Al Sineidi Khalfan, Wali Yasser A, Pathare Anil V, Al Lamki Zakia
Department of Child Health (Haematology/Oncology Unit), College of Medicine & Health Sciences, Sultan Qaboos University, P.O.Box: 35, Al Khod-123, Sultanate of Oman.
J Sci Res Med Sci. 2002 Apr;4(1-2):45-8.
The paper reports the case of a previously healthy 4-year-old-girl who presented with pallor, fever and hepatosplenomegaly. Laboratory findings included pancytopenia, hypertriglyceridemia and hyperferritinemia. Initial diagnosis of kala-azar could not be confirmed because of the absence of clinical evidence, negativity of bone marrow aspiration or specific serology for visceral leishmaniasis. Repeated marrow aspiration, performed due lack of clinical response, revealed histiocytes showing haemophagocytosis consistent with haemophagocytic lymphohistocytosis (HLH) and appropriate treatment was started. She continued to have high-grade fever, and a third bone marrow aspiration ultimately revealed presence of Leishmania amastigotes with evidence of active haemophagocytosis. The girl was treated with liposomal amphotericin (AmBisome) for 5 days, following which she recovered rapidly with definitive remission.
该论文报道了一名此前健康的4岁女童的病例，她出现了面色苍白、发热和肝脾肿大的症状。实验室检查结果包括全血细胞减少、高甘油三酯血症和高铁蛋白血症。由于缺乏临床证据、骨髓穿刺结果为阴性以及内脏利什曼病的特异性血清学检查结果为阴性，最初的黑热病诊断无法得到证实。由于缺乏临床反应而进行的重复骨髓穿刺显示，组织细胞呈现噬血细胞现象，符合噬血细胞性淋巴组织细胞增生症（HLH），于是开始了适当的治疗。她持续高热，第三次骨髓穿刺最终发现了利什曼原虫无鞭毛体，并伴有活跃的噬血细胞现象。该女童接受了5天的脂质体两性霉素（安必素）治疗，随后迅速康复并完全缓解。
