Shah S J, Warrier R P, Ode D L, Lele H E, Yu L C
Louisiana State University Medical Center and Children's Hospital, New Orleans, USA.
J Pediatr Hematol Oncol. 1996 May;18(2):227-9. doi: 10.1097/00043426-199605000-00029.
We discuss an unusual clinical presentation of Hodgkin disease with immune thrombocytopenia and autoimmune hemolytic anemia.
A 4-year-old boy presented to us with a large anterior mediastinal mass, thrombocytopenia, and Coombs' positive hemolytic anemia refractory to transfusion therapy. Biopsy of the anterior mediastinal mass was possible only after administration of intravenous immunoglobulin to raise the platelet count. The immune manifestations decreased with initiation of appropriate chemotherapy.
The child was able to successfully complete chemotherapy and radiation therapy and has no clinical or laboratory evidence of persistent autoimmune phenomena.
Immune thrombocytopenia with autoimmune hemolytic anaemia is a rare presenting manifestation of Hodgkin disease and can present difficulty in diagnosis and management.
我们讨论霍奇金病伴免疫性血小板减少症和自身免疫性溶血性贫血的一种不寻常临床表现。
一名4岁男孩因前纵隔巨大肿块、血小板减少症以及对输血治疗无效的库姆斯试验阳性溶血性贫血前来就诊。仅在静脉注射免疫球蛋白以提高血小板计数后才得以对前纵隔肿块进行活检。随着适当化疗的开始,免疫表现有所减轻。
该患儿能够成功完成化疗和放疗,且没有持续自身免疫现象的临床或实验室证据。
免疫性血小板减少症伴自身免疫性溶血性贫血是霍奇金病一种罕见的表现形式,在诊断和管理方面可能存在困难。