Journal of clinical and experimental hematopathology : JCEH最新文献

{"title":"Clinicopathological features of gastric Langerhans cell histiocytosis and a literature review.","authors":"Xue Chen, Yunjin Wu, Xueli Wang, Muye Yang, Xiaoxiang Gao, Suxia Zhang, Huihui Sun, Yunhui Zhang, Hongmei Yi, Yu Zeng","doi":"10.3960/jslrt.25082","DOIUrl":"10.3960/jslrt.25082","url":null,"abstract":"<p><p>Objective This study aims to review the clinicopathological features, molecular profiles, and clinical behavior of gastric LCH to increase diagnostic accuracy and improve the understanding of its prognosis. Methods We analyzed five cases of gastric LCH and integrated clinical, endoscopic, and pathological data. Diagnoses were confirmed via immunohistochemistry (S-100, CD1a, and Langerin). BRAF-V600E mutation status was assessed by PCR in three patients. A literature review of 17 additional cases was included for a comprehensive analysis. Results Patients presented with nonspecific symptoms such as abdominal pain. Endoscopy predominantly revealed solitary polyps (<1 cm) or mucosal thickening, primarily in the gastric body. Histology revealed characteristic neoplastic cells with grooved nuclei and eosinophilic cytoplasm, accompanied by eosinophil infiltration. All patients were positive for S-100, CD1a, and Langerin. The BRAF-V600E mutation was detected in all three tested cases. Systemic involvement occurred in two patients. Treatment ranged from surveillance to chemotherapy. All patients were alive after follow-up (8-62 months), with most patients showing no progression. Conclusion Gastric LCH typically presents as a small, solitary polyp. S-100, CD1a, and Langerin positivity was observed by immunohistochemistry. The high frequency of BRAF-V600E mutations provides a diagnostic adjunct and potential therapeutic target. Gastric LCH patients have a good prognosis and long overall survival when the cancer is a localized lesion. However, systemic involvement may necessitate chemotherapy, and regular follow-up is recommended.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":" ","pages":"11-18"},"PeriodicalIF":1.4,"publicationDate":"2026-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13160645/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147328868","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":"Pseudoprogression of bladder extramedullary disease of multiple myeloma following the treatment with elranatamab, an anti-BCMA/CD3 bispecific antibody.","authors":"Kiyoka Ito, Haruya Okamoto, Chika Maekura, Takahiro Fujino, Taku Tsukamoto, Shinsuke Mizutani, Yuji Shimura, Junya Kuroda","doi":"10.3960/jslrt.25080","DOIUrl":"10.3960/jslrt.25080","url":null,"abstract":"","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":" ","pages":"103-107"},"PeriodicalIF":1.4,"publicationDate":"2026-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13160647/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147328944","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":"Efficacy of venetoclax combined with azacitidine for advanced blastic plasmacytoid dendritic cell neoplasm: experience from two Japanese cases.","authors":"Asuka Shibamiya, Chikako Ohwada, Yuka Tsutsui, Akane Tanaka, Reiko Watanabe, Taro Akatsuka, Toshihisa Hamada, Makoto Sugaya, Yuichiro Hayashi, Takayuki Shiomi, Chiaki Nakaseko","doi":"10.3960/jslrt.25056","DOIUrl":"10.3960/jslrt.25056","url":null,"abstract":"<p><p>Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy with no established standard treatment. Recently, the combination of venetoclax (VEN) and azacitidine (Aza) has shown promising efficacy in elderly or unfit patients; however, clinical data in Japanese patients remain limited. We report two Japanese cases with BPDCN that responded favorably to VEN plus Aza therapy. The first case involved a 50-year-old man who relapsed shortly after Hyper-CVAD/MA therapy with leukemic transformation. Despite requiring dose reductions due to prolonged cytopenia and infections, he achieved a complete response after eight cycles of VEN plus Aza. The second case was an 81-year-old man with widespread skin and bone marrow lesions. He showed a marked clinical response after only 7 days of treatment, although therapy was discontinued due to COVID-19 infection. Both cases demonstrate that VEN plus Aza therapy can induce rapid and clinically meaningful responses even in short or attenuated regimens in patients with refractory or elderly BPDCN. However, cytopenias and infections are significant limitations, warranting that individualized dosing may be necessary depending on patient characteristics, including ethnicity and body size. Our findings support the consideration of VEN plus Aza as a feasible therapeutic option for patients with BPDCN.