Journal of Hematopathology最新文献

{"title":"HHV8-associated multicentric Castleman disease with Kaposi sarcoma in an immunocompetent patient: a case report and review of the literature.","authors":"K Hoerschgen, W Zhang, A Rappolt, A Ashford, J C Padussis, J D Khoury, A Ronaghy","doi":"10.1007/s12308-026-00744-z","DOIUrl":"https://doi.org/10.1007/s12308-026-00744-z","url":null,"abstract":"<p><p>Cases of concurrent Kaposi sarcoma (KS) and HHV8-associated Multicentric Castleman Disease (HHV8-MCD) are exceedingly rare in immunocompetent individuals. We present a case of a 57-year-old man with a 5-month history of generalized lymphadenopathy/weakness, cough, fever, and weight loss following travel to Mexico. Tests for HIV, CMV, EBV and tuberculosis were negative. Histological examination showed KS and HHV8-MCD in the same lymph node. Targeted next generation sequencing of over 517 genes performed separately on the two lesions showed no driver mutations but identified shared nonsynonymous polymorphisms in 8 genes: ATRX, EML4, FGF4, IKBKE, KLF4, KMT2A, NTRK3, and RASA, suggesting that several convergent oncogenic pathways might be altered in both the KS/HHV8-MCD lesions, with postulated impact on chromatin remodeling/epigenetic regulation, growth factor/receptor tyrosine kinase signaling, NF-κB signaling, and RAS/MAPK pathways. The patient was initially treated with rituximab and doxorubicin but expired from complications associated with his illness despite intensive therapy. Finding both Kaposi sarcoma and HHV8-MCD in the same lymph node, particularly without mucocutaneous manifestations, is rare amongst HIV-negative patients. To our knowledge, only 23 other cases with similar findings have been reported to date, but none included mutation profiling characterization of the concurrent lesions. Review of the literature was notable for KSHV-MCD being identified concurrently with Kaposi sarcoma in 10-20% of cases with immunocompetent patients. In summary, our case highlights not only the importance of early recognition of MCD but also demonstrates a rare occurrence of HHV8-MCD/KS involving the same lymph node in an HIV negative, immunocompetent patient.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149276696","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Myeloid neoplasm with PDGFRA rearrangement presenting in blast-phase with basophilia.","authors":"Anne-Cecilia van Marle, Evan Bowen","doi":"10.1007/s12308-026-00743-0","DOIUrl":"10.1007/s12308-026-00743-0","url":null,"abstract":"<p><p>An adult man, from Southern Africa, presented to a public health centre with symptoms of progressive fatigue. Peripheral blood morphology showed marked basophilia and 21% myeloblasts, confirmed by flow cytometry, suggesting acute myeloid leukaemia with basophilia. Bone marrow biopsy revealed increased mast cells, raising systemic mastocytosis as a differential. However, next-generation sequencing identified a PDGFRA fusion, establishing the diagnosis of blast phase of a myeloid/lymphoid neoplasm with PDGFRA rearrangement. The presence of cells with abnormal basophilic granulation and absence of peripheral blood eosinophilia, together with lengthy turnaround times (TAT) for specialised tests in the public health sector contributed to the delayed diagnosis. This case highlights the diagnostic challenges posed by overlapping morphological features and underscores the critical role of molecular testing in identifying rare but clinically significant tyrosine kinase fusion-driven haematological malignancies, which are highly responsive to targeted therapy.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13661702/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149185843","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"EBV-positive Hodgkin lymphoma with an immunosuppressive microenvironment during guselkumab (interleukin-23 inhibitor) therapy for chronic plaque psoriasis.","authors":"Bhaumik Shah, Janusz Franco-Barraza, Kathy Q Cai, Mariia Dmitrieva, Marcus Messmer, Yibin Yang, Mariusz Wasik, Edna Cukierman, Reza Nejati","doi":"10.1007/s12308-026-00739-w","DOIUrl":"10.1007/s12308-026-00739-w","url":null,"abstract":"<p><strong>Background: </strong>Psoriasis is an independent risk factor for classic Hodgkin lymphoma (cHL). Guselkumab has a favorable malignancy safety profile in clinical trials.</p><p><strong>Purpose: </strong>To report the first documented Epstein-Barr virus (EBV)-positive cHL arising in a biologic-naive patient with psoriatic arthritis during guselkumab therapy for chronic plaque psoriasis, and to describe its tumor microenvironment.