Frank G R, Cherrick I, Karayalcin G, Valderrama E, Lanzkowsky P
Schneider Children's Hospital of the Long Island Jewish Medical Center, New Hyde Park, NY 11042.
Am J Pediatr Hematol Oncol. 1994 Aug;16(3):271-4. doi: 10.1097/00043426-199408000-00016.
We describe for the first time the case of a child with Kawasaki syndrome and associated transient erythroblastopenia.
A 5 1/2-month-old infant with Kawasaki syndrome as evidenced by lymphadenopathy, fever, rash conjunctival injection, and extremity changes had associated anemia and reticulocytopenia requiring transfusion and thrombocytopenia. Bone marrow aspiration was consistent with a transient erythroblastopenia. She developed cardiac aneurysms despite therapy with i.v. immunoglobulin and aspirin.
The anemia and reticulocytopenia resolved with normalization of her hemoglobin. The platelet count increased and she developed a thrombocytosis characteristic of this clinical entity. She completely recovered without recurrence of either the anemia or reticulocytopenia.
We speculate that the cause of the erythroblastopenia and thrombocytopenia is an inflammatory insult of Kawasaki syndrome on the bone marrow and its various lineages causing the myriad hematological abnormalities now associated with Kawasaki syndrome. It is possible that the i.v. immunoglobulin aids in neutralizing the triggering agent and therefore shortening the duration of the marrow insult.
我们首次描述了一例患有川崎病并伴有短暂性红细胞生成减少症的儿童病例。
一名5个半月大的婴儿,出现淋巴结病、发热、皮疹、结膜充血及四肢变化,确诊为川崎病,伴有贫血、网织红细胞减少症(需要输血)及血小板减少症。骨髓穿刺结果符合短暂性红细胞生成减少症。尽管接受了静脉注射免疫球蛋白和阿司匹林治疗,但她仍出现了心脏动脉瘤。
随着血红蛋白恢复正常,贫血和网织红细胞减少症得到缓解。血小板计数增加,她出现了这种临床病症特有的血小板增多症。她完全康复,贫血和网织红细胞减少症均未复发。
我们推测,红细胞生成减少症和血小板减少症的病因是川崎病对骨髓及其各种谱系的炎性损伤,导致了目前与川崎病相关的众多血液学异常。静脉注射免疫球蛋白可能有助于中和触发因子,从而缩短骨髓损伤的持续时间。