{"title":"Novel case of cytoplasmic CD3 positive, surface CD4 positive high-grade B-cell lymphoma with MYC rearrangement.","authors":"Vladimir Miltchev, Natalia Golardi","doi":"10.1007/s12308-026-00728-z","DOIUrl":"10.1007/s12308-026-00728-z","url":null,"abstract":"<p><p>High-grade B-cell lymphoma, not otherwise specified (HGBCL-NOS), is an aggressive mature B-cell neoplasm whose diagnosis remains challenging due to significant morphologic overlap with diffuse large B-cell lymphoma and Burkitt lymphoma, and the absence of defining molecular alterations. Aberrant antigen expression can further complicate diagnoses and may result in lineage ambiguity. We report a unique case of HGBCL-NOS with MYC rearrangement demonstrating aberrant cytoplasmic CD3 and surface CD4 expression, leading to an initial misdiagnosis as a T-cell lymphoma at an outside hospital. A 50-year-old man with a previous diagnosis of peripheral T-cell lymphoma presented to our institution with central nervous system symptoms and a persistent bladder mass. Evaluation by bladder biopsy and cerebrospinal fluid flow cytometry revealed a high-grade B-cell neoplasm with diffuse CD10 and CD79a expression, patchy CD20 positivity, strong PAX5 expression, lambda light-chain restriction, Ki-67 approaching 100%, and flow cytometry confirmed expression of cytoplasmic CD3 and dim surface CD4 expression in a clonal B-cell population. Fluorescence in situ hybridization demonstrated a t(8;14)(q24;q32) MYC::IGH rearrangement without BCL2 or BCL6 rearrangements. This extremely rare phenotype has not previously been described. This case expands the spectrum of aberrant immunophenotypes reported in both B-cell lymphomas and HGBCL-NOS and highlights the importance of comprehensive lineage assessment in lymphomas with unusual antigen expression.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13522020/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148842042","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":"Flow cytometric detection of isolated central nervous system relapse in acute myeloid leukemia under FLT3-targeted therapy.","authors":"Chen Glait Santar, Odelia Amit, Ben Zion Katz","doi":"10.1007/s12308-026-00727-0","DOIUrl":"https://doi.org/10.1007/s12308-026-00727-0","url":null,"abstract":"<p><p>Central nervous system (CNS) relapse in acute myeloid leukemia (AML) is rare in adults and infrequently occurs as an isolated manifestation following allogeneic hematopoietic stem cell transplantation. We report a 68-year-old man with FLT3-ITD-mutated AML with monocytic differentiation who achieved sustained morphological and molecular remission following induction chemotherapy, consolidation, allogeneic transplantation, and FLT3 inhibitor maintenance therapy. Approximately 18 months after diagnosis, the patient developed isolated CNS relapse despite continued bone marrow remission and complete donor chimerism. Cerebrospinal fluid (CSF) flow cytometry identified leukemic blasts, and PCR detected FLT3-ITD, confirming CNS involvement. Following intrathecal chemotherapy, systemic therapy, gilteritinib treatment, and a second transplantation, the patient developed recurrent CNS disease with optic nerve involvement and vision loss. Notably, repeat CSF studies demonstrated persistent leukemic infiltration detectable by flow cytometry despite repeated negative FLT3-ITD PCR results, suggesting immunophenotypic-molecular discordance under FLT3-targeted therapy. This case highlights the CNS as a sanctuary site in AML and underscores the critical role of CSF flow cytometry in the diagnosis and monitoring of CNS relapse, particularly when molecular studies are negative. This case provides a valuable teaching point highlighting the limitations of relying on a single diagnostic method.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-08-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148814475","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":"Acute myeloid leukemia with unusual mature lymphoid-like morphology in a subset of blasts.","authors":"Christopher Vossen, Clarissa E Jordan","doi":"10.1007/s12308-026-00726-1","DOIUrl":"10.1007/s12308-026-00726-1","url":null,"abstract":"<p><p>We report a case of newly diagnosed acute myeloid leukemia with atypical blast morphology. Peripheral blood smear showed an atypical, dimorphic population of myeloblasts, including a subset with typical morphology and a separate subset with unusual mature lymphoid-like morphology. Flow cytometric immunophenotyping was crucial in characterizing the dimorphic population as a single myeloid blast population.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13498632/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148801396","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}
Rebecca Manzo, Changqing Xia, Zhenya Tang, Joseph D Khoury, Shanxiang Zhang
