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一名4个月大女童致命性川崎病急性期的噬血细胞现象

Hemophagocytosis in the Acute Phase of Fatal Kawasaki Disease in a 4 Month-Old Girl.

作者信息

Doğan Vehbi, Karaaslan Erhan, Özer Samet, Gümüşer Rüveyda, Yılmaz Resul

机构信息

Department of Pediatric Cardiology, Dr. Sami Ulus Maternity and Children Training and Research Hospital, Ankara, Turkey.

Department of Pediatrics, Gaziosmanpaşa University School of Medicine, Tokat, Turkey.

出版信息

Balkan Med J. 2016 Jul;33(4):470-2. doi: 10.5152/balkanmedj.2016.150371. Epub 2016 Jul 1.

DOI:10.5152/balkanmedj.2016.150371
PMID:27606147
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5001829/
Abstract

BACKGROUND

Kawasaki disease is a systemic vasculitis predominately affecting coronary arteries. Hemophagocytic lymphohistiocytosis can complicate the course of Kawasaki disease. Rare cases of secondary hemophagocytic lymphohistiocytosis occurring during the acute phase of Kawasaki disease have been reported.

CASE REPORT

We report here a 4 month-old girl with diffuse coronary ectasia and secondary hemophagocytic lymphohistiocytosis occurring during the acute phase of incomplete Kawasaki disease.

CONCLUSION

Due to the large overlap in clinical symptoms, the presence of atypical findings for Kawasaki disease should suggest the possible diagnosis of hemophagocytic lymphohistiocytosis in these patients.

摘要

背景

川崎病是一种主要影响冠状动脉的全身性血管炎。噬血细胞性淋巴组织细胞增生症可使川崎病病程复杂化。已有报道称在川崎病急性期出现罕见的继发性噬血细胞性淋巴组织细胞增生症病例。

病例报告

我们在此报告一名4个月大的女童，患有弥漫性冠状动脉扩张，并在不完全性川崎病急性期出现继发性噬血细胞性淋巴组织细胞增生症。

结论

由于临床症状有很大重叠，川崎病非典型表现的存在应提示这些患者可能诊断为噬血细胞性淋巴组织细胞增生症。

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本文引用的文献

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Macrophage activation syndrome in the acute phase of Kawasaki disease.川崎病急性期的巨噬细胞活化综合征
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Hemophagocytic lymphohistiocytosis at initiation of kawasaki disease and their differential diagnosis.川崎病发病初期的噬血细胞性淋巴组织细胞增生症及其鉴别诊断。
Pediatr Hematol Oncol. 2010 Apr;27(3):244-9. doi: 10.3109/08880011003623642.
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Hemophagocytic lymphohistiocytosis (HLH) and related disorders.噬血细胞性淋巴组织细胞增生症（HLH）及相关疾病。
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Hemophagocytic lymphohistiocytosis and Kawasaki disease: combined manifestation and differential diagnosis.噬血细胞性淋巴组织细胞增生症与川崎病：联合表现及鉴别诊断
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Clinical spectrum of Kawasaki disease in infants.婴儿川崎病的临床谱
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Hemophagocytosis complicating Kawasaki disease.噬血细胞增多症并发川崎病。
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Atypical and complicated Kawasaki disease in infants. Do we need criteria?婴儿非典型和复杂型川崎病。我们需要标准吗？
West J Med. 1995 Apr;162(4):322-7.