Takahashi Tohru
Department of Hematology, Tenshi Hospital, Japan.
Intern Med. 2014;53(7):783-7. doi: 10.2169/internalmedicine.53.1325. Epub 2012 Mar 1.
This report describes a patient with autoimmune myelofibrosis accompanied by Sjögren's syndrome (SS). A 36-year-old woman was admitted due to petechiae, purpura, gingival bleeding, dyspnea on exertion, and a lack of concentration. She had pancytopenia and was diagnosed with SS. A bone marrow study showed hypercellular marrow with reticulin fibrosis. Lymphocytic infiltrates and aggregates composed of a mixture of T and B cells in the marrow were also observed. A chromosomal analysis of the marrow cells showed 47, XXX and an analysis of peripheral lymphocytes revealed 47, XXX/46, XX mosaic results. The patient's cytopenia resolved following treatment with oral prednisolone.
本报告描述了一名患有自身免疫性骨髓纤维化并伴有干燥综合征（SS）的患者。一名36岁女性因瘀点、紫癜、牙龈出血、劳力性呼吸困难和注意力不集中入院。她全血细胞减少，被诊断为干燥综合征。骨髓检查显示骨髓细胞增多伴有网状纤维增生。还观察到骨髓中由T细胞和B细胞混合组成的淋巴细胞浸润和聚集。骨髓细胞的染色体分析显示为47，XXX，外周淋巴细胞分析显示为47，XXX/46，XX嵌合结果。患者经口服泼尼松龙治疗后血细胞减少症得到缓解。