{"title":"Erythroblastic sarcoma with biallelic TP53 mutations and novel NUP214:: ABL1-new finding in erythroblastic sarcoma/acute erythroid leukemia.","authors":"Rebecca Manzo, Changqing Xia, Zhenya Tang, Joseph D Khoury, Shanxiang Zhang","doi":"10.1007/s12308-026-00723-4","DOIUrl":"https://doi.org/10.1007/s12308-026-00723-4","url":null,"abstract":"<p><p>NUP214::ABL1 generates a fusion protein with constitutively activated tyrosine kinase activity and is present in rare T-lymphoblastic leukemia (ALL), B-ALL, and in rare cases of myelodysplastic neoplasm/acute myeloid leukemia (MDS/AML). Here, we for the first time reported NUP214::ABL1 in a case of erythroblastic sarcoma (ES), a rare extramedullary form of acute erythroid leukemia (AEL). A 55-year-old female with a reported three-year history of MDS presented with worsening lower extremity paresthesia. Magnetic resonance imaging revealed an intraspinal T2-T4 extradural mass. Examination of the mass biopsy supported by extensive immunoprofiling was diagnostic of ES. Fluorescence in situ hybridization (FISH) identified concurrent deletions of 5p15.2 and 5q31 (likely monosomy 5), trisomy 8, and deletion of 7q31 while absent for translocations included in AML FISH panel. Next-generation sequencing (NGS) including DNA (406 genes) and RNA (265 genes) panels revealed NUP214::ABL1 (N31; A2) and TP53 I50fs*2 (VAF 85.9%). The patient died approximately two weeks after the ES diagnosis. This informative case emphasizes the importance for pathologists to be familiar with ES as a rare extramedullary form of AEL and a potential transformation from MDS. It is critical to apply comprehensive studies including cytogenetics/FISH and NGS in rendering prompt diagnosis for optimal management.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148801428","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}
Julia Lunt, Rowan Burns, Christine Mg Schammel, Jenny Knight
{"title":"Morphologic mimicry of cutaneous T-cell lymphoma as classic Hodgkin lymphoma-a case report.","authors":"Julia Lunt, Rowan Burns, Christine Mg Schammel, Jenny Knight","doi":"10.1007/s12308-026-00706-5","DOIUrl":"10.1007/s12308-026-00706-5","url":null,"abstract":"<p><strong>Background: </strong>Cutaneous T-cell lymphoma (CTCL) and classic Hodgkin lymphoma (cHL) are distinct lymphoid neoplasms with differing cells of origin and immunophenotypes. Morphological overlap between these entities can pose significant diagnostic challenges, particularly in patients with a concurrent or prior lymphoma diagnosis.</p><p><strong>Case presentation: </strong>A 67-year-old male with a history of cHL and mycosis fungoides/lymphomatoid papulosis presented with new PET-positive right groin lymphadenopathy. Biopsy revealed atypical cells morphologically consistent with Hodgkin/Reed-Sternberg cells. However, clonal T-cell receptor beta gene rearrangement and immunophenotypic findings were inconsistent with cHL, and integration of these results with the patient's clinical history led to a final diagnosis of nodal involvement by CTCL.</p><p><strong>Conclusion: </strong>CTCL can morphologically mimic cHL, representing a significant diagnostic pitfall. This case underscores the critical role of immunophenotyping, molecular studies, and clinical context in accurately diagnosing complex hematolymphoid neoplasms.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-08-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13468865/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148721892","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}
Soham Kale, Zbigniew Rudzki, Bernard Maybury, Kaviya Selvapandian, Gerald Langman, Claire Shannon-Lowe
{"title":"Primary pulmonary presentation extranodal NK/T-cell lymphoma, small cell variant, illustrating the value of flow EBER in situ hybridisation assay.","authors":"Soham Kale, Zbigniew Rudzki, Bernard Maybury, Kaviya Selvapandian, Gerald Langman, Claire Shannon-Lowe","doi":"10.1007/s12308-026-00725-2","DOIUrl":"https://doi.org/10.1007/s12308-026-00725-2","url":null,"abstract":"<p><p>A 57-year-old male from Guinea presented with 6 months of progressive shortness of breath, productive cough, and occasional night sweats. A lung biopsy revealed an angiocentric pulmonary infiltrate of small bland lymphocytes and lymphoepithelial lesions mimicking a MALT lymphoma. The infiltrate expressed CD3, CD56, and Epstein-Barr virus-encoded RNA with little Ki-67 staining. Blood EBV copies were 519 IU/mL. FDG-PET scan showed no uptake outside the thorax. A diagnosis of small-cell variant pulmonary NK/T-cell lymphoma was made. Following asparaginase/cisplatin-based treatment, the patient developed recurrent cough, and nasal biopsy suggested lymphoma relapse. Following transplant conditioning, the patient developed symptoms of fever and cough, with rising blood EBV load. Lymphoma progression was suspected. A flow RNA assay demonstrated a small population of EBER + CD8 + T-cells in blood. NK/T-cell lymphoma usually present with sino-nasal symptoms. The small-cell variant is rare. The diagnosis is challenging when the clinical presentation and cytological features are atypical. POT1 variants have not been reported in ENKTL to our knowledge, but germline variants have been associated with lymphomas. A multicolour EBER hybridisation flow assay can help identify the cellular origin of EBV reactivation.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148690335","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":"Leukemic presentation of ALK-positive anaplastic large cell lymphoma in an adult patient with hemophagocytic lymphohistiocytosis.","authors":"Mark Oliver, Jenny Byrd, Eduard Matkovic","doi":"10.1007/s12308-026-00724-3","DOIUrl":"10.1007/s12308-026-00724-3","url":null,"abstract":"<p><p>Systemic ALK + anaplastic large cell lymphoma (ALCL) is a CD30-positive T cell neoplasm that commonly affects lymph nodes with frequent involvement of extranodal sites. A leukemic phase with peripheral blood involvement is extremely rare, and therefore, recognition of this uncommon phenomenon is crucial to avoid misdiagnosis and delayed treatment. We report a case of a previously healthy adult patient who presented with peripheralizing ALK-positive ALCL accompanied by hemophagocytic lymphohistiocytosis (HLH). Comprehensive ancillary studies were essential for establishing the diagnosis and determining blood involvement.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13424219/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148622285","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}
