Hemophagocytic Lymphohistiocytosis during the Postpartum Stage of Pregnancy: A Report of Eight Cases
Hemophagocytic Lymphohistiocytosis during the Postpartum Stage of Pregnancy: A Report of Eight Cases
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DOI：
10.1159/000493879
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发表时间：
2019-01-01
影响因子：
2.4
通讯作者：
Wang, Zhao
中科院分区：
文献类型：
--
作者：
Song, Yue;Wang, Jing-Shi;Wang, Zhao
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见且严重的临床综合征，可分为原发性和继发性。继发性HLH可由多种疾病引发。临床上已观察到与妊娠相关的HLH。然而，这些病例大多发生在妊娠期。考虑到分娩前后时期具有不同的临床特征，我们呈现了首个在妊娠产后阶段出现的HLH病例系列。从这些病例中，我们得出结论，产后HLH是与妊娠相关的HLH的一种常见形式。产后HLH患者可能有更复杂的发病机制。血细胞减少不如其他类型的HLH常见，但几乎所有病例都存在肝功能障碍。HLH - 94/04标准疗法非常有效，产后HLH的预后更好。（C）2018 S. Karger AG，巴塞尔
Haemophagocytic lymphohistiocytosis (HLH) is a rare and severe clinical syndrome that can be classified as either primary or secondary. Secondary HLH can be triggered by a variety of diseases. Pregnancy-related HLH has already been observed clinically. However, most of these cases occur during pregnancy. Considering that the periods before and after delivery have different clinical features, we presented the first case series of HLH that presented during the postpartum stage of pregnancy. From these cases, we concluded that postpartum HLH is a common form of pregnancy-related HLH. Patients with postpartum HLH may suffer more complicated pathogenesis. Cytopenia was not as common as in other types of HLH, but liver dysfunction was present in almost all cases. The standard therapy of HLH-94/04 was very effective, and the outcomes of postpartum HLH were better. (C) 2018 S. Karger AG, Basel