{"title":"EBV-positive inflammatory follicular dendritic cell sarcoma of fibroblastic reticular cell phenotype arising in intrapancreatic accessory spleen.","authors":"Akihiko Sugimoto, Rintaro Ohe, Shinichi Taniwaki, Megumi Sato, Yuka Kondo, Sayaka Sumiyoshi, Sachiko Minamiguchi","doi":"10.3960/jslrt.25036","DOIUrl":"10.3960/jslrt.25036","url":null,"abstract":"<p><p>Epstein-Barr Virus (EBV)-positive inflammatory follicular dendritic cell sarcoma (EBV-IFDCS) is a rare tumor of low malignant potential. Abundant lymphocytes and plasma cells often infiltrate into this tumor tissue with lymphoid follicles and sarcoid-like granulomas, so that the differential diagnosis of this tumor includes inflammatory lesions such as IgG4-related disease, morphologically. This tumor typically arises in the spleen or liver, and shows follicular dendritic cell (FDC)-like immunophenotype. Here we presented a case of EBV-IFDCS arising in intrapancreatic accessory spleen, which was preoperatively considered as a pancreatic tumor. Immunohistochemistry revealed its fibroblastic reticular cell-like phenotype, namely alpha SMA, l-caldesmon, tenascin C, and transglutaminase II positivity, while FDC marker expression such as CD21 and clusterin was focal. Positivity for EBV-encoded small RNA in situ hybridization (EBER ISH) distinguished it from inflammatory lesions or conventional FDC sarcoma. The differential diagnosis of EBV-IFDCS is broad, ranging from non-neoplastic lesions to sarcoma, particularly when it arises in unusual sites. Therefore, careful gross and microscopic examination and adequate immunohistochemistry application including EBER ISH are important to diagnose it properly.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":"65 3","pages":"202-205"},"PeriodicalIF":1.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12598720/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145188228","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}
{"title":"Pemigatinib therapy in myeloid/lymphoid neoplasm with FGFR1 rearrangement manifesting as aggressive B-cell lymphoma.","authors":"Kenichi Ito, Kazuhiko Hirano, Naohiro Sekiguchi","doi":"10.3960/jslrt.25027","DOIUrl":"10.3960/jslrt.25027","url":null,"abstract":"<p><p>Myeloid/lymphoid neoplasm with FGFR1 rearrangement (MLNF) is one of the rare hematologic malignancies with variable clinical presentations, including a chronic phase resembling myeloproliferative neoplasms and an acute phase presenting as myeloid/lymphoid leukemia or lymphoma, often associated with eosinophilia. The prognosis of MLNF has been poor, and allogeneic hematopoietic stem cell transplantation (HSCT) remains the only curative approach, although selective FGFR1 inhibitors, such as pemigatinib, have recently emerged as therapeutic options. Nevertheless, the efficacy of pemigatinib in aggressive or blast-phase MLNF remains unclear. Herein, we report a case of a 67-year-old woman initially diagnosed with Richter's syndrome. The patient achieved a complete response with six cycles of rituximab-based chemoimmunotherapy followed by acalabrutinib maintenance. Two years later, the patient developed leukocytosis, eosinophilia, and recurrent lymphadenopathy. Bone marrow examination revealed disease recurrence with marked eosinophilia, and FGFR1 rearrangement was confirmed by fluorescence in situ hybridization. The rearrangement was also confirmed from the lymph node specimen of the initial diagnosis; thus, we revised the diagnosis to relapsed MLNF. The patient received pemigatinib, but rapid disease progression was observed. The patient was ineligible for HSCT and salvage chemotherapies were unsuccessful, resulting in death four months later. The present case report highlights a rare lymphoma-like clinical presentation of MLNF, and we discuss the therapeutic options, including pemigatinib.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":"65 2","pages":"146-152"},"PeriodicalIF":1.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12351243/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144532825","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}
