Suehiro Satoru, Shiratsuchi Motoaki, Suehiro Youko, Oshima Koichi, Shiokawa Satoshi, Nishimura Junji
Department of Rheumatology and Medicine, Medical Institute of Bioregulation, Kyushu University.
Rinsho Ketsueki. 2002 Sep;43(9):841-5.
We present a case of angioimmunoblastic T cell lymphoma (AITL) with autoimmune thrombocytopenia. A 85-year-old man was admitted to our hospital with thrombocytopenia, generalized lymphadenopathy, pleural effusion, and splenomegaly in June 2000. Blood chemistry revealed hemoglobin and platelet counts of 8.8 g/dL and 26 x 10(9)/L, respectively. The level of platelet-associate-IgG was 2568.9 ng/10(7) cells. The direct Coombs test was positive. The level of serum IL-6 was 10.2 pg/ml. Megakaryocytes in the bone marrow increased. Lymph node biopsy showed diffuse proliferation of atypical lymphoid cells with a clear cytoplasm accompanied by plasma cells and small vessels. He was diagnosed as having AITL with autoimmune thrombocytopenia and hemolytic anemia. He received repeated platelet transfusion, and a limited effect of prednisolone therapy on his platelet count was observed. Combination chemotherapy lessened the extent of the lymphadenopathy and slightly elongated the interval of platelet transfusion. We next performed splenic irradiation and a slight increase in the platelet count was observed. He died of pneumonia in August 2000. Autoimmune thrombocytopenia associated with AITL is rare and the therapy containing prednisolone and chemotherapy is reported to be partly effective. Our case showed a minor response of autoimmune thrombocytopenia to splenic irradiation. Therapeutic intervention for hypersplenism should be considered if thrombocytopenia is not improved by chemotherapy alone.
我们报告一例伴有自身免疫性血小板减少症的血管免疫母细胞性T细胞淋巴瘤（AITL）。一名85岁男性于2000年6月因血小板减少、全身淋巴结肿大、胸腔积液和脾肿大入住我院。血液生化检查显示血红蛋白和血小板计数分别为8.8 g/dL和26×10⁹/L。血小板相关IgG水平为2568.9 ng/10⁷细胞。直接抗人球蛋白试验呈阳性。血清IL-6水平为10.2 pg/ml。骨髓中的巨核细胞增多。淋巴结活检显示非典型淋巴细胞弥漫性增生，胞质清晰，伴有浆细胞和小血管。他被诊断为患有AITL并伴有自身免疫性血小板减少症和溶血性贫血。他接受了多次血小板输注，观察到泼尼松龙治疗对其血小板计数的效果有限。联合化疗减轻了淋巴结肿大的程度，并略微延长了血小板输注的间隔时间。接下来我们进行了脾照射，观察到血小板计数略有增加。他于2000年8月死于肺炎。与AITL相关的自身免疫性血小板减少症很罕见，据报道含泼尼松龙和化疗的治疗有部分效果。我们的病例显示自身免疫性血小板减少症对脾照射有轻微反应。如果单纯化疗不能改善血小板减少症，应考虑对脾功能亢进进行治疗干预。
