on mortality remains uncertain. The decision to use G-CSF should consider initial neutrophil count, presence of severe infection or sepsis, age, and comorbidities. Despite the accumulated experience, randomized controlled trials are still lacking, and treatment algorithms remain empirical.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 1","pages":""},"PeriodicalIF":1.9,"publicationDate":"2026-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12921758/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146257883","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
Fluid Overload-Associated Large B-Cell Lymphoma Presenting as Isolated Pleural Effusion. 液体超载相关的大b细胞淋巴瘤表现为孤立的胸腔积液。
IF 1.9
Hematology Reports Pub Date : 2026-02-02 DOI: 10.3390/hematolrep18010013
Kevin Leeper, Lauren Borecky, Mojtaba Akhtari, Jun Wang
{"title":"Fluid Overload-Associated Large B-Cell Lymphoma Presenting as Isolated Pleural Effusion.","authors":"Kevin Leeper, Lauren Borecky, Mojtaba Akhtari, Jun Wang","doi":"10.3390/hematolrep18010013","DOIUrl":"10.3390/hematolrep18010013","url":null,"abstract":"<p><p>Primary effusion-based lymphomas are uncommon and may pose significant diagnostic challenges. Fluid overload-associated large B-cell lymphoma is a recently recognized entity in the 5th edition of the World Health Organization Classification of Hematolymphoid Tumors and should be included in the differential diagnosis of effusion-based lymphomas, particularly in elderly immunocompetent patients with conditions that predispose to fluid overload. <b>Background and Clinical Significance:</b> We report a case of fluid overload-associated large B-cell lymphoma to add to the limited literature and highlight distinguishing features from other primary effusion lymphomas. <b>Case Presentation</b>: A 77-year-old male with end-stage renal disease on hemodialysis and heart failure with reduced ejection fraction was admitted for respiratory failure and found to have a right-sided pleural effusion. Two pleural fluid specimens examined several weeks apart revealed sheets of large atypical lymphoid cells positive for CD20, Pax-5, CD79a, CD45, MUM1, BCL2, BCL6 (weak) and negative for TTF1, CD68, MOC31, BER EP4, WT1, Calretinin, CD3, CD138, CD30, and cMYC. Human Herpesvirus-8 and Epstein-Barr virus were negative. Staging showed a few mildly fluorodeoxyglucose-avid mediastinal lymph nodes which were benign. Ultimately, the patient was diagnosed with fluid overload-associated large B-cell lymphoma and treated with rituximab, cyclophosphamide, vincristine sulfate, and prednisone, but passed away three months after diagnosis. <b>Conclusions</b>: Fluid overload-associated large B-cell lymphoma is a new and important diagnostic consideration in effusion-based lymphomas. It may be mistaken for other conditions such as primary effusion lymphoma or other diffuse large B-cell lymphomas. The presence of a Human Herpesvirus-8-negative effusion-based lymphoma in an elderly immunocompetent patient without nodal or tissue involvement should prompt consideration of fluid overload-associated large B-cell lymphoma.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 1","pages":""},"PeriodicalIF":1.9,"publicationDate":"2026-02-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12922083/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146257918","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
Primary Indolent Acute Promyelocytic Leukemia. 原发性惰性急性早幼粒细胞白血病。
IF 1.9
Hematology Reports Pub Date : 2026-01-27 DOI: 10.3390/hematolrep18010012
Breanne Wolfenbarger, Daley Morera, Brandol Wolfenbarger, Anand Jillella, Mei Zheng
{"title":"Primary Indolent Acute Promyelocytic Leukemia.","authors":"Breanne Wolfenbarger, Daley Morera, Brandol Wolfenbarger, Anand Jillella, Mei Zheng","doi":"10.3390/hematolrep18010012","DOIUrl":"10.3390/hematolrep18010012","url":null,"abstract":"<p><p><b>Background and Clinical Significance:</b> Acute promyelocytic leukemia (APL) is a rapidly progressive subtype of acute myeloid leukemia defined by <i>PML::RARA</i> fusion and characterized by life-threatening coagulopathy. Because the disease typically follows an aggressive course, immediate treatment is essential once APL is suspected. This case report describes an atypical de novo presentation marked by indolent progression rather than the expected aggressive trajectory. <b>Case Presentation:</b> A 37-year-old female exhibited gradually declining white blood cell and neutrophil counts over the course of a year, followed by unexplained pancytopenia with severe neutropenia (0.1 × 10<sup>9</sup>/L). Evaluation for nutritional deficiencies and autoimmune disease was unrevealing aside from a positive ANA without clinical features of autoimmunity. Bone-marrow biopsy demonstrated morphologic and flow cytometric findings suggestive of APL, low-level t(15;17), <i>PML::RARA</i> fusion, and concomitant <i>TP53</i> loss and <i>ETV6</i> mutation. Despite the indolent clinical presentation and low disease burden, the molecular and cytogenetic findings confirmed the diagnosis of classical APL with <i>TP53</i> loss and <i>ETV6</i> mutation. Induction therapy with all-trans-retinoic acid and arsenic trioxide resulted in hematologic remission. <b>Conclusions:</b> This case highlights an unusually indolent form of de novo APL not previously documented in the literature, expanding the recognized clinical spectrum of the disease. The findings emphasize the importance of still considering severe diagnoses, such as APL, when presentations deviate from classical patterns. Atypical clinical trajectories should prompt careful assessment of marrow morphology and immunophenotypic features. Continued characterization of such cases may refine diagnostic criteria and direct individualized approaches to therapy.