Hemophagocytic lymphohistiocytosis: a case series analysis in a pediatric hospital

Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical laboratory condition with high mortality rates, resulting from ineffective overactivation of the immune system. Data in the Brazilian literature is scarce, contributing to the challenge in standardizing conducts and performing an early diag...

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Bibliographic Details
Published in:Hematology, Transfusion and Cell Therapy Transfusion and Cell Therapy, 2023-01, Vol.45 (1), p.32-37
Main Authors: Santos, Itallo Oliveira, Neto, Ricardo Pasquini, Bom, Ana Paula Kuczynski Pedro
Format: Article
Language:eng ; por
Subjects:
Hemophagocytic lymphohistiocytosis
Hemophagocytic syndrome
MEDICINE, GENERAL & INTERNAL
Original
Pediatrics
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Summary:Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical laboratory condition with high mortality rates, resulting from ineffective overactivation of the immune system. Data in the Brazilian literature is scarce, contributing to the challenge in standardizing conducts and performing an early diagnosis of HLH. To describe the clinical, laboratory, and evolutionary findings on HLH patients treated at a pediatric hospital. This is an observational, cross-sectional and retrospective study on children diagnosed with HLH, hospitalized between 2009 and 2019. The diagnostic criteria were those described in the Histiocyte Society protocol. The authors evaluated HLH patient laboratory tests, myelograms and bone marrow biopsies, clinical characteristics and therapy. Twenty-three patients were included, 52.2% of whom were males. The age at diagnosis ranged from one to one hundred and eighty months. Four cases were classified as Primary HLH and nineteen, as Secondary HLH. The main triggers were infections and rheumatological diseases. All children had bicytopenia, and 95.4% had hyperferritinemia. Nineteen patients had liver dysfunction, sixteen had neurological disorders and fourteen had kidney injury. Pulmonary involvement was seen in 61.9%, acting as a worse prognosis for death (p = 0.01). Nine patients underwent the immuno-chemotherapy protocol proposed in the HLH 2004. The time to confirm the diagnosis varied from five to eighty days. The lethality found was 56.3%. The present study is the most extensive retrospective exclusively pediatric study published in Brazil to date. Despite the limitations, it was possible to demonstrate the importance of discussing HLH as a pediatric emergency.
ISSN:2531-1379
2531-1387
2531-1387
DOI:10.1016/j.htct.2021.04.006