DOI: 10.36347/sjams.2019.v07i10.042

Pages: 3434-3440

Introduction: Haemophagocytic lymphohistiocytosis (HLH) is an aggressive and life threatening syndrome of highly stimulated but ineffective immune process. It can be triggered by a variety of events that disrupt immune homeostasis. Infection is a common trigger both in those with a genetic predisposition and in sporadic cases. Acquired HLH, with or without genetic disorders, may be due to infectious like bacterial, fungal, parasitic and viral or non-infectious etiologies such as malignancies, autoimmune disorders, and drugs. Although an early diagnosis is crucial to decrease mortality, it is often challenging due to lack of specificity of the clinical and laboratory findings and less availability of genetic tests in developing country. Materials and Method: In this prospective study, total 370 patients referred to our department for bone marrow aspiration from other departments of Calcutta National Medical College and Hospital from July’17 to July’19 and out of these 370 patients, 150 patients who are suspected for hemophagocytosis, evaluated for etiology. Result: Among 150 patients clinically suspicious of haemophagocytic lymphohistiocytosis 30 patients had haemophagocytic lymphohistiocytosis. 18 patients (60%) are male and 12 patients (40%) are female. The patients’ age ranged from 1 year to 85 years. Among them 8 cases (26.68%) associated with infective etiology, 3 cases (10.0%) with megaloblastic anemia, 4 cases (13.33%) with acute leukemia, 5 cases (16.67%) with myelodysplastic syndrome, 3 cases (10.0%) associated with plasma cell dyscrasia, 4 cases (13.33%) have normoblastic erythroid hyperplasia and 3cases (10.0%) are of unknown etiology. Conclusion: Haemophagocytic lymphohistiocytosis has a wide spectrum of causes which can be diagnosed by detailed history, peripheral smear examination supported by bone marrow examination, biochemical tests, specific antibody detection and other relevant investigations.