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Open Access
CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2019; 40(02): 286
DOI: 10.4103/ijmpo.ijmpo_197_17
Case Report

Hemophagocytic Lymphohistiocytosis Secondary to Malignancy and Chemotherapy in Pediatric Patients: A Single-Institution Experience

Authors

  • Justin Haloot

    Texas College of Osteopathic Medicine, University of North Texas Health Science Center, Fort Worth, Texas, USA

  • Mahdi Kaheri

    Texas College of Osteopathic Medicine, University of North Texas Health Science Center, Fort Worth, Texas, USA

  • Tyler Hamby

    Departments of Research Operations, Cook Children’s Health Care System, Fort Worth, Texas, USA

  • Anish Ray

    Departments of Pediatric Hematology/Oncology, Cook Children’s Health Care System, Fort Worth, Texas, USA

Financial support and sponsorship Nil.
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Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an uncommon yet potentially devastating systemic disease, arising from uncontrolled activation of the immune system. While the primary form of this disease can be caused by genetic mutation(s), the secondary form may be triggered by infection and hematologic, malignant, and metabolic conditions. The diagnosis of HLH remains a clinical challenge due to nonspecific symptoms. Proper diagnosis is significantly more difficult among patients with acute leukemia who have received chemotherapy. The objective of this study is to describe three unique cases of secondary HLH, describe the specific treatment, and improve the awareness of this condition. Two patients with acute myeloid leukemia (AML) and one with acute lymphoblastic leukemia were diagnosed with HLH, having fulfilled the criteria as outlined in the HLH-2004 protocol. They then received HLH-specific treatment. Two patients passed – one from refractory HLH and one from primary disease (i.e., AML) – and one patient remains alive 22 months after her allogeneic bone marrow transplant. The diagnosis of HLH requires the presence of any five of the eight criteria. Due to its heterogeneous presentation, it remains imperative that treating clinicians remain cognizant about HLH so that prompt diagnosis may allow appropriate treatment.

Keywords

Acute leukemia - ferritin - hemophagocytic lymphohistiocytosis


Publication History

Article published online:
03 June 2021

© 2019. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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