Gökhan Burul, Büşra Tuğçe Tonyalı, İsa Yalçınkaya, Sena Beyazyıldırım, Merve İnceman, İstemi Serin
{"title":"HIV-negative KSHV/HHV8-associated multicentric Castleman disease with concurrent Kaposi sarcoma and POEMS-like manifestations.","authors":"Gökhan Burul, Büşra Tuğçe Tonyalı, İsa Yalçınkaya, Sena Beyazyıldırım, Merve İnceman, İstemi Serin","doi":"10.1007/s12308-026-00722-5","DOIUrl":"https://doi.org/10.1007/s12308-026-00722-5","url":null,"abstract":"<p><strong>Introduction: </strong>Castleman disease (CD) is a heterogeneous lymphoproliferative disorder with unicentric and multicentric forms. Multicentric CD (MCD) is etiologically classified into idiopathic MCD (iMCD), POEMS-associated MCD, and Kaposi sarcoma-associated herpesvirus (KSHV/HHV-8)-associated MCD, and it typically presents with systemic inflammation. KSHV/HHV-8-associated MCD is most commonly seen in HIV-positive patients, although it may rarely occur in HIV-negative individuals.</p><p><strong>Case presentation: </strong>We report a case of a 65-year-old HIV-negative woman diagnosed with mixed-type KSHV/HHV-8-associated MCD accompanied by Kaposi sarcoma and POEMS-like clinical features. The patient presented with generalized lymphadenopathy, splenomegaly, cytopenias, hypercalcemia, and monoclonal IgG lambda gammopathy. PET-CT revealed widespread hypermetabolic lymphadenopathy and a sclerotic bone lesion in the left humerus. Bone marrow examination was normocellular and showed polytypic plasma cell proliferation. Lymph node biopsy demonstrated HHV-8 (LANA-1) positivity consistent with mixed-type MCD and Kaposi sarcoma. HHV-8 DNA positivity was also confirmed.</p><p><strong>Conclusion: </strong>The patient was treated with a regimen including daratumumab, bortezomib, cyclophosphamide, and dexamethasone, achieving a clinical and hematologic response after the third cycle. This case highlights that KSHV/HHV-8-associated MCD may occur in HIV-negative individuals and may present concurrently with Kaposi sarcoma and POEMS-like manifestations, representing a rare diagnostic challenge.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148594363","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}
Nour Louati, Imen Krichen, Ines Jedidi, Ikram Ben Amor
{"title":"Auer rod-like inclusions in marginal zone lymphoma: a potential diagnostic pitfall.","authors":"Nour Louati, Imen Krichen, Ines Jedidi, Ikram Ben Amor","doi":"10.1007/s12308-026-00721-6","DOIUrl":"https://doi.org/10.1007/s12308-026-00721-6","url":null,"abstract":"<p><p>A 47-year-old man presented with leukocytosis and absolute lymphocytosis. Peripheral blood smear examination revealed atypical lymphoid cells containing striking Auer rod-like cytoplasmic inclusions, creating a strong morphologic impression of acute promyelocytic leukemia. However, flow cytometry demonstrated a mature monoclonal B cell population lacking myeloid differentiation, and bone marrow biopsy established the diagnosis of marginal zone lymphoma. This rare case highlights a major diagnostic pitfall and underscores that Auer rod-like inclusions are not exclusively restricted to myeloid neoplasms.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148581132","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}
Michael T Mulvena, Sam Sadigh, Eric D Jacobsen, Sarah J Wu
{"title":"CD5+ CD10+ lymphoplasmacytic lymphoma with cutaneous histologic transformation in a post-transplant setting.","authors":"Michael T Mulvena, Sam Sadigh, Eric D Jacobsen, Sarah J Wu","doi":"10.1007/s12308-026-00720-7","DOIUrl":"10.1007/s12308-026-00720-7","url":null,"abstract":"<p><p>Lymphoplasmacytic lymphoma (LPL) is a lymphoproliferative neoplasm characterized by small plasmacytic cells infiltrating the bone marrow and is typically associated with IgM paraprotein detection. In rare cases, LPL may undergo histologic transformation (HT) to a more aggressive, large B cell lymphoma, such as diffuse large B-cell lymphoma (DLBCL). HT with cutaneous involvement is exceedingly rare. Here, we report a case of cutaneous involvement by a CD5+ CD10+ large B cell lymphoma in a post-transplant setting, initially attributed to follicular lymphoma but determined to be LPL with additional molecular testing. These findings highlight a potential diagnostic pitfall that was resolved by using molecular techniques in tandem with morphological observations.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"19 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148474082","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}