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":"66 1","pages":"37-44"},"PeriodicalIF":1.4,"publicationDate":"2026-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13160642/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147596888","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":"Discordant lymphoma consisting of splenic marginal zone lymphoma and diffuse large B-cell lymphoma in a hepatitis B virus carrier suggests virus-associated lymphomagenesis.","authors":"Takumi Kaieda, Kenichi Makishima, Sakurako Suma, Yuya Sasaki, Yasuhito Suehara, Hajime Yasuda, Ryota Matsuoka, Fumiaki Matsumura, Tatsuhiro Sakamoto, Keiichiro Hattori, Takayasu Kato, Toru Nanmoku, Naoki Kurita, Hidekazu Nishiki, Mamiko Sakata-Yanagimoto","doi":"10.3960/jslrt.25068","DOIUrl":"10.3960/jslrt.25068","url":null,"abstract":"","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":"66 1","pages":"69-74"},"PeriodicalIF":1.4,"publicationDate":"2026-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13160641/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147597618","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":"Fine-needle aspiration cytology of Hodgkin-like ALK-negative anaplastic large cell lymphoma: Cytomorphological clues and the diagnostic value of subtle background cell atypia.","authors":"Hidetoshi Satomi, Risa Yoshioka, Sayoko Tsuzaki, Masanori Kitamura, Satoshi Tanada, Kennosuke Karube, Keiichiro Honma","doi":"10.3960/jslrt.25060","DOIUrl":"10.3960/jslrt.25060","url":null,"abstract":"<p><p>Hodgkin-like ALK-negative anaplastic large cell lymphoma (ALCL) is a morphologically challenging variant that closely resembles classical Hodgkin lymphoma (CHL), particularly in cytological specimens. Fine-needle aspiration (FNA) cytology of Hodgkin-like ALCL has been rarely reported; therefore, detailed cytomorphological analysis is valuable for diagnostic practice and education. We report a rare case of Hodgkin-like ALCL in a 75-year-old Japanese man who presented with a progressively enlarging right axillary mass. Initial FNA cytology revealed scattered large atypical cells with Hodgkin and Reed-Sternberg-like morphology in an inflammatory background, strongly suggesting CHL. However, retrospective review identified subtle yet important diagnostic clues that had initially been overlooked, including nuclear irregularities and atypia in small- to medium-sized background cells, distinctive distribution patterns of atypical cells, and the presence of vascular stromal fragments.Histopathologic examination confirmed ALCL with a characteristic immunophenotype (CD30+, CD15+, PAX5-, Granzyme B+, CD4+, CD25+, EMA+, ALK-). The absence of PAX5 expression was a key discriminator, effectively excluding CHL despite the striking morphological resemblance. This case highlights the diagnostic pitfalls in differentiating Hodgkin-like ALCL from CHL in cytological preparations and emphasizes the importance of systematically evaluating background cellular composition beyond the conspicuous large atypical cells. Careful assessment of nuclear morphology and cell distribution patterns can provide valuable diagnostic clues to prevent misdiagnosis. This rare cytological presentation offers critical educational insights for cytopathologists and underscores the diagnostic value of FNA cytology, particularly when integrated with immunophenotypic analysis and retrospective morphological review in evaluating morphologically challenging lymphoid malignancies.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":" ","pages":"56-62"},"PeriodicalIF":1.4,"publicationDate":"2026-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13160651/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145758865","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":"Cooccurrence of myelodysplastic neoplasm and myeloid sarcoma with background mediastinal germ cell tumor: an autopsy case.","authors":"Yuto Kaimi, Toshihiro Haga, Yuhei Yamaguchi, Kazuki Sudo, Kan Yonemori, Yasushi Yatabe","doi":"10.3960/jslrt.25059","DOIUrl":"10.3960/jslrt.25059","url":null,"abstract":"<p><p>Hematological malignancy (HM) is developed in 2-6% patients with primary mediastinal germ cell tumor (MGCT). Symptoms of HM associated with MGCT usually appear after diagnosis of MGCT. Patients with MGCT underlying HM have poor prognosis. We report an extremely rare autopsy case of simultaneous presentation of MGCT, myelodysplastic neoplasm (MDS) with low blasts, and myeloid sarcoma (MS). A 27-year-old man presented with intermittent fever, general fatigue, and cough. Computed