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 1","pages":""},"PeriodicalIF":1.9,"publicationDate":"2026-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12921931/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146257935","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
Primary Bone Lymphoma of the Jaw Masquerading as Infection and Delaying Treatment. 颌骨原发性骨淋巴瘤伪装成感染和延迟治疗。
IF 1.9
Hematology Reports Pub Date : 2026-01-22 DOI: 10.3390/hematolrep18010011
Emily Hamburger, Anne W Beaven
{"title":"Primary Bone Lymphoma of the Jaw Masquerading as Infection and Delaying Treatment.","authors":"Emily Hamburger, Anne W Beaven","doi":"10.3390/hematolrep18010011","DOIUrl":"10.3390/hematolrep18010011","url":null,"abstract":"<p><p><b>Background:</b> Diffuse large B cell lymphoma is an aggressive, heterogeneous yet treatable disease. Primary bone lymphoma is a lymphoma involving a single or multiple osseous sites with or without regional nodal involvement. It is exceedingly rare, representing <1% of new non-Hodgkin lymphoma cases per year. Most cases of primary bone lymphoma are diffuse large B cell lymphoma. They infrequently involve the craniofacial bones and mandible; its rarity can lead to delays in diagnosis. <b>Case Series Presentation:</b> Two 64-year-old male patients initially presented to local dentists with concerns of tooth pain and numbness. Both underwent extensive dental procedures including extraction and debridement, with an initial diagnosis of osteomyelitis. They were placed on long-term antibiotics. After months without improvement, further testing was pursued, including imaging and repeat biopsies. The patients were finally diagnosed with primary bone diffuse large B cell lymphoma. From the initial treatment of osteomyelitis, a median time of 8.5 months passed before diagnosis of lymphoma. Treatment with cytotoxic chemotherapy was initiated and both patients achieved remission. <b>Conclusions:</b> As in the two cases presented here, the initial point of entry into the medical system may be a visit to the local dentist. When patients present with periodontal complaints, it is imperative to maintain a broad differential, including lymphoma. This is especially crucial when the patient's clinical course does not respond to initial treatment. This results in delays of diagnosis and initiation of therapy for a treatable cancer.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 1","pages":""},"PeriodicalIF":1.9,"publicationDate":"2026-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12922053/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146257980","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
Therapeutic Potential of Exportin 1 and Aurora Kinase A Inhibition in Multiple Myeloma Cells. 出口蛋白1和极光激酶A抑制在多发性骨髓瘤细胞中的治疗潜力。
IF 1.2
Hematology Reports Pub Date : 2026-01-09 DOI: 10.3390/hematolrep18010010
Seiichi Okabe, Yuko Tanaka, Shunsuke Otsuki, Mitsuru Moriyama, Seiichiro Yoshizawa, Akihiko Gotoh, Daigo Akahane
{"title":"Therapeutic Potential of Exportin 1 and Aurora Kinase A Inhibition in Multiple Myeloma Cells.","authors":"Seiichi Okabe, Yuko Tanaka, Shunsuke Otsuki, Mitsuru Moriyama, Seiichiro Yoshizawa, Akihiko Gotoh, Daigo Akahane","doi":"10.3390/hematolrep18010010","DOIUrl":"10.3390/hematolrep18010010","url":null,"abstract":"<p><p><b>Background/Objectives:</b> Aurora kinases (AURKs) are key regulators of mitosis, and their dysregulation contributes to plasma cell disorders, including multiple myeloma (MM) and plasma cell leukemia (PCL). <b>Methods:</b> The expression and prognostic relevance of AURK family members were examined, and the therapeutic potential of AURKA inhibition was evaluated. <b>Results:</b> Gene expression analysis demonstrated significant upregulation of AURKA in PCL. Treatment of MM cells with the selective AURKA inhibitor LY3295668 induced dose-dependent cytotoxicity, caspase-3/7 activation, and cellular senescence. Similarly, selinexor, a selective exportin-1 inhibitor, elicited dose-dependent cytotoxicity and apoptosis. Combined treatment with LY3295668 and selinexor significantly improved apoptosis compared with either agent alone, and AURKA knockdown further sensitized MM cells to selinexor, thereby increasing apoptosis. In bortezomib-resistant MM cells and primary PCL samples, the combination therapy induced cytotoxicity and caspase-3/7 activation. <b>Conclusions:</b> These findings underscore AURKA expression as a prognostic marker in plasma cell disorders and support the therapeutic potential of combining AURKA inhibition with selinexor for bortezomib-resistant MM and PCL. To explore biomarker-driven strategies for optimizing therapeutic outcomes, future studies are warranted.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 1","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-01-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12821534/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146010202","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
