open access

Vol 54, No 1 (2023)
Original research article
Submitted: 2022-09-12
Accepted: 2022-11-01
Published online: 2023-02-02
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Clinicomorphological spectrum of hemophagocytic syndrome in a tertiary care hospital

Abinaya Sundari1, Prasanna N Kumar1
DOI: 10.5603/AHP.a2023.0005
·
Acta Haematol Pol 2023;54(1):36-42.
Affiliations
  1. PSG Institute of Medical Sciences & Research, Coimbatore, India

open access

Vol 54, No 1 (2023)
ORIGINAL RESEARCH ARTICLE
Submitted: 2022-09-12
Accepted: 2022-11-01
Published online: 2023-02-02

Abstract

Introduction: The HLH-2004 trial established the diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH), a severe hyperinflammatory condition. It typically develops due to inappropriate macrophage activation. Our objective was to assess the spectrum of hemophagocytic syndrome presentations by identifying hemophagocytic activity in the bone marrow, and to unravel the etiopathogenesis of this condition. Material and methods: A retrospective study was carried out in the Department of Pathology in a tertiary care hospital reporting the clinical and laboratory findings of patients who had been previously diagnozed with hemophagocytosis in the bone marrow. The parameters in the diagnostic criteria of HLH of the same patients were documented and analyzed. Results: The characteristics of the 32 patients who presented with hemophagocytosis in the bone marrow were documented. Persistent fever was the most frequent presentation. Mild to moderate anemia (69%), severe leucopenia (59%), and mild to moderate thrombocytopenia (63%) were other frequent findings. The incidence of primary HLH was found to be only 3%; 87% had hyperferritinemia, 78% had bicytopenia, 59% had hypertriglyceridemia, and 53% had splenomegaly. Infections followed by malignancies were shown to be the most frequent cause of secondary HLH, while the prognosis for malignancy-associated HLH appeared to be poor. Conclusions: Based on the findings of this study, conclusions about the clinical symptoms and etiologies of HLH may be drawn, which will assist in early identification. Hence, all subjects with a clinical suspicion of HLH should be thoroughly investigated for a possible etiology.

Abstract

Introduction: The HLH-2004 trial established the diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH), a severe hyperinflammatory condition. It typically develops due to inappropriate macrophage activation. Our objective was to assess the spectrum of hemophagocytic syndrome presentations by identifying hemophagocytic activity in the bone marrow, and to unravel the etiopathogenesis of this condition. Material and methods: A retrospective study was carried out in the Department of Pathology in a tertiary care hospital reporting the clinical and laboratory findings of patients who had been previously diagnozed with hemophagocytosis in the bone marrow. The parameters in the diagnostic criteria of HLH of the same patients were documented and analyzed. Results: The characteristics of the 32 patients who presented with hemophagocytosis in the bone marrow were documented. Persistent fever was the most frequent presentation. Mild to moderate anemia (69%), severe leucopenia (59%), and mild to moderate thrombocytopenia (63%) were other frequent findings. The incidence of primary HLH was found to be only 3%; 87% had hyperferritinemia, 78% had bicytopenia, 59% had hypertriglyceridemia, and 53% had splenomegaly. Infections followed by malignancies were shown to be the most frequent cause of secondary HLH, while the prognosis for malignancy-associated HLH appeared to be poor. Conclusions: Based on the findings of this study, conclusions about the clinical symptoms and etiologies of HLH may be drawn, which will assist in early identification. Hence, all subjects with a clinical suspicion of HLH should be thoroughly investigated for a possible etiology.

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Keywords

bone marrow, hemophagocytic lymphohistiocytosis (HLH), hyperferritinemia, primary HLH, secondary HLH

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Title

Clinicomorphological spectrum of hemophagocytic syndrome in a tertiary care hospital

Journal

Acta Haematologica Polonica

Issue

Vol 54, No 1 (2023)

Article type

Original research article

Pages

36-42

Published online

2023-02-02

Page views

430

Article views/downloads

44

DOI

10.5603/AHP.a2023.0005

Bibliographic record

Acta Haematol Pol 2023;54(1):36-42.

Keywords

bone marrow
hemophagocytic lymphohistiocytosis (HLH)
hyperferritinemia
primary HLH
secondary HLH

Authors

Abinaya Sundari
Prasanna N Kumar

References (20)
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