</p><p><strong>Methods: </strong>Excisional biopsy with immunohistochemistry, EBER in situ hybridization, and targeted sequencing, plus spatial single-cell proteomics (9-plex sequential immunofluorescence) of the diagnostic lymph node.</p><p><strong>Results: </strong>A man in his early 40s developed night sweats, syncope, and bulky right cervical lymphadenopathy 7 months after starting guselkumab. Biopsy showed mixed-cellularity EBV-positive cHL (latency IIa: EBER+, LMP1+, EBNA-2-). HRS cells were weak PAX5+, CD20 negative, CD15+, strong CD30+, OCT2 negative/weak, BOB1 negative/weak, and strong CD200+. The microenvironment contained CTLA4+ immunosuppressive T cells (10.15%), M2 macrophages (10.35%), modest Th2 cells (0.38%), a low Th1/Th2 ratio (~0.39), and Th17 cells (~13%). GATA3 and CCR4 immunohistochemistry confirmed modest Th2 skewing. After guselkumab was stopped, he achieved a complete metabolic response with ABVD and involved-site radiotherapy and remains in remission on apremilast.</p><p><strong>Conclusions: </strong>The short latency and atypical Th2-skewed, myeloid-enriched microenvironment, in contrast to the typical Th1-dominant profile of EBV-positive cHL, raise the possibility of immune-milieu alteration under IL-23 inhibition. This case supports continued pharmacovigilance for rare lymphomas in patients receiving IL-23 inhibitors and the need for larger studies to clarify potential class effects.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149149524","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Histiocytoid melanoma presenting as lymphadenopathy and mimicking Rosai-Dorfman disease.","authors":"Stephen J Babcock, Valentina Nardi","doi":"10.1007/s12308-026-00742-1","DOIUrl":"10.1007/s12308-026-00742-1","url":null,"abstract":"<p><p>We report a case of a patient with large, unilateral inguinal lymphadenopathy. Excisional biopsy demonstrated expansion of the lymph node sinuses by a population of large histiocytoid cells with abundant granular eosinophilic cytoplasm and large, eccentric nuclei with prominent central nucleoli. The findings were morphologically suspicious for Rosai-Dorfman disease; however, emperipolesis was conspicuously absent. Immunohistochemical work-up demonstrated that the infiltrate consisted of metastatic melanoma with histiocytoid morphology and admixed reactive sinus histiocytes.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149139178","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Early-onset VEXAS syndrome mimicking orbital cellulitis and successfully treated with allogeneic hematopoietic stem cell transplantation.","authors":"Gökhan Burul, Çagla Karaoglan, Erdem Bektaş, Cemal Bes, Leylagül Kaynar, İstemi Serin, Şeniz Öngören","doi":"10.1007/s12308-026-00741-2","DOIUrl":"10.1007/s12308-026-00741-2","url":null,"abstract":"<p><strong>Background: </strong>VEXAS syndrome is a somatic autoinflammatory disorder caused by UBA1 mutations in hematopoietic progenitor cells, typically affecting older men and characterized by systemic inflammation and hematologic abnormalities. Early-onset cases are extremely rare.</p><p><strong>Case presentation: </strong>We report a 25-year-old male patient presenting with recurrent fever, weight loss, arthralgia, and inflammation mimicking orbital cellulitis. Imaging demonstrated orbital soft tissue inflammation. Laboratory investigations revealed macrocytic anemia, leukopenia, thrombocytopenia, and elevated inflammatory markers; FDG-PET/CT showed splenomegaly and hypermetabolic lymphadenopathy. Bone marrow examination revealed hypercellularity, multilineage morphological abnormalities, and vacuolated blasts. Following exclusion of infectious and classical autoinflammatory diseases, the somatic UBA1 c.121A > G (p.Met41Val) mutation confirmed VEXAS syndrome. The patient initially responded to corticosteroids but subsequently developed refractory disease and progressive pancytopenia despite tocilizumab therapy. He underwent allogeneic hematopoietic stem cell transplantation from a 10/10 HLA-matched sibling donor using a myeloablative conditioning regimen. At 3 months post-transplantation, 99% donor chimerism and hematologic remission were achieved.</p><p><strong>Conclusion: </strong>VEXAS syndrome should be considered in young adults presenting with unexplained systemic inflammation and cytopenias, even with atypical manifestations such as orbital cellulitis. Early genetic evaluation is crucial for diagnosis, and allogeneic stem cell transplantation may provide effective disease control in refractory cases.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149118401","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Concurrent presentation of distinct extranodal and splenic marginal zone lymphomas.","authors":"Jeremy D Grevet, Yong Zhang, Sam Sadigh, Austin I Kim","doi":"10.1007/s12308-026-00740-3","DOIUrl":"10.1007/s12308-026-00740-3","url":null,"abstract":"<p><p>Marginal zone lymphomas (MZLs) encompass extranodal, splenic, and nodal subtypes that are thought to clonally arise from antigen-exposed B-cells. Here, we present the case of a patient with a concurrent diagnosis of extranodal marginal zone lymphoma (EMZL) of the ocular adnexa and splenic marginal zone lymphoma (SMZL). These were identified on a core biopsy of the ocular adnexa and a bone marrow biopsy, respectively. While sequential occurrences of MZLs have been previously documented, this represents a concurrent diagnosis of two distinct MZLs.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-10-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149118271","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Primary ALK-positive anaplastic large cell lymphoma of the urinary bladder in a pediatric patient: a case report and review of literature.","authors":"Navdeep Kaur, Akhila Aravind, Sarika P Jain","doi":"10.1007/s12308-026-00737-y","DOIUrl":"10.1007/s12308-026-00737-y","url":null,"abstract":"<p><strong>Background: </strong>Primary lymphomas of the urinary bladder are rare, most of which are low-grade B-cell lymphomas. Primary anaplastic large cell lymphoma (ALCL) with bladder involvement is extremely rare, with most cases seen in adult patients. Here, we report a case of ALK-positive ALCL in a 14-year-old male.</p><p><strong>Case presentation: </strong>A 14-year-old male presented with several months of gross hematuria that had become painful. Computed tomography (CT) urogram showed a mass-like thickening of the anterior bladder wall. Cystoscopy showed a 3.5-cm pedunculated lesion at the bladder dome; transurethral excision was performed. Histology revealed sheets of large pleomorphic cells with horseshoe-shaped nuclei and brisk mitotic activity. Tumor cells were positive for CD4, CD5, and CD30 (diffuse/strong) and negative for CD45. ALK immunostain showed nuclear and cytoplasmic staining. A diagnosis of ALK + ALCL was made. Staging CT was negative for disease. The bone marrow was uninvolved. The patient received multi-agent chemotherapy including brentuximab vedotin. A 2-month follow-up cystoscopy showed a well-healed resection site. Seven months after completing therapy, the patient had recurrent hematuria. Cystoscopy revealed an ulcerated lesion, but biopsies were reactive and showed no lymphoma. The patient remains under surveillance with urology and oncology.</p><p><strong>Conclusion: </strong>Primary bladder ALK + ALCL is exceedingly rare in children. Uniform CD30 positivity and ALK positivity are the key diagnostic clues.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13615910/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149065855","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinicopathological spectrum and outcomes of biopsy-proven post-transplant lymphoproliferative disorder after kidney transplantation.","authors":"Yagmur Tahillioglu, Safak Mirioglu, Yunus Catma, Rabia Hacer Hocaoglu, Ali Yilmaz Altay, Metban Mastanzade, Ahmet Burak Dirim, Ayse Serra Ucar, Gulcin Yegen, Ozgur Akin Oto, Savas Ozturk, Aydin Turkmen, Sevgi Kalayoglu Besisik, Halil Yazici","doi":"10.1007/s12308-026-00738-x","DOIUrl":"10.1007/s12308-026-00738-x","url":null,"abstract":"<p><strong>Background/aim: </strong>Post-transplant lymphoproliferative disorder (PTLD) is a malignancy that has gained increasing importance in transplantation practice due to the rising frequency of transplant procedures and the widespread use of immunosuppressive therapies. This study aimed to evaluate the clinical, laboratory, and treatment characteristics, as well as survival outcomes, of patients diagnosed with post-transplant PTLD following kidney transplantation.</p><p><strong>Materials and methods: </strong>In this retrospective study, a total of 21 patients who underwent kidney transplantation between 1977 and 2025 and were subsequently diagnosed with PTLD were analyzed. Demographic data, primary kidney diseases, transplant characteristics, immunosuppressive regimens, PTLD subtypes, laboratory parameters at diagnosis, treatment approaches, and follow-up outcomes were assessed. Patient survival was evaluated, and exploratory analyses of selected prognostic indicators were performed.