Bouyssou-Gauthier M L, Bedane C, Jaccard A, Dang P M, Labrousse F, Leboutet M J, Bernard P, Bonnetblanc J M
Service de Dermatologie, CHRU Dupuytren, Limoges.
Ann Dermatol Venereol. 1996;123(8):460-3.
The aim of this work was to present a case of multicentric histiocytosis with haematologic involvement.
A 68-year-old man presented with poor general health and a nodular eruption of the skin and larynx. On clinical examination there was an enlarged spleen and laboratory results revealed an inflammatory syndrome, platelet count 60,000 and myelemia with 10 p. 100 immature elements in a white cell count of 14,000. Pathology and ultrastructure examinations led to the diagnosis of multicentric histiocytosis. Bone marrow aspiration was normal. Pancytopenia then developed with bone marrow hypoplasia without infiltration. Corticosteroids then cyclophosphamide were uneffective for either the skin disease or the hematologic disorder. The patient developed severe buccal aphthosis which responded well to thalidomide. This treatment reduced the size and the number of skin nodules. Finally, renal failure of unknown origin was observed.
Electron microscopy is essential for positive and differential diagnosis of atypical multicentric histiocytosis. Hematological disorders associated with multicentric histiocytosis may either be specific or totally independent.
本研究旨在报告一例伴有血液系统受累的多中心组织细胞增多症病例。
一名68岁男性，全身健康状况较差，皮肤和喉部出现结节性皮疹。临床检查发现脾脏肿大，实验室检查结果显示存在炎症综合征，血小板计数为60,000，白细胞计数为14,000，其中10%为幼稚细胞，提示髓细胞血症。病理及超微结构检查确诊为多中心组织细胞增多症。骨髓穿刺结果正常。随后出现全血细胞减少伴骨髓发育不全且无浸润。皮质类固醇及环磷酰胺对皮肤病及血液系统疾病均无效。患者出现严重口腔阿弗他溃疡，沙利度胺治疗效果良好。该治疗使皮肤结节的大小及数量均有所减少。最后，观察到不明原因的肾衰竭。
电子显微镜检查对于非典型多中心组织细胞增多症的阳性及鉴别诊断至关重要。与多中心组织细胞增多症相关的血液系统疾病可能具有特异性，也可能完全独立存在。
