Singh Z N, Rakheja D, Yadav T P, Shome D K
Department of Pathology, Maulana Azad Medical College, New Delhi, India.
Clin Lab Haematol. 2005 Oct;27(5):312-5. doi: 10.1111/j.1365-2257.2005.00717.x.
Reactive proliferation and inappropriate activation of mature histiocytes with haemophagocytosis (HP) may occur in association with a wide variety of infections, neoplasms, collagen vascular diseases, and acquired and inherited immunodeficiency states. The association with infections is particularly important because overwhelming HP can obscure the typical clinical features of the primary disease and negatively affects outcome. A high index of suspicion is required for early recognition of associated HP as the cause of cytopenias. Institution of specific therapy is crucial for survival. This study highlights the wide spectrum of tropical diseases that can have associated reactive HP. Thirty cases with documented prominent HP on bone marrow aspiration smears were reviewed. Twenty-one (69%) of the marrows were from patients who had common tropical infections: malaria, typhoid and visceral leishmaniasis and 11 of 15 patients (73%) who were followed up improved on specific infection-directed and supportive measures. The presence of severe HP in bone marrow smears correlated with marked cytopenias. Recognition of HP in this geographical region should stimulate the search for one of these infections as early institution of specific therapy is crucial for patient survival.
成熟组织细胞的反应性增殖及伴有噬血细胞作用(HP)的不适当激活可能与多种感染、肿瘤、胶原血管疾病以及获得性和遗传性免疫缺陷状态相关。与感染的关联尤为重要,因为严重的HP可能掩盖原发性疾病的典型临床特征,并对预后产生负面影响。对于早期识别相关HP作为血细胞减少症的病因,需要高度的怀疑指数。实施特异性治疗对生存至关重要。本研究强调了一系列可伴有反应性HP的热带疾病。回顾了30例骨髓穿刺涂片记录有显著HP的病例。其中21例(69%)骨髓来自患有常见热带感染(疟疾、伤寒和内脏利什曼病)的患者,15例接受随访的患者中有11例(73%)在针对特定感染的治疗及支持措施下病情改善。骨髓涂片中有严重HP与明显的血细胞减少相关。在该地理区域识别HP应促使寻找这些感染之一,因为早期实施特异性治疗对患者生存至关重要。