[Lymphoplasmacytic lymphoma accompanied by severe myelofibrosis].

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI:10.11406/rinketsu.64.54

Ayako Takarada, Haruka Momose, Naoki Kurita, Ryota Matsuoka, Naoya Nakamura, Tasuhiro Sakamoto, Takayasu Kato, Keiichiro Hattori, Yasuhito Suehara, Yasuhisa Yokoyama, Hidekazu Nishikii, Yumiko Maruyama, Naoshi Obara, Shigeru Chiba, Mamiko Sakata-Yanagimoto

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Abstract

A 61-year-old female was referred to our hospital because of pancytopenia and febrile neutropenia. On admission, computed tomography showed mild hepatosplenomegaly and intra-abdominal abscess formation in the right pelvic region; however, no lymphadenopathy was found. Bone marrow (BM) examination showed severe fibrosis by silver staining. Several small- to medium-sized lymphocytes with a constriction in the nuclei were observed, exhibiting CD3 (-), CD10 (-), CD20 (+), BCL-2 (+-), and CD138 (+-). Genetic testing revealed that BM cells were positive for MYD88 mutation and positive for IgH rearrangement, whereas neither JAK2 nor CALR mutation was positive. A diagnosis of BM infiltration of lymphoplasmacytic lymphoma (LPL) was made. Rituximab monotherapy was administered once a week for four times. BM examination 4 weeks after the end of treatment showed that lymphoma cells had disappeared and that myelofibrosis had been almost gone. The MYD88 mutation of BM turned out to be negative at that moment.

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[淋巴浆细胞性淋巴瘤伴严重骨髓纤维化]。

一位61岁女性因全血细胞减少症和发热性中性粒细胞减少症转诊至我院。入院时，计算机断层扫描显示轻度肝脾肿大，右侧骨盆区腹腔内脓肿形成;但未发现淋巴结病变。骨髓(BM)银染色显示严重纤维化。核内可见少量中小淋巴细胞缩窄，表现为CD3(-)、CD10(-)、CD20(+)、BCL-2(+-)和CD138(+-)。基因检测显示BM细胞MYD88突变阳性，IgH重排阳性，而JAK2和CALR突变均未阳性。诊断为骨髓浸润性淋巴浆细胞性淋巴瘤(LPL)。利妥昔单抗单药治疗每周1次，共4次。治疗结束4周后BM检查显示淋巴瘤细胞消失，骨髓纤维化基本消失。此时BM的MYD88突变为阴性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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[Rinsho ketsueki] The Japanese journal of clinical hematology

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