Bortezomib-Induced Sensorineural Hearing Loss May Be Reversible with Intratympanic Dexamethasone. 硼替佐米引起的感音神经性听力损失可能可通过鼓腔内地塞米松逆转。
IF 1.2
Hematology Reports Pub Date : 2026-01-06 DOI: 10.3390/hematolrep18010009
Natalia Peláez Casillas, Jose Maria Verdaguer Muñoz, Antonio Rodríguez Valiente, Irene Romera Martínez, Jose Ramón García Berrocal
{"title":"Bortezomib-Induced Sensorineural Hearing Loss May Be Reversible with Intratympanic Dexamethasone.","authors":"Natalia Peláez Casillas, Jose Maria Verdaguer Muñoz, Antonio Rodríguez Valiente, Irene Romera Martínez, Jose Ramón García Berrocal","doi":"10.3390/hematolrep18010009","DOIUrl":"10.3390/hematolrep18010009","url":null,"abstract":"<p><p><b>Background</b>: Bortezomib, a proteasome inhibitor used in multiple myeloma (MM), is associated with several adverse effects, most notably peripheral neuropathy. Ototoxicity, however, remains a rare and underrecognized complication. <b>Case presentation</b>: We report the case of a 74-year-old man with MM who developed sudden unilateral sensorineural hearing loss following subcutaneous bortezomib administration. Audiometry confirmed severe right-sided hearing loss. MRI of the internal auditory canal was normal. Given the absence of other ototoxic agents, bortezomib was identified as the likely causative drug. The patient was treated with intratympanic dexamethasone injections, achieving partial hearing recovery. Subsequent chemotherapy re-exposure triggered another hearing decline, which again improved after repeated intratympanic treatment. <b>Conclusions</b>: Bortezomib-related ototoxicity is a rare but potentially reversible adverse event. This case suggests that early intratympanic corticosteroid therapy may mitigate cochlear injury, allowing continuation of chemotherapy for patients responding well to bortezomib.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 1","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12821528/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146010131","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
Paraneoplastic Neuro-Ophthalmologic Symptoms as Initial Manifestation of Hodgkin Lymphoma. 副肿瘤神经-眼科症状是霍奇金淋巴瘤的初始表现。
IF 1.2
Hematology Reports Pub Date : 2026-01-05 DOI: 10.3390/hematolrep18010008
Sophie-Charlott Seidenfaden, Thomas Graversgaard Adams, Peter Kamper, Sanne Jespersen, Martin Bjerregård Pedersen
{"title":"Paraneoplastic Neuro-Ophthalmologic Symptoms as Initial Manifestation of Hodgkin Lymphoma.","authors":"Sophie-Charlott Seidenfaden, Thomas Graversgaard Adams, Peter Kamper, Sanne Jespersen, Martin Bjerregård Pedersen","doi":"10.3390/hematolrep18010008","DOIUrl":"10.3390/hematolrep18010008","url":null,"abstract":"<p><p><b>Background and Clinical Significance:</b> Patients with Hodgkin lymphoma (HL) often present with lymphadenopathy, biochemical inflammation, and constitutional symptoms, but may experience symptoms from extra-nodal organs. Symptoms are caused by either lymphoma or a paraneoplastic phenomenon but overt central nervous system (CNS) involvement in HL is very uncommon. However, in rare cases, paraneoplastic neuro-ophthalmologic manifestations occur. <b>Case Presentation:</b> This case report describes a young female diagnosed with HL initially presenting with visual loss, reduced visual field, impaired balance, and sensory disturbances but no evidence of CNS-lymphoma. After treatment with bleomycin, etoposide, adriamycin, cyclophosphamide, vincristine, procarbazine, and prednisolone (escalated BEACOPP), she experienced full recovery of all neurological and ophthalmological symptoms. She experienced complete remission without any signs of relapse at follow-up after 2.5 years. Paraneoplastic cerebellar degeneration (PCD) related to HL have been described as a rare neurological syndrome, with varying neurological symptoms preceding the diagnosis of HL. PCD is typically associated with anti-Tr antibodies. Despite being negative for anti-Tr antibodies in both serum and cerebrospinal fluid (CSF), the neuro-ophthalmologic symptoms were interpreted as a paraneoplastic phenomenon in HL resembling PCD. The exact pathophysiology in this case is unknown but might be associated with undetected antigens and T-cell-mediated autoimmunity because of the presence of non-malignant T-cells in the CSF. <b>Conclusions:</b> This manuscript describes a case of an atypical presentation of HL with neuro-ophthalmologic symptoms which fully recovered upon anti-lymphoma treatment. Because of the good prognosis, we aim to emphasize the awareness of rare cases of HL initially presenting such manifestations to avoid diagnostic delays.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 1","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-01-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12821392/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146010209","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
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