{"title":"Evaluation of the performance of new daratumumab-specific immunofixation electrophoresis reflex agent and the timing of M-protein measurement.","authors":"Naomi Yasuda, Masako Nishikawa, Naoyuki Yoshikawa, Yoshikazu Ono, Yutaka Yatomi, Makoto Kurano","doi":"10.3960/jslrt.25058","DOIUrl":"10.3960/jslrt.25058","url":null,"abstract":"<p><p>Accurate detection of M-proteins in multiple myeloma (MM) patients treated with daratumumab (DARA) is challenging because DARA, an IgG-κ monoclonal antibody, produces immunofixation electrophoresis (IFE) bands that mimic disease-derived M-proteins. Although the Daratumumab-Specific Immunofixation Reflex Assay (DIRA) can resolve this interference, it is not always feasible in routine practice, highlighting the need for alternative methods. D-Clean is a novel reagent designed to neutralize DARA interference and is compatible with standard IFE systems without specialized equipment. We evaluated the performance of D-Clean compared with DIRA and examined clinical factors influencing DARA interference, including the interval since the last administration and serum IgG levels. Serum samples from 20 DARA-treated patients, 32 untreated patients, and normal serum spiked with DARA were analyzed.D-Clean eliminated DARA-derived IgG-κ bands in 19 of 20 patients, yielding results comparable to DIRA. Notably, in two cases, D-Clean detected small Bence Jones-type M-proteins that were missed by DIRA, suggesting slightly higher sensitivity. Among 76 samples from DARA-treated patients, DARA-derived bands appeared only within 33 days after the last dose and were more frequent in patients with low polyclonal IgG levels. To our knowledge, this is the first study to demonstrate that serum IgG levels significantly affect the detectability of DARA-derived bands on IFE. These findings indicate that D-Clean is a reliable and practical alternative to DIRA, although the results are preliminary and require validation in multicenter studies. For clinicians, awareness of DARA dosing intervals and patients' IgG status is essential for accurate response assessment and treatment decision-making.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":"65 4","pages":"276-282"},"PeriodicalIF":1.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12883028/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145829690","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}
{"title":"A case of primary testicular adult T-cell leukemia/lymphoma (ATLL): ATLL of immune privileged site.","authors":"Reiji Muto, Toshiro Kawakita, Yoshihiro Maeda, Hiroko Muta, Takanori Negishi, Hiroaki Miyoshi","doi":"10.3960/jslrt.25052","DOIUrl":"10.3960/jslrt.25052","url":null,"abstract":"<p><p>Adult T-cell leukemia/lymphoma (ATLL) is an aggressive matured T-cell lymphoma caused by human T-lymphotropic virus type I. In advanced stage, multiple organs including bone marrow, skin, liver, spleen, gastrointestinal tract and lung may be involved, in addition to the lymph nodes and peripheral blood. However, to our best of knowledge, no case of primary testicular ATLL has been previously reported: namely primary ATLL of immune privileged site. In this paper, we present a case of primary testicular ATLL case with a poor prognosis despite being in limited stage (Stage I). The report of primary testicular T-cell lymphoma is scant, and accumulation of cases is required.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":"65 4","pages":"318-321"},"PeriodicalIF":1.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12883023/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145829689","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}
{"title":"CRS and ICANS in CAR T-cell therapy for large B-cell lymphoma.","authors":"Hideki Goto","doi":"10.3960/jslrt.25072","DOIUrl":"10.3960/jslrt.25072","url":null,"abstract":"<p><p>Chimeric antigen receptor T-cell (CAR T-cell) therapy has become an established treatment option for relapsed or refractory large B-cell lymphoma, offering durable remission in some previously incurable cases. However, the effective management of immune-mediated toxicities, particularly cytokine release syndrome (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS), remains critical to ensure safety. Treatment algorithms have been proposed based on the American Society for Transplantation and Cellular Therapy consensus grading system by the Joint Accreditation Committee of the International Society for Cellular Therapy and the European Group for Blood and Marrow Transplantation, as well as the National Comprehensive Cancer Network guidelines. Recently, early intervention to ensure that CAR T-cell therapy is performed safely has also been proposed. This review provides an update and overview of the treatment strategies for CRS and ICANS in CAR T-cell therapy for malignant lymphoma.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":"65 4","pages":"268-275"},"PeriodicalIF":1.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12884143/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145829697","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}
