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 retrospective study, total 857 patients referred to our department for bone marrow aspiration from other departments of Calcutta National Medical College and Hospital from February’16 to October ’18 and out of these 857 patients, 18 patients who have haemophagocytes in their bone marrow findings, were evaluated for etiology.
Result- Among 857 patients, 18 patients (2.1%) had bone marrow haemophagocytic lymphohistiocytosis, of whom 11patients (61.11%) were male and 7 patients (38.89%) were female. The patients’ age ranged from 1 year to 74 years. Among them 4 cases (22.22%) associated with infective etiology, 2 cases (11.11%) with megaloblastic anemia, 2 cases (11.11%) with acute leukemia, 2 cases (11.11%) with myelodysplastic syndrome, 2 cases (11.11%) associated with plasma cell dyscrasia and 6 cases (33.33%) 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. It’s an expected situation for haemophagocytic lymphohistiocytosis reasons that the high rate of infection is one of the major causes in tertiary care centre.