</p><p><strong>Results: </strong>The mean age of patients diagnosed with PTLD was 48 ± 15 years, and 38.1% were female. The most common PTLD subtype was diffuse large B-cell lymphoma (DLBCL) (66.7%). Extranodal involvement was present in 81% of patients. The median follow-up duration after PTLD diagnosis was 18 months (3-43). In univariate Cox regression analysis, sex, age at transplantation, age at diagnosis, clinical and laboratory parameters, donor characteristics, immunosuppressive therapy, and clinical scores including the renal-PTLD index and Hemoglobin, albumin, lymphocyte, and platelet (HALP) score, were not significantly associated with overall survival. International Prognostic Index (IPI) data were available for only 12 of 21 patients (8 low-risk and 4 high-risk). In this small subgroup, higher IPI category showed a trend toward increased mortality risk in univariate analysis (HR: 8.047; 95% CI: 0.830-77.996; p = 0.072). Exploratory Kaplan-Meier analysis showed shorter estimated median survival in the high-IPI group than in the low-IPI group (3 months [95% CI: 0-8.4] vs. 42 months [95% CI: 13.6-70.4]; log-rank p = 0.029); however, this finding was based on a limited number of patients.</p><p><strong>Conclusion: </strong>PTLD is a rare but serious complication following kidney transplantation, associated with substantial morbidity and mortality. Although this study was not powered to identify definitive prognostic factors, exploratory analyses suggested shorter survival among patients with higher IPI categories, supporting further evaluation of IPI in larger multicenter cohorts.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149065777","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Concurrent follicular lymphoma and follicular dendritic cell sarcoma: composite neoplasm or transdifferentiation?","authors":"Mahsa Hajizadeh, Jinbo Fan, Jeffrey W Craig, Ifeyinwa E Obiorah","doi":"10.1007/s12308-026-00736-z","DOIUrl":"10.1007/s12308-026-00736-z","url":null,"abstract":"<p><p>Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm, and its synchronous occurrence with follicular lymphoma (FL) has not been reported. Whether composite tumors arise independently or through clonal evolution remains unclear. A 52-year-old woman presented with night sweats, weight loss, and an obstructing hepatic flexure colonic mass with mesenteric disease. Biopsy showed two tumor populations: an atypical B cell infiltrate positive for CD20, CD10, focal BCL6, and weak/focal MUM1, with kappa predominance and Ki-67 of 20-50%, consistent with follicular large B cell lymphoma/FL grade 3B, and epithelioid-to-spindled cells expressing CD21, CD23, CD35, CXCL13, and D2-40 and lacking B cell and epithelial markers, supporting FDCS. Fluorescence in situ hybridization demonstrated shared BCL6 rearrangements, IGH and MYC copy-number gains in both components, IGH rearrangement testing was negative, and next-generation sequencing identified alterations in JAK1, APC, FGFR4, NOTCH3, and TSC1. This case highlights biologic challenges in distinguishing transdifferentiation from synchronous neoplasms.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13615919/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149065825","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Acute myeloid leukemia arising in treated chronic lymphocytic leukemia/small lymphocytic lymphoma: a challenge in analysis of lineage relationship.","authors":"Aruna Rangan, Min Shi, Paul Ollila, Patricia T Greipp, Rong He, David S Viswanatha","doi":"10.1007/s12308-026-00734-1","DOIUrl":"10.1007/s12308-026-00734-1","url":null,"abstract":"<p><p>Herein, we report a 75-year-old female with a history of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) post obinutuzumab and venetoclax therapy, presenting with CD4+/CD56+/CD123+ acute myeloid leukemia (AML) with histiocytic morphology. The unusual morphology triggered a preliminary diagnosis of CLL transdifferentiation to histiocytic sarcoma (HS); however, a comprehensive workup led to the correct diagnosis of de novo AML. This case showcases the utility of ancillary studies in the work up of secondary myeloid neoplasms post B-cell lymphomas and underlines pitfalls to consider for accurate characterization of these complex malignancies.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149008989","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}