{"title":"Quantitative evaluation of treatment response to lenalidomide by applying fluorescence in situ hybridization for peripheral blood granulocytes in a patient with 5q- syndrome.","authors":"Hirotaka Sakai, Ikuo Miura, Ayako Arai","doi":"10.3960/jslrt.22001","DOIUrl":"https://doi.org/10.3960/jslrt.22001","url":null,"abstract":"The introduction of lenalidomide has significantly improved clinical outcomes in myelodysplastic syndrome (MDS) with isolated interstitial deletion of the long arm of chromosome 5 (del(5q)) (5q– syndrome). These days, MDS with isolated del(5q) includes cases with one additional chromosome abnormality other than monosomy 7 or del(7q), and so we need a better way to monitor tumor cells in each patient than the clinical parameters used to date. An 82-year-old woman with MDS with isolated del(5q) was treated with lenalidomide daily for 21 days in a 4-week cycle. Fluorescence in situ hybridization with CSF1R located at 5q was applied to the peripheral blood samples. Because mature lymphocytes are not involved in the MDS clone, based on the nuclear morphology, polymorphonuclear cells (PMNs) and round-shaped nuclear cells (RSNs) were separately evaluated during treatment. After a single course of treatment, the number of PMNs with del(5q) decreased; by the end of the second course of treatment, both PMNs and RSNs with del(5q) had disappeared. The dynamics of 5q– PMNs is a simple but rapid and reliable indicator to confirm the effect of lenalidomide in MDS with del(5q).","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":" ","pages":"158-163"},"PeriodicalIF":1.5,"publicationDate":"2022-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/d1/a8/jslrt-62-158.PMC9635032.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40193356","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}
{"title":"Surface CD3-negative monomorphic epitheliotropic intestinal T-cell lymphoma.","authors":"Hideharu Domoto, Takahiro Araki, Asuka Ogai, Michiko Inukai, Chien K Chen, Sakura Tomita, Kiyoshi Mukai, Naoya Nakamura","doi":"10.3960/jslrt.22005","DOIUrl":"https://doi.org/10.3960/jslrt.22005","url":null,"abstract":"<p><p>Intestinal T/NK-cell lymphomas include enteropathy-associated T-cell lymphoma (EATL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), indolent T-cell lymphoproliferative disorders of the GI tract (ITCLPD), extranodal NK/T-cell lymphoma, nasal type (ENKTL), and intestinal T-cell lymphoma NOS (ITCL-NOS). Here we describe a case of surface CD3-negative MEITL. A 63-year-old Japanese female had a tumor located in the conglomerated ileum, which formed multiple mass lesions. The resected tissue showed a diffuse infiltration of monomorphic medium-sized lymphocytes with epitheliotropism. Flowcytometry using a fresh specimen of the tumor revealed positivity for CD7, CD8, CD38, and CD56, but not surface CD3. On immunohistochemistry, the tumor showed positivity for cytoplasmic CD3, CD8, CD56, TIA-1, Granzyme B, and perforin. EBER with in situ hybridization was negative. Moreover, H3K36me3, which is negative in MEITL with SETD2-mutation, was positive. This is an important case of MEITL due to its oncogenesis.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":" ","pages":"169-174"},"PeriodicalIF":1.5,"publicationDate":"2022-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/46/4e/jslrt-62-169.PMC9635036.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40719536","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}
{"title":"Long-term remission of primary refractory ALK-positive anaplastic large cell lymphoma after allogeneic hematopoietic stem cell transplantation.","authors":"Masahiro Miyazaki, Satoshi Ichikawa, Yasushi Onishi, Noriko Fukuhara, Eijiro Furukawa, Koichi Onodera, Hisayuki Yokoyama, Ryo Ichinohasama, Hideo Harigae","doi":"10.3960/jslrt.22003","DOIUrl":"https://doi.org/10.3960/jslrt.22003","url":null,"abstract":"<p><p>ALK-positive anaplastic large cell lymphoma (ALK+ ALCL) has a favorable prognosis in general; however, some cases are resistant to chemotherapy, which leads to a poor clinical outcome. We herein report the case of a 32-year-old male with aggressive ALK+ ALCL who presented with hemorrhage from a large tumor in the duodenum and multiple tumors in the lungs, mediastinum, and peritoneal cavity. Although induction chemotherapy resulted in a marked reduction of the tumor lesions, premature progression with massive pulmonary infiltration and central nervous system invasion occurred immediately after the completion of chemotherapy. The patient was then promptly treated with brentuximab vedotin (BV) and high-dose methotrexate, which resulted in complete remission. Subsequently, he successfully underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) from an unrelated donor and has been healthy and did not relapse for more than 3 years after transplantation without any additional therapy. Allo-HSCT may be a promising treatment option for ALK+ ALCL due to its graft-versus-lymphoma effect. In addition, molecular targeting agents, such as BV, may be promising as a bridging therapy before allo-HSCT to achieve disease remission.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":" ","pages":"164-168"},"PeriodicalIF":1.5,"publicationDate":"2022-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/13/c1/jslrt-62-164.PMC9635028.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40193357","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}
{"title":"CD19 immunostaining of a stored paraffin-embedded vitrectomy cell block of intraocular lymphoma contributing to chimera antigen receptor T-cell therapy.","authors":"Toshihiko Matsuo, Takehiro Tanaka, Nobuharu Fujii, Kentaro Fujii, Eisei Kondo","doi":"10.3960/jslrt.22007","DOIUrl":"https://doi.org/10.3960/jslrt.22007","url":null,"abstract":"autologous peripheral blood hematopoietic stem cell transplantation in preconditioning with busulfan and thiotepa. 5 She maintained complete remission for 3 years until the age of 50 years when head magnetic resonance imaging showed high-signal lesions on the T2-weighted FLAIR image again in the right occipital lobe. She had 5 courses of methotrexate CHOP chemotherapy combined with rituximab for half a year, leading to complete remission, and then underwent chimera antigen receptor T-cell therapy (tisagenlecleucel) based on the CD19 expression of lymphoma cells in the preserved vitrectomy cell block (Fig. 1C). One year later, she devel-oped impending branch retinal vein occlusion with retinal blot hemorrhages, venous dilation and tortuosity in the right eye, and showed spontaneous resolution in the 4 months. Otherwise, she had shown neither retinal manifestations nor recurrent vitreous opacity throughout the course. She was systemically well for 1.5 years and maintained the best-cor-rected visual acuity of 1.5 in both eyes until the latest follow-up at the age of 52 years.","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":" ","pages":"187-189"},"PeriodicalIF":1.5,"publicationDate":"2022-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/72/f2/jslrt-62-187.PMC9635033.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40193358","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}
Ai Omi, Fumi Nomura, Shigeharu Tsujioka, Akiko Fujino, Reiko Akizuki
{"title":"Efficacy and safety of ibrutinib in relapsed/refractory CLL and SLL in Japan: a post-marketing surveillance.","authors":"Ai Omi, Fumi Nomura, Shigeharu Tsujioka, Akiko Fujino, Reiko Akizuki","doi":"10.3960/jslrt.22002","DOIUrl":"https://doi.org/10.3960/jslrt.22002","url":null,"abstract":"<p><p>Ibrutinib is approved in Japan for the treatment of chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL) based on the results of global and domestic clinical studies. Following approval, we conducted an all-case post-marketing surveillance in Japanese patients with relapsed/refractory CLL/SLL newly initiated on ibrutinib treatment between May 2016-September 2017. Of the 323 patients enrolled, the safety and efficacy analysis sets comprised 289 and 205 patients, respectively. The overall response rate with ibrutinib treatment was 64.4%, and the estimated 52-week progression-free survival (PFS) and overall survival (OS) rates were 71.7 and 79.1%, respectively. No significant difference in the PFS rate was observed among patients with and without del(17p) (P = 0.160); however, PFS was significantly longer in patients who received 1 prior line of therapy versus >1 prior lines of therapy (P = 0.007). Adverse events occurred in 74.0% of patients, and typically occurred early (≤12 weeks) after ibrutinib initiation, followed by a decline in incidence thereafter. The overall rates of infection, bleeding, and arrhythmia were 22.5, 12.8, and 4.8%, respectively. Grade ≥3 bleeding events and atrial fibrillation occurred in 2.4% of patients each. The efficacy and safety profile of ibrutinib treatment in routine clinical practice was consistent with clinical trials and previously reported domestic data.UMIN-CTR Clinical Trials Register ID: UMIN000021963.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":" ","pages":"136-146"},"PeriodicalIF":1.5,"publicationDate":"2022-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/b4/23/jslrt-62-136.PMC9635026.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40587553","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}