Hemophagocytic lymphohistiocytosis in adults: An under recognized entity

Abdul Rashid Shah; Tariq Muzzafar; Rita Assi; Dawid Schellingerhout; Zeev Estrov; Gevorg Tamamyan; Hagop Kantarjian; Naval Daver

doi:10.1016/j.bbacli.2016.12.002

Hemophagocytic lymphohistiocytosis in adults: An under recognized entity

Abdul Rashid Shah, Tariq Muzzafar, Rita Assi, Dawid Schellingerhout, Zeev Estrov, Gevorg Tamamyan, Hagop Kantarjian, Naval Daver

Research output: Contribution to journal › Article › peer-review

14 Scopus citations

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation with macrophage and T-cell infiltration resulting in, multi organ damage. HLH may be primary or secondary in etiology. A high index of suspicion is essential for early diagnosis and treatment. Diagnostic criteria need to be refined and newer treatment options to be explored in order to improve survival especially in adult HLH and malignancy-associated HLH (M-HLH). We report a case of malignancy associated HLH (M-HLH) in adult treated on one of the only FDA-approved protocols for adult HLH to highlight the diagnostic and therapeutic challenges of this disease entity.

Original language	English (US)
Pages (from-to)	36-40
Number of pages	5
Journal	BBA Clinical
Volume	7
DOIs	https://doi.org/10.1016/j.bbacli.2016.12.002
State	Published - Jun 1 2017

Keywords

Adults
Ferritin
Hemophagocytosis
Lymphohistiocytosis

ASJC Scopus subject areas

Pathology and Forensic Medicine
Molecular Medicine
Physiology (medical)

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10.1016/j.bbacli.2016.12.002

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title = "Hemophagocytic lymphohistiocytosis in adults: An under recognized entity",

abstract = "Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation with macrophage and T-cell infiltration resulting in, multi organ damage. HLH may be primary or secondary in etiology. A high index of suspicion is essential for early diagnosis and treatment. Diagnostic criteria need to be refined and newer treatment options to be explored in order to improve survival especially in adult HLH and malignancy-associated HLH (M-HLH). We report a case of malignancy associated HLH (M-HLH) in adult treated on one of the only FDA-approved protocols for adult HLH to highlight the diagnostic and therapeutic challenges of this disease entity.",

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T1 - Hemophagocytic lymphohistiocytosis in adults

T2 - An under recognized entity

AU - Shah, Abdul Rashid

AU - Muzzafar, Tariq

AU - Assi, Rita

AU - Schellingerhout, Dawid

AU - Estrov, Zeev

AU - Tamamyan, Gevorg

AU - Kantarjian, Hagop

AU - Daver, Naval

PY - 2017/6/1

Y1 - 2017/6/1

N2 - Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation with macrophage and T-cell infiltration resulting in, multi organ damage. HLH may be primary or secondary in etiology. A high index of suspicion is essential for early diagnosis and treatment. Diagnostic criteria need to be refined and newer treatment options to be explored in order to improve survival especially in adult HLH and malignancy-associated HLH (M-HLH). We report a case of malignancy associated HLH (M-HLH) in adult treated on one of the only FDA-approved protocols for adult HLH to highlight the diagnostic and therapeutic challenges of this disease entity.

AB - Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation with macrophage and T-cell infiltration resulting in, multi organ damage. HLH may be primary or secondary in etiology. A high index of suspicion is essential for early diagnosis and treatment. Diagnostic criteria need to be refined and newer treatment options to be explored in order to improve survival especially in adult HLH and malignancy-associated HLH (M-HLH). We report a case of malignancy associated HLH (M-HLH) in adult treated on one of the only FDA-approved protocols for adult HLH to highlight the diagnostic and therapeutic challenges of this disease entity.

KW - Adults

KW - Ferritin

KW - Hemophagocytosis

KW - Lymphohistiocytosis

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JO - BBA Clinical

JF - BBA Clinical

ER -

Hemophagocytic lymphohistiocytosis in adults: An under recognized entity

Abstract

Keywords

ASJC Scopus subject areas

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