tomography revealed 11 cm mass in anterior mediastinum, hepatosplenomegaly, and gastric, pericholedochal, and mesenteric lymphadenopathies. The mass compressed the left pulmonary artery. Laboratory examination revealed thrombocytopenia (59,000 /mm³). Serological tests revealed elevated alpha-fetoprotein 7,219 ng/mL and human chorionic gonadotropin 899 IU/L. Bone marrow aspiration smear showed presence of micromegakaryocytes and blast count of 1.2%. G-banding detected monosomy 13 and complex karyotype. The autopsy revealed that MGCT was mainly composed of teratoma and also included yolk sac tumor, seminoma, and choriocarcinoma components. Transformation to acute myeloid leukemia was not observed in the bone marrow but CD33 and MPO-positive immature myeloid blasts proliferated in the lymph nodes, liver, spleen, and small intestine. Loss of p53 expression was observed in bone marrow and extramedullary immature myeloid blasts. Next gene sequencing detected TP53 c.919+3del splice site variant and KRAS N116H. It is important to consider HMs when extramediastinal lesions or thrombocytopenia appear in patients with MGCT.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":" ","pages":"52-55"},"PeriodicalIF":1.4,"publicationDate":"2026-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13160639/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147328950","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":"Pseudoprogression of extramedullary lesions during elranatamab therapy in relapsed or refractory multiple myeloma.","authors":"Akihiro Kawatsuki, Hiroki Kobayashi, Shoka Kimura, Soichiro Fushimi, Yuki Doi, Yuki Fujiwara, Tomoko Yamasaki, Takeru Asano, Yasushi Hiramatsu","doi":"10.3960/jslrt.25065","DOIUrl":"10.3960/jslrt.25065","url":null,"abstract":"<p><p>Pseudoprogression comprises a transient increase in tumor burden caused by immune cell infiltration or inflammatory responses after immunotherapy, including chimeric antigen receptor T-cell therapy and the use of bispecific antibodies. Although pseudoprogression has been reported following several immunotherapy types, cases of pseudoprogression associated with elranatamab remain rare. Herein, we describe the case of a 67-year-old woman with relapsed/refractory multiple myeloma who developed pseudoprogression of extramedullary lesions following treatment with elranatamab. On day 4 of therapy, the patient presented with left periorbital swelling and lagophthalmos. Computed tomography (CT) revealed the enlargement of multiple extramedullary lesions in this patient. Dexamethasone administration improved the ocular symptoms. Lagophthalmos recurred after administration of the second dose of elranatamab but responded to dexamethasone. On day 13, a CT-guided biopsy of the chest wall lesion revealed dense T cell infiltration within the tumor tissue, consistent with pseudoprogression rather than true progression. The patient's serum M-protein levels decreased within 2 weeks of treatment initiation. At 2 months, follow-up CT demonstrated a reduction in the extramedullary lesions, and the patient achieved a very good partial response. This case highlights that pseudoprogression may occur during elranatamab therapy, similar to that which may occur with other T-cell-redirecting immunotherapies. Differentiating pseudoprogression from true progression is clinically important, as misinterpretation may lead to premature discontinuation of effective therapy. Careful clinical evaluation, integration of imaging findings, and histopathological confirmation (whenever feasible) are essential for the appropriate management of such cases.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":"66 1","pages":"63-68"},"PeriodicalIF":1.4,"publicationDate":"2026-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13160643/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147596821","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":"CD83 as a novel prognostic biomarker for diffuse large B-cell lymphoma arising in immune deficiency/dysregulation among rheumatoid arthritis patients treated with methotrexate.","authors":"Keisuke Sawada, Takumi Takahashi, Yuki Fukumura, Hiroko Onagi, Karin Ashizawa, Takahisa Yamashita, Wataru Yamamoto, Natsuko Takayanagi, Akiko Adachi, Makoto Kashimura, Takayuki Tabayashi, Jun-Ichi Tamaru, Morihiro Higashi, Shuji Momose","doi":"10.3960/jslrt.25087","DOIUrl":"10.3960/jslrt.25087","url":null,"abstract":"<p><p>Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of methotrexate-associated lymphoma arising in immune deficiency/dysregulation (MTX-associated IDD-DLBCL) among rheumatoid arthritis patients treated with MTX and is characterized by frequent spontaneous regression (SR) after MTX withdrawal. However, some patients do not achieve SR and have poor outcomes. Epstein-Barr virus (EBV) infection correlates with frequent SR but does not fully explain clinical heterogeneity. We investigated prognostic factors irrespective of EBV infection status. We analyzed 21 MTX-associated IDD-DLBCL cases applying the nCounter PanCancer Immune Profiling Panel and immunohistochemistry (IHC) to identify predictors of non-SR cases. Ten patients were classified as SR and 11 as non-SR. Gene expression profiling revealed higher expression of CD83, ICOSLG, IL21R, BCL6, CD40, PAX5, CXCR5, CD79A, DMBT1, and TNFRSF13C in non-SR cases. We therefore focused on CD83, which showed the highest fold change and the most significant P value among these markers. Although CD83 is reported to be a surface marker of mature dendritic cells, IHC analysis revealed that CD83 was more frequently expressed on tumor cells than on dendritic cells. High CD83 IHC positivity (≥15%) in tumor cells correlated with mRNA levels and predicted non-SR after MTX withdrawal. Multivariate analysis identified CD83 IHC high expression as an independent predictor of non-SR cases. High CD83 expression is an independent prognostic factor in MTX-associated IDD-DLBCL, and combined evaluation may refine risk stratification and guide clinical decisions.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":"66 1","pages":"27-36"},"PeriodicalIF":1.4,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13160655/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147597696","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":"Synchronous diffuse large B-cell lymphoma and gynecological malignancy treated with paclitaxel and carboplatin: A report of two cases.","authors":"Yuma Nato, Kana Miyazaki, Tsuyoshi Matsumoto, Kota Okamoto, Yasuhiro Teishikata, Akihide Nakamura, Hiroshi Imai, Takashi Watanabe, Isao Tawara, Motoko Yamaguchi","doi":"10.3960/jslrt.26006","DOIUrl":"10.3960/jslrt.26006","url":null,"abstract":"<p><p>Diffuse large B-cell lymphoma (DLBCL) can occur at various sites and is rarely diagnosed as synchronous multiple primary malignancies. A treatment approach for this synchronous malignancy has not been established. Here, we report two cases of synchronous DLBCL and gynecological malignancy treated with the paclitaxel and carboplatin (TC) regimen. Patient #1: A 67-year-old female presented with right axillary lymphadenopathy. A computed tomography (CT) revealed a hepatic mass and an ovarian tumor. Biopsy of the lymphadenopathy diagnosed DLBCL (stage I). Ovarian and peritoneal biopsies confirmed ovarian cancer (stage IVB, peritoneal dissemination, and liver metastasis). She received 6 cycles of TC chemotherapy for ovarian cancer, followed by surgery (pT3cN0M1) and an additional 2 cycles of TC. A complete metabolic response (CMR) of both malignancies was confirmed 1 year later. Patient #2: A 77-year-old female presented with left knee discomfort. Biopsy of a left femoral tumor diagnosed DLBCL (stage I). PET-CT revealed abnormal fluorodeoxyglucose uptake in the left femur and in the uterus, with an endometrial biopsy confirming endometrial cancer. She received radiation therapy for DLBCL, followed by surgery for endometrial cancer (pT1bNxM0). Postoperatively, 6 cycles of TC chemotherapy were performed, and she achieved a CMR. No Grade 3/4 nonhematologic adverse events were observed in both patients. In conclusion, the TC regimen was safely administered in our two patients. In one of them, TC therapy alone was effective for DLBCL. The clinical courses may provide a valuable reference for the management of similar patients.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":"66 2","pages":"136-141"},"PeriodicalIF":1.4,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13472208/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148348753","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":"Real-world treatment outcomes of peripheral T-cell lymphoma in the era of molecular targeted therapy.","authors":"Masuho Saburi, Takumi Nishikawa, Kuniaki Maehara, Hiroyuki Takata, Masanori Sakata, Hitohiro Sasaki, Miyuki Abe, Kazuhiro Kohno, Takahiro Kusaba, Shogo Urabe, Yoshiyuki Kondo, Toshiyuki Nakayama, Eiichi Ohtsuka","doi":"10.3960/jslrt.26009","DOIUrl":"10.3960/jslrt.26009","url":null,"abstract":"","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":"66 2","pages":"182-188"},"PeriodicalIF":1.4,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13472246/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148348715","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}