Secondary Hemophagocytic Lymphohistiocytosis in
Children : An Analysis of Etiology and Outcome
GA VIV ANN VEERAKUL, M.D.*, KLEEBSABAI SANPAKIT, M.D.*,
VORA V ARN S. TANPHAICHITR, M.D., M.S.*, CHULARATANA MAHASANDANA, M.D.*,
NONGNAPA JIRARATTANASOPA, M.D.*
Affiliation : *Division of Hematology/Oncology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University,
Bangkok 10700, Thailand.
AbstractFifty-two pediatric patients were diagnosed with secondary hemophagocytic lymphohistio
cytosis (HLH) at the Department of Pediatrics, Siriraj Hospital between 1989 and 1998. Of these,
15 were infection-associated (IAHS), 25 were malignancy-associated (MAHS) and 12 were idiopathic
HLH. Causative organisms for IAHS were Salmonella (3), Staphylococcus (2), enterobactor (2),
dengue virus (3), malaria (2) and one each of Ebstein Barr virus (EBV), Serratia marcesens and
Penicillium maneffei. Unlike those reported in adults and in the Western literature, 47 of 52 children
in the present series were immunocompetent hosts. In addition, the proportion of MAHS was higher
than expected (48.1 %). Twenty-two of 25 MAHS presented with hemophagocytic syndrome and
were subsequently found to have malignant diseases. Sixty per cent of MAHS (15 cases) were
associated with non-Hodgkin's lymphoma (NHL), mainly T-cell. Other malignancies included acute
leukemias (7) MDS (1), Langerhans cell histiocytosis (1) and histiocytic sarcoma (1). Treatment
approaches were specific therapy for individuals with known cause~;. Supportive treatment with blood
components transfusions, steroid, intravenous immunoglobulins (IVIG), and chemotherapeutic agents,
mainly vinblastine and etoposides, were used in indicated cases. Of the 52 cases, 15 (28.8%) had a
fatal outcome during the acute phase, and other 4 died of their subsequent malignant diseases. There
was a statistically significant association between poorer prognosis and patients' age < 3 years (p=
0.004) or MAHS (p=0.005).
Conclusion : Secondary HLH is not uncommon in Thai children who are immunocom
petent. Malignancies, particulary NHL, are highly suspicious especially for cases not responsive to
conventional therapy. Poor prognostic factors are age less than 3 years and MAHS.
Keywords : Secondary Hemophagocytosis, Infection-Associated Hemophagocytic Syndrome
(IAHS), Malignancy-Associated Hemophagocytic Syndrome (MAHS)
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