遗传性球形红细胞增多症患者与细小病毒b19相关的嗜血淋巴组织细胞炎1例报告

Cheong Cs, Gancevici Gg, Bee Pc, Chen Tm, Lim Cc
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引用次数: 3

摘要

噬血细胞淋巴组织细胞病(HLH)是一种临床病理实体,由网状内皮系统中良性巨噬细胞增殖和活化增加引起的噬血细胞症。与病毒相关的HLH是一个公认的实体。虽然大多数细小病毒B19相关的HLH不需要任何特异性治疗,预后良好,但儿童的预后比成人差。我们在此报告一例HLH合并急性细小病毒B19感染的年轻健康患者,其潜在的遗传性球形细胞增多症,骨髓表现为典型的细小病毒感染。虽然该患者细胞计数自发恢复,但由于长时间通气并发症而死亡。出乎意料的是,尽管他患有呼吸机相关性肺炎,但他的免疫球蛋白水平却异常正常,这反映了对感染的体液免疫反应不足。
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PARVOVIRUS B19 ASSOCIATED HAEMOPHAGOCYTIC LYMPHOHISTIOCYTISIS IN HEREDITARY SPHEROCYTOSIS PATIENT: A CASE REPORT
Haemophagocytic lymphohistiocytosis (HLH) is a clinico-pathologic entity caused by increased proliferation and activation of benign macrophages with haemophagocytosis throughout the reticulo-endothelial system. Virus-associated HLH is a well-recognised entity. Although majority of parvovirus B19 associated HLH does not require any specific treatment and carries good prognosis, outcome of children is worse than adults. We report here a case of HLH associated with acute parvovirus B19 infection in a young healthy patient with underlying hereditary spherocytosis, with bone marrow findings typical of parvovirus infection. Although this patient had spontaneous recovery of cell counts, he succumbed due to complication from prolonged ventilation. Unexpectedly, his immunoglobulin levels were inappropriately normal despite on-going ventilator associated pneumonia, which reflects inadequate humoral immune response towards infection.
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