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一例脾脏原发性非白血病性髓系肉瘤,经手术及造血干细胞移植成功治疗。

A case of primary nonleukemic myeloid sarcoma of the spleen, successfully treated by surgery and hematopoietic stem cell transplantation.

作者信息

Ono Asuka, Kitano Yuki, Imai Katsunori, Matsumoto Takashi, Endo Shinya, Tokunaga Kenji, Hayashi Hiromitsu, Yamashita Yo-Ichi, Matsuoka Masao, Baba Hideo

机构信息

Department of Gastroenterological Surgery, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Chuo-ku, Kumamoto, 860-8556, Japan.

Departments of Hematology, Rheumatology, and Infectious Disease, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.

出版信息

Surg Case Rep. 2021 Aug 11;7(1):180. doi: 10.1186/s40792-021-01257-w.

Abstract

BACKGROUND

Myeloid sarcoma (MS) is a rare disease, mostly found in conjunction with acute myelogenous leukemia or other diseases, and primary nonleukemic MS of the spleen is particularly rare.

CASE PRESENTATION

We report a 57-year-old male who presented with a spleen mass that was found incidentally, and was enlarged. As a result of various examinations, he was diagnosed with primary MS of the spleen with suspected involvement of the transverse colon, left kidney, pancreatic tail, and left diaphragm. He underwent a total splenectomy, partial pancreatectomy, partial colectomy, left nephrectomy, and left diaphragm partial resection. Histological examination revealed splenic primary MS. Bone marrow biopsy and immunophenotypic flow cytometry revealed no evidence of myeloid leukemia. He underwent umbilical cord blood transplantation, and he is currently living without a sign of recurrence at 10 months after surgery.

CONCLUSIONS

We experienced a very rare case of primary spleen MS that was discovered without a hematologic malignancy. Two cases of surgically resected primary splenic MS have been reported, including the present case.

摘要

背景

髓系肉瘤(MS)是一种罕见疾病,大多与急性髓系白血病或其他疾病同时出现,而脾脏原发性非白血病性MS尤为罕见。

病例介绍

我们报告一名57岁男性,偶然发现脾脏肿块且脾脏肿大。经过各项检查,他被诊断为脾脏原发性MS,怀疑横结肠、左肾、胰尾和左膈受累。他接受了全脾切除术、部分胰腺切除术、部分结肠切除术、左肾切除术和左膈部分切除术。组织学检查显示为脾脏原发性MS。骨髓活检和免疫表型流式细胞术未发现髓系白血病证据。他接受了脐带血移植,术后10个月目前生活状态良好,无复发迹象。

结论

我们遇到了一例非常罕见的无血液系统恶性肿瘤的原发性脾脏MS病例。包括本病例在内,已报告了两例手术切除的原发性脾脏MS病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d71a/8357860/960ca0b052f7/40792_2021_1257_Fig1_HTML.jpg

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