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原发性骨髓纤维性变,又称髓细胞样脾肿大。本文介绍2例原发性骨髓纤维性变病程中出现皮肤骨髓样组织变形的特异性损害,资料中尚无类似病例报告。例1,男性,62岁。脾脏肿大伴白细胞增高和髓细胞血症。胸骨穿刺确诊。发病3年后出现周围淋巴结肿大和剧烈瘙痒,无皮疹。接着产生弥漫性皮肤浸润和轻度红斑。皱折部位皮损尤著,整个皮肤增厚。临床表现逐渐加剧,但无急性转移的血液系统表现。于发病后第5年因感染和严重贫血而死亡。淋巴结活检示淋巴结有骨髓细胞状组织变形。
Primary myelofibrosis, also known as myeloid splenomegaly. This article describes the two cases of primary myelofibrosis in the process of bone marrow-like tissue degeneration specific damage, the data there is no similar case report. Example 1, male, 62 years old. Splenomegaly with leukocytosis and myelocytosis. Sternal puncture diagnosis. 3 years after onset of peripheral lymphadenopathy and severe itching, no rash. Followed by diffuse skin infiltration and mild erythema. Wrinkled parts of the skin especially, the entire skin thickening. Clinical manifestations gradually increased, but no acute metastatic blood system performance. In the fifth year after the onset of death due to infection and severe anemia. Lymph node biopsy showed bone marrow cell deformity.