{"title":"Acute undifferentiated leukemia limited to neck lymph nodes and a large mediastinal mass.","authors":"Kenta Hayashino, Masayuki Matsuda, Keigo Fujishita, Jun Iwata, Miki Mizobuchi, Munenori Uemura, Kenji Yorita, Akiko Maeshima, Toshi Imai","doi":"10.3960/jslrt.22012","DOIUrl":"https://doi.org/10.3960/jslrt.22012","url":null,"abstract":"<p><p>In the 2016 update of the World Health Organization (WHO) classification of myeloid neoplasms, acute undifferentiated leukemia (AUL) was defined by a lack of lineage-specific markers. AUL has very poor prognosis and no established therapies due to its rarity. We report a case of a 31-year-old man with AUL who showed complete molecular response to an acute lymphoblastic leukemia (ALL)-based regimen and received allogeneic hematopoietic stem cell transplantation. The patient's blast cells were CD7-positive and localized to lymph nodes in the neck and to a large mediastinal mass; there was also rearrangement of the T-cell receptor delta locus. Although the tumor showed characteristics of T-cell lymphoblastic lymphoma, it was categorized as AUL based on WHO classification. This case suggests that a high-intensity conditioning regimen could be effective for rare cases of AUL that present only in the extramedullary mass, and chemotherapy for AUL should be selected based on the characteristics of the blasts.</p>","PeriodicalId":45936,"journal":{"name":"Journal of Clinical and Experimental Hematopathology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2022-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/f6/e3/jslrt-62-222.PMC9898720.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10700334","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":"Diffuse large B-cell lymphoma in the course of systemic sarcoidosis: A case report and review of 30 Japanese patients with sarcoidosis-lymphoma syndrome.","authors":"Toshihiko Matsuo, Takehiro Tanaka, Rika Omote, Toshiaki Okada, Kenji Notohara, Kazuya Okada","doi":"10.3960/jslrt.22015","DOIUrl":"https://doi.org/10.3960/jslrt.22015","url":null,"abstract":"<p><p>We report a patient with sarcoidosis who developed diffuse large B-cell lymphoma. A 71-year-old woman with persistent cough was diagnosed pathologically with sarcoidosis by resection of the right upper lung lobe with a nodule after an unsuccessful attempt of transbronchial needle aspiration for mediastinal lymphadenopathy. She was referred for an eye examination and found to have spotty retinal degeneration on the lower fundi of both eyes, together with residual macular edema and vitreous opacity in the left eye. At 76 years, she underwent cataract surgery and vitrectomy to gain a visual acuity of 0.6 in the left eye. At 77 years, she developed a cough and fever, and showed leukopenia and thrombocytopenia. Computed tomography showed multiple small nodular lesions in both lungs, and bilateral hilar, mediastinal, and hepatic lymphadenopathy. Fluorodeoxyglucose positron emission tomography demonstrated high uptake in the liver, spleen, pancreatic head, and lymph nodes. Bone marrow biopsy was intact, but liver biopsy revealed anomalous large lymphoid cells in the sinusoids which were positive for CD20 and showed a high Ki-67 index, leading to the diagnosis of diffuse large B-cell lymphoma. Chemotherapy with 8 courses of THP-COP (cyclophosphamide, pirarubicin, vincristine, and prednisolone) with rituximab, followed by intrathecal injection of methotrexate, cytarabine, and dexamethasone, resulted in complete remission. She maintained complete remission for 10 years until 88 years old at present. The literature review found 30 patients, including this case, who developed lymphoma in the course of sarcoidosis. A novel pathological diagnosis is required in the setting of acute symptomatic changes and novel lesions on imaging in patients with sarcoidosis.</p>","PeriodicalId":45936,"journal":{"name":"Journal of Clinical and Experimental Hematopathology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2022-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/1e/48/jslrt-62-226.PMC9898715.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10695884","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":"Immunohistochemistry for IRTA1 and MNDA helps differentiate gastric MALT lymphoma from chronic gastritis/reactive lymphocyte hyperplasia.","authors":"Yoshiyuki Ayada, Takuro Igawa, Yusuke Naoi, Kyosuke Horikawa, Tetsuya Tabata, Takehiro Tanaka, Tadashi Yoshino","doi":"10.3960/jslrt.22021","DOIUrl":"https://doi.org/10.3960/jslrt.22021","url":null,"abstract":"<p><p>It is difficult to histologically differentiate extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) from chronic gastritis (CG)/ reactive lymphoid hyperplasia (RLH). To determine whether immunohistochemistry for IRTA1 and MNDA can differentiate gastric MALT lymphoma from CG/RLH, we investigated 81 stomach biopsy specimens [Wotherspoon grade (WG) 1, 11 cases; WG 2, 9 cases; WG 3, 20 cases; WG 4, 31 cases; and WG 5, 10 cases]. According to a previously reported algorithm involving PCR for immunoglobulin heavy (IgH) chain locus rearrangement, all 81 cases were divided into three groups: CG/RLH (55 cases), MALT lymphoma (19 cases) groups, and IgH undetectable group (7 cases). We analyzed the CG/RLH and MALT lymphoma groups. The median percentage of IRTA1-positive cells was 0% (range 0%-90.6%) in the CG/RLH group and 43.5% (range 0%-97.6%) in the MALT lymphoma group (p < 0.0001). The median percentage of MNDA-positive cells was 32.4% (range 0%-97.6%) in the CG/RLH group and 55.1% (range 0%-97.6%) in the MALT lymphoma group (p = 0.0044). These results indicate that immunohistochemistry for IRTA1 and MNDA can help differentiate gastric MALT lymphoma from CG/RLH.</p>","PeriodicalId":45936,"journal":{"name":"Journal of Clinical and Experimental Hematopathology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2022-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/c9/29/jslrt-62-195.PMC9898717.