Haemophagocytic Lymphohistiocytosis–A Report of Two Cases and Review of Literature

Dr Sangeeta Sharma; Dr Ujwala Maheshwari; Dr Kuldeep Singh

Volume : IV, Issue : II, February - 2015

Haemophagocytic Lymphohistiocytosis–A Report of Two Cases and Review of Literature

Dr Sangeeta Sharma, Dr Ujwala Maheshwari, Dr Kuldeep Singh

Abstract :

Haemophagocytic lymphohistiocytosis (HLH) is a rare and unusual syndrome, more common in the paediatric age group, characterized by fever, splenomegaly, bicytopenia or pancytopenia, and proliferation of macrophages and histiocytes in the bone marrow, liver, spleen. These histiocytes are morphologically benign but hyperactive and demonstrate phagocytosis of other blood cells including erythrocytes, leukocytes, platelets and their precursor haemopoietic cells. Primary or Familial HLH is an autosomal recessive inherited disorder, while Secondary or Acquired HLH is caused by abnormal immunological activation such as in immunodeficiency or after systemic infections. We present two cases of Secondary HLH, which presented with pyrexia and cytopenias, raised triglycerides, raised LDH levels, and bone marrow findings typically suggestive of Haemophagocytic lymphohistiocytosis. The two cases had different trigger factors - one patient had infection induced HLH, while the other patient had autoimmune disease induced HLH.