Komata Daiki, Takahata Mutsumi, Makino Yoshinori, Ishio Takashi, Iwasaki Hiroshi, Ichihara Shin, Tsuda Masumi, Tanaka Shinya, Ibata Makoto
Department of Hematology, Sapporo-Kosei General Hospital.
Department of Pathology, Sapporo-Kosei General Hospital.
Rinsho Ketsueki. 2024;65(8):737-741. doi: 10.11406/rinketsu.65.737.
Histiocytic sarcoma (HS) is a rare aggressive hematological malignancy reported to occur secondary to B cell lymphoma. We report a case of HS secondary to splenic marginal zone lymphoma (SMZL) complicated by autoimmune hemolytic anemia (AIHA) in a 64-year-old man. He was referred to our department with anemia and was diagnosed as having AIHA. After starting treatment with prednisolone, atypical lymphocytes appeared in his blood tests, and a bone marrow biopsy revealed invasion by B cell lymphoma. A CT scan showed splenomegaly and a pancreatic mass, which confirmed the diagnosis of SMZL. The patient received bendamustine and rituximab as chemotherapy, which rapidly improved the anemia and splenomegaly and reduced atypical lymphocytes. However, left lumbar back pain appeared along with an increase in the pancreatic mass, and he died suddenly of acute renal failure. An autopsy revealed that the tumor had invaded several organs including the pancreas, and immunohistochemistry was positive for CD163, leading to the diagnosis of HS. Furthermore, the specimens of SMZL and HS were positive for IgH gene reconstitution, and exome analysis showed genetic abnormalities in 226 genes including CARD11, suggesting that the SMZL and HS had the same origin.
组织细胞肉瘤（HS）是一种罕见的侵袭性血液系统恶性肿瘤，据报道继发于B细胞淋巴瘤。我们报告一例64岁男性患者，其脾脏边缘区淋巴瘤（SMZL）继发组织细胞肉瘤并合并自身免疫性溶血性贫血（AIHA）。他因贫血转诊至我科，被诊断为AIHA。在开始使用泼尼松龙治疗后，他的血液检查中出现了非典型淋巴细胞，骨髓活检显示有B细胞淋巴瘤浸润。CT扫描显示脾肿大和胰腺肿块，确诊为SMZL。患者接受苯达莫司汀和利妥昔单抗化疗，贫血和脾肿大迅速改善，非典型淋巴细胞减少。然而，随着胰腺肿块增大，患者出现左腰背痛，并因急性肾衰竭突然死亡。尸检显示肿瘤已侵犯包括胰腺在内的多个器官，免疫组化CD163呈阳性，确诊为HS。此外，SMZL和HS标本的IgH基因重排呈阳性，外显子分析显示包括CARD11在内的226个基因存在基因异常，提示SMZL和HS起源相同。
