Abstract Ref Number = APCP915
HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS A CASE REPORT OF 9 MONTH OLD BOY WITH RECURRENT PANCYTOPENIA
Selvi Mella Maharani,Amallia Nugetsiana,Bambang Sudarmanto
Department of Pediatric Faculty of Medicine Diponegoro University Dr Kariadi Hospital Semarang
Background : Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome caused by immune dysregulation, commonly appears in infancy. It is characterized by pancytopenia, hepatosplenomegaly and hemophagocityc in bone marrow, liver or lymph nodes. This case report aims to outline the clinician awareness about clinical presentation of HLH.
Case Presentation Summary : 9 month old boy, first born of unconsanguineous marriage was admitted to Kariadi Hospital, referred from secondary hospital with complain of prolonged fever, hepatosplenomegally and pancytopenia. There was history of packed red cell and thrombocyte concentrate transfusion. Physical examination showed good general and nutritional status, hepatosplenomegally, no pale appearance, spontaneous bleeding, and lymph enlargement. Laboratory investigation revealed moderate anaemia, severe neutropenia, and thrombocytopenia. Blood smear showed giant thrombocyte, reticular cell and hypersegmentation of neutrophil, lymphocyte atypical that indicated to infection. The chest x ray associated with bronchopneumonia. Bone marrow aspiration revelead hypercellularity, elevated reticular cell activity along with hemophagocytosis, elevated micromegacariocyte and no blast cell dominancy, those were confirmed to HLH. Cefepime 25 mg/BW/12 hr as supportive theraphy was given due to febrile neutropenia. He was planned to receive chemoteraphy consist of dexamethasone, etoposide, methotrexate, prednisolone and cyclosporine-A. He was unable to got maximum chemoteraphy because he got worsened condition which were spontaneous bleeding, general seizure and decrease consciousness. He died three months after diagnosis.
Learning Points/Discussion : Hemophagocytic lymphohistiocytosis is a fatal disease. In many cases, patient has diagnosed in the late condition, that increases the mortality. Clinician should improve the awareness in patient who present with unexplained prolonged fever, organomegally and pancytopenia, due to a fatal hematology malignancy diagnosis.
Keywords: hemophagocytic lymphohistiocytosis infant