Fathalla Mahmoud, Hashim Javad, Alkindy Hussein, Wali Yasser
Department of Child Health, College of Medicine and Health Sciences, Sultan Qaboos University, P. O. Box 35, Al-Khod 123, Muscat, Sultanate of Oman.
Sultan Qaboos Univ Med J. 2007 Dec;7(3):253-6.
Hemophagocytic Lymphohistiocytosis (HLH) implies a benign generalized histiocytic proliferate with erythrophagocytosis and it includes familial hemophagocytic lymphohistiocytosis and secondary hemophgocytosis. Spinal fluid changes like mild to moderate pleocytosis (most of the cells are lymphocytes and macrophages) and sometimes hemophagocytosis are seen in primary HLH but are not reported in secondary HLH. Here we report a case of a previously healthy 10 months old male infant who was diagnosed as familial HLH with evidence of CSF hemophagocytosis. He was started on the HLH 2004 treatment protocol with no improvement. A second bone marrow aspiration revealed leshmania donovani antibodies and he was started on anti-leishmania treatment with dramatic response.To the best of our knowledge, this is the first case of secondary HLH with evidence of hemophagocytosis in cerebrospinal fluid.
噬血细胞性淋巴组织细胞增生症（HLH）是一种伴有红细胞吞噬现象的良性全身性组织细胞增生性疾病，包括家族性噬血细胞性淋巴组织细胞增生症和继发性噬血细胞增多症。原发性HLH可见脑脊液改变，如轻度至中度细胞增多（大多数细胞为淋巴细胞和巨噬细胞），有时可见噬血细胞现象，但继发性HLH未见相关报道。我们在此报告一例先前健康的10个月大男婴，被诊断为家族性HLH，有脑脊液噬血细胞现象的证据。他开始接受2004年HLH治疗方案，但无改善。第二次骨髓穿刺显示杜氏利什曼原虫抗体，随后他开始接受抗利什曼原虫治疗，反应显著。据我们所知，这是首例有脑脊液噬血细胞现象证据的继发性HLH病例。