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10690107","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":"Two cases of follicular lymphoma with MYC gene abnormalities that presented with bone marrow necrosis.","authors":"Yuri Miyazawa, Hisashi Takei, Nobuhiko Kobayashi, Naoki Akashi, Yukiko Sairenji, Manato Sugisaki, Chiaki Naito, Tetsuya Ishikawa, Hiroaki Shimizu, Takuma Ishizaki, Akihiko Yokohama, Norifumi Tsukamoto, Yuka Yoshida, Nozomi Matsumura, Yoshiyasu Takayama, Hiroshi Handa","doi":"10.3960/jslrt.22004","DOIUrl":"https://doi.org/10.3960/jslrt.22004","url":null,"abstract":"<p><p>Bone marrow necrosis (BMN) occurs most frequently in hematological malignancies and sometimes in non-hematological disorders. Lymphoid diseases causing necrosis are regarded as high-grade disease. B-lymphoblastic leukemia/lymphoma is the most common malignant cause of BMN. Here, we present two patients with follicular lymphoma (FL) and MYC gene abnormalities who developed BMN. In one case of BMN, the necrosis disappeared in response to chemotherapy, and the patient survived with complete remission. In the other case, BMN remained even after chemotherapy, and effective chemotherapy could not be administered due to suppressed hematopoiesis, which led to the lymphoma worsening and the patient's death. Indolent lymphomas, such as FL, as in these cases, have the potential to develop BMN. It is important to detect the development of BMN and administer chemotherapy early to improve patient prognosis, since severe BMN prevents patients from receiving effective treatment.</p>","PeriodicalId":45936,"journal":{"name":"Journal of Clinical and Experimental Hematopathology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2022-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/9d/39/jslrt-62-208.PMC9898713.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10700330","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":"Cholesterol metabolism and lipid droplet vacuoles; a potential target for the therapy of aggressive lymphoma.","authors":"Hiromu Yano, Yukio Fujiwara, Yoshihiro Komohara","doi":"10.3960/jslrt.22023","DOIUrl":"https://doi.org/10.3960/jslrt.22023","url":null,"abstract":"<p><p>Cholesterol uptake via LDL receptor (LDLR) is increased in some malignant tumors, and incorporated LDL contribute to lipid droplet formation. Burkitt's lymphoma is known to have a large number of vacuoles in the cytoplasm, however, intracellular vacuoles are also seen in high-grade lymphomas such as adult T-cell leukemia/lymphoma, diffuse large B-cell lymphoma and primary central nervous system lymphoma. Recent studies have shown that esterified cholesterol is the main component of these vacuoles and the expression of cholesterol metabolism-related molecules such as LDLR, acetyl-CoA acetyltransferase 1 (ACAT1) which esterifies free cholesterol, and scavenger receptor class B type I (SR-BI) which effluxes free cholesterol, was significantly upregulated in lymphoma cells. Moreover, negative feedback of LDLR was not regulated even under cholesterol-rich conditions in lymphoma cells. We found that cytoplasmic free cholesterol was increased by ACAT and SR-BI inhibitors (CI-976 and BLT-1, respectively), and the accumulation of free cholesterol induced lymphoma cell apoptosis. In addition, overexpression of lipid droplet surface proteins has been correlated with poor prognosis in several malignant tumor such as ovarian cancer and clear cell renal cell carcinoma, and it is important to evaluate lipid droplet formation in malignant tumors including lymphomas.</p>","PeriodicalId":45936,"journal":{"name":"Journal of Clinical and Experimental Hematopathology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2022-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/3f/6d/jslrt-62-190.PMC9898721.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10700877","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":"An experience with ibrutinib monotherapy for Richter's syndrome isolated in the central nervous system.","authors":"Yuma Nato, Keiki Nagaharu, Kanako Inoue, Kodai Yabu, Akihiko Sawaki, Takuya Shiotani, Yuki Kageyama, Ken Tanaka, Koichi Ohshima, Hiroyuki Miyashita","doi":"10.3960/jslrt.22017","DOIUrl":"https://doi.org/10.3960/jslrt.22017","url":null,"abstract":"<p><p>Richter's syndrome (RS) of the central nervous system (CNS) is known to have an extremely poor prognosis. Ibrutinib has been reported to have some activity in patients with RS, despite its poor prognosis. Although ibrutinib crosses the blood-brain barrier, its efficacy in RS patients with CNS involvement remains unknown. Here, we report a case of RS isolated in the CNS that was confirmed to be clonally related to chronic lymphocytic leukemia (CLL) by immunoglobulin heavy chain gene analysis. Although the median survival of patients with RS clonally related to CLL was significantly shorter than that of patients with RS clonally unrelated to CLL, the patient received ibrutinib monotherapy without experiencing any significant adverse events, and the disease remained stable with ibrutinib until 6 weeks later. Following whole-brain radiation therapy (40 Gy in 20 fractions) with dexamethasone, the patient has survived for five months after diagnosis. Thus, ibrutinib may be a safe and effective therapeutic option for patients with RS and CNS involvement.</p>","PeriodicalId":45936,"journal":{"name":"Journal of Clinical and Experimental Hematopathology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2022-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ea/2e/jslrt-62-238.PMC9898719.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10690104","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":45936,"journal":{"name":"Journal of Clinical and Experimental Hematopathology","volume":null,"pages":null},"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":"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":45936,"journal":{"name":"Journal of Clinical and Experimental Hematopathology","volume":null,"pages":null},"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":"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":45936,"journal":{"name":"Journal of Clinical and Experimental Hematopathology","volume":null,"pages":null},"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":"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":45936,"journal":{"name":"Journal of Clinical and Experimental Hematopathology","volume":null,"pages":null},"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}