Features of Hemophagocytic Lymphohistiocytosis in Infants With Severe Combined Immunodeficiency: Our Experience From Chandigarh, North India.

ABSTRACT:

Background

Hemophagocytic lymphohistiocytosis (HLH) is characterized by uncontrolled and excessive inflammation leading to high mortality. Aetiology of HLH can be primarily due to genetic causes or secondarily due to infections or rheumatological illness. However, rarely T-cell deficiencies like severe combined immunodeficiency (SCID) can develop HLH.

Objective

To describe clinical and laboratory features of SCID cases who developed HLH.

Methods

We collected clinical, laboratory, and molecular details of patients with SCID who developed HLH at our center at Chandigarh, North India.

Results

Of the 94 cases with SCID, 6 were noted to have developed HLH-like manifestations. Male-female ratio was 5:1. Median (inter-quartile range) age of onset of clinical symptoms

SUBMITTER: Vignesh P 

PROVIDER: S-EPMC9260510 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature