川崎综合征患儿短暂性红细胞减少1例。

G R Frank, I Cherrick, G Karayalcin, E Valderrama, P Lanzkowsky
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引用次数: 7

摘要

目的:我们首次描述了一例儿童与川崎综合征和相关的短暂性红细胞减少症。患者和方法:一例5个半月大的川崎综合征婴儿,表现为淋巴结病、发热、结膜注射皮疹和四肢改变,伴有贫血和网状红细胞减少症,需要输血和血小板减少症。骨髓穿刺符合短暂性红细胞减少。尽管静脉注射免疫球蛋白和阿司匹林治疗,她还是患上了心脏动脉瘤。结果:血红蛋白恢复正常,贫血和网状红细胞减少症消失。血小板计数增加,她出现了这种临床实体的血小板增多特征。她完全康复,没有贫血或网状红细胞减少症复发。结论:我们推测,导致红细胞减少和血小板减少的原因是川崎综合征对骨髓及其各种谱系的炎症性损伤,导致无数的血液学异常,现在与川崎综合征相关。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。
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Transient erythroblastopenia in a child with Kawasaki syndrome: a case report.

Purpose: We describe for the first time the case of a child with Kawasaki syndrome and associated transient erythroblastopenia.

Patients and methods: 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.

Results: 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.

Conclusions: 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.

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