Abstract Ref Number = APCP936
A CASE REPORT OF HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS
Ruth Yanti Pratiwi Silaban,Max Nathanael,Esther Rita Haris,Melissa A Tjahyadi,Edi S Tehuteru
RS Umum Bethesda Serukam General Practitioner, RS Umum Bethesda Serukam, Bengkayang, Kalimantan Barat Department of Paediatric, RS Umum Bethesda Serukam, Bengkayang, Kalimantan Barat Department of Paediatric, SubHematology Oncology, RS Dharmais, Jakarta
Background : Hemophagocytic lymphohistiocytosis (HLH) is a rare disease with major diagnostic and therapeutic difficulties especially in the rural hospitals. There are primary form familial HLH and secondary form HLH. The diagnosis based on molecular examination or diagnostic criteria as follows : fever, splenomegaly, cytopenias, hypertriglyseridemia and or hypofibrinogenemia, hemophagocytosis in bone marrow/spleen/lymph nodes, no evidence of malignancy, low or absent NK cell activity, elevation of ferritin, and elevation of CD 25. Due to the rarity of HLH, this case was presented to enlighten clinicians about the disease to prevent misdiagnosis and further delayed the treatment.
Case Presentation Summary : A ten years old girl came with complains of weakness and pallor. She also had fever, vomitus, and weight loss. In physical examination we found gum bleeding, lymphadenopathy, hepatosplenomegaly, and petechiae in both hands. Her blood exam showed pancytopenias. She’s admitted before in another hospital and treated as dengue fever, also given blood transfusion, but there was no improvement. Bone marrow aspiration and puncture was performed in our hospital and the sample was sent to a cancer hospital in Jakarta. The result was hemophagocytosis syndrome. Then we decided to give the chemotherapy using the protocol HLH (dexamethasone, etoposide, cyclosporine A). During the period of treatment , she got pneumonia and treated well. At the end of the treatment we repeated the bone marrow analysis to evaluate the outcome and showed remission. Now she has finished the treatment and being well. She came every 3 months for follow up.
Learning Points/Discussion : The outcome of HLH has improved during the last decades, thus early diagnosis and treatment are important to increase the survival rate.
Keywords: hemophagocytic lymphohistiocytosis chemotherapy