Page 247 - Abstract Book KONIKA 18

Hemato-Oncology

P-HO-038
Hemophagocytic Lymphohystiocytosis in Disseminated Histoplasmosis Infection:
A Case Report

Gerin Orviyanti, Yetty Movieta Nency
Department of Child Health, Faculty of Medicine Universitas Diponegoro, Dr. Kariadi General Hospital,
Semarang, Central Java, Indonesia

Abstract
Background Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal condition marked by
disregulated and prolonged activation of cytotoxic T lymphocytes and natural killer cells. Histoplasmosis-
associated HLH is a rare condition with limited information on appropriate therapy and its outcome in
children. Objective We report the clinical characteristics, therapy, and outcome of patients with histoplasmosis-
associated HLH. Case A 10 years old boy presented with prolonged fever, pale, epistaxis, and cough. Upon
physical examination, he had dyspnea, splenomegaly and petechiae. Six out of 8 clinical criteria of Histiocyte
Society HLH-2004 was met : fever, cytopenia (hemoglobin 6.8 gr/dL, ANC 490/uL, platelet (26.000/
uL), hypertriglyceridemia (652 mg/dL) and hypofibrinogenemia (97 mg/dL), hyperferitinemia (>24.000
ng/mL), and hemophagocytosis in bone marrow. Bone marrow histology showed increased activity of
hemophagocytosis and there were Histoplasma capsulatum inside. Culture of bone marrow aspirate found
Histoplasma capsulatum growing. Both indicated a HLH secondary to histoplasmosis. He was treated with
intravenous antibiotic, antifungal, corticosteroid, and transfusions. His condition worsened and complicated
with metabolic acidosis. He was died few days later because of the disseminated intravascular coagulation
(DIC) condition. Conclusion Histoplasmosis-associated HLH has poor prognosis with DIC as most common
complication. Knowledge of HLH clinical characteristics is important to establish diagnosis early and provide
early management.
Keywords hemophagocytic lymphohystiocytosis; disseminated histoplasmosis

P-HO-039
Association between Serum Ferritin Levels and Ocular Abnormalities
in Children with Beta Thalassemia Major

Olga Rasiyanti Siregar , Bidasari Lubis , Siska Mayasari Lubis , Balgis Desy Sulistyowati 2
1
1
1
Department of Child Health, Faculty of Medicine, Universitas Sumatera Utara and
1
2
Department of Pediatric Opthalmology, Sabang Merauke Eye Centre (SMEC) , Medan, North Sumatera, Indonesia
Abstract
Background Mechanism of ocular abnormalities in thalassemia is multifactorial such as regular blood
transfusion therapy which causes iron overload. Iron deposition in the eye as a result of the disease or
as a side effect of iron chelators may lead to multiple ocular manifestations. Objective Assessing the
association between serum ferritin levels and ocular abnormalities in children with beta thalassemia major.
Methods A cross-sectional study was undertaken at the Thalassemia day-care centre, H. Adam Malik
General Hospital in Medan, Indonesia. Subjects who had history of corneal disease, using contact lens,
ocular trauma, previous ocular surgery, and those taking topical medications were excluded in this study.
The anterior region, fundus, and retina were screened for ocular anomalies. Chi square test and Spearman
test were used for statistical analysis. Results Thirty seven beta thalassemia major children ranging from 3
to 18 years old were enrolled. The median serum ferritin level was 2842 ng/mL, only 28 individuals (78.4%)
underwent iron chelation therapy. Papil edema was the most common ocular finding among the subjects
(27%), followed by complicated cataract (8.1%) and atrophy (5.4%). Reduced visual acuity was noticed
in 8 patients (21.6%). There was no significant difference between ocular abnormalities and serum ferritin
levels. Conclusion There was no significant difference between ocular abnormalities and serum ferritin levels,
however, regular ophthalmologic assessments were required to detect retinopathy and early alterations in
their visual system for a better quality of life.
Keywords: ocular abnormalities; thalassemia; children

KONIKA XVIII Abstract Book 199