Abstract
Haemophagocytic lymphohistiocytosis (HLH) is a rare disease but potentially life threatening clinical syndrome. It is caused by a multisystemic hyperinflammatory process secondary to severe hypercytokinemia with excessive and uncontrolled activation of the immune response. We report a case of familial HLH with no apparent causes in 6 months-old Malay girl presented with recurrent fever associated with severe anaemia and bleeding tendency requiring extensive treatment but refractory to the treatment which lead to mortality due to neutropenic sepsis indicating of poor prognosis of this disease. This familial type of HLH should be suspected in all children after excluding all the secondary causes with collective laboratory features and requiring extensive management as it associated with high mortality. Bangladesh Journal of Medical Science Vol. 21(1) 2022 Page : 196-200
Highlights
Haemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by fever, cytopenia, splenomegaly and hemophagocytosis activity in bone marrow, spleen, or lymph nodes which can be familial or acquired.1,2Familial HLH is associated with genetic mutation contribution, acquired HLH can be secondary to viral, bacterial, fungal or parasitic infection, autoimmune disorder, malignancies or post transplants recipients.[3]
HLH is characterized by multisystem inflammation. It is a reactive process from prolonged and excessive activation of antigen-presenting cells and CD8+ T cells, which include excessive proliferation and ectopic migration of T cells.[7]. This disease known as histiocytic medullary reticulosis.[8,9]
HLH can be subdivided into 1. primary which can be due to familial or immune deficiency syndromes; and 2. secondary which can be due to infection, autoimmune disease, malignancy or immunosuppression.[10]
Summary
Haemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by fever, cytopenia, splenomegaly and hemophagocytosis activity in bone marrow, spleen, or lymph nodes which can be familial or acquired.1,2Familial HLH is associated with genetic mutation contribution, acquired HLH can be secondary to viral, bacterial, fungal or parasitic infection, autoimmune disorder, malignancies or post transplants recipients.[3]. Case Report: up as outpatient for investigating the cause of hepatosplenomegaly until presented again at the age of 6 months with similar problem of high grade fever, generalized bruises and worsening of abdominal distension. Oral azithromycin for 5 days) and received packed She was diagnosed with familial type HLH based red cells and platelet transfusion and recovered on clinical presentation at very young age, blood with the initial treatment. She had regular follow count, biochemical results and BMAT findings, and
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