Abstract Ref Number = APCP945
HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS: AN APPROACH TO GENERAL PEDIATRICS IN ESTABLISHING DIAGNOSIS
Fajar Subroto, Syarif Rohimi, Ina Zarlina, Endah Citraresmi, Hermien W Moeryono,
Yosanti Elsa, Lydia Pratanu, Novitria Dwinanda, Tinuk Meilany, Amar Widhiani
Department of Pediatrics, Department of Clinical Pathology, Clinical Genetics Laboratory
Harapan Kita Women and Children Hospital
Background: Hemophagocytic lymphohistiocytosis (HLH) is rare, fatal, and diverse with many causes and under-recognised, contributes to high morbidity and mortality. Early recognition is crucial for therapy.
Case: A 2-year-old boy, 15 kg, was referred with fever for 6 days, with working diagnosis acute leukemia dd/ sepsis. Upon admission patient was alert, no jaundice. Heart rate 155 bpm, respiratory rate 28x/m without dyspnea, temperature 37,8-39oC, blood pressure 100/60 mmHg. He had silver-coloured hair, edematous eyelids, anemic conjunctiva, normal jugular venous pressure, no lymph enlargement. Patient had ascites, liver enlarged 3 cm bac/3 cm bpx, spleen enlarged Schuffner III, rash with non-pitting edema on upper, lower extremities, and scrotum. Hb 6 g/dL, WBC 6420, diff count 5/6/0/17/58/18, Ht 29%, Platelet 70.000, MCV 67, MCH 22, MCHC 33, BSR 19, CRP 23, albumin 2.8 g/dL. Abdominal ultrasound found no tumor, acites, hepatomegaly, splenomegaly, gallbladder edema, bilateral pleura effusion. Peripheral blood smear: abnormal WBC, neutropenia, no azurophilic granule, abnormal thrombocyte count with normal morphology. Bone marrow aspiration found MDS without any signs of leukemia. During 1st week patient still suffered from fever, acites, hepatosplenomegaly, and hypoalbuminemia. Total protein 4.5, albumin 2.5, AST 94, ALT, GGT 54, alkali phosphatase 186, hypertriglyseridemia procalcitonin<0.5, ferritin 13,900, NK cell and Ig GAME normal, IgG Cytomegalovirus 22,0. The patient had 5 out of 9 HLH diagnostic criteria: fever, splenomegaly, cytopenias, hypertriglyceridemia, elevated ferritin, low or absent natural killer, elevated CD25.
Conclusion:. HLH can be diagnosed with 5 of 9 diagnostic criteria
Keywords: HLH diagnostic criteria