Secondary Hemophagocytic Lymphohistiocytosis in Post-COVID-19 Patients: A Report of Two Cases

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Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a disease that can affect both children and adults. HLH can be categorized as primary or secondary. Secondary HLH (sHLH) may be secondary to various viral infections. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) virus infection is a pandemic with multi-system involvement. HLH in COVID-19 positive patients is a recognized entity. However, in post-COVID-19 patients who have recovered and are negative by serological tests and reverse transcription-polymerase chain reaction test may present with sHLH due to dysregulation of the immune system. We highlight this unusual finding of post-COVID-19 sHLH in two cases, who were diagnosed by the new revised H-score.

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Most cited references 15

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Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome.

Laurence Fardet, Lionel Galicier, Olivier Lambotte … (2014)

Because it has no unique clinical, biologic, or histologic features, reactive hemophagocytic syndrome may be difficult to distinguish from other diseases such as severe sepsis or hematologic malignancies. This study was undertaken to develop and validate a diagnostic score for reactive hemophagocytic syndrome.

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Hemophagocytic lymphohistiocytosis: review of etiologies and management

Melissa George (2014)

Hemophagocytic lymphohistiocytosis (HLH) covers a wide array of related life-threatening conditions featuring ineffective immunity characterized by an uncontrolled hyperinflammatory response. HLH is often triggered by infection. Familial forms result from genetic defects in natural killer cells and cytotoxic T-cells, typically affecting perforin and intracellular vesicles. HLH is likely under-recognized, which contributes to its high morbidity and mortality. Early recognition is crucial for any reasonable attempt at curative therapy to be made. Current treatment regimens include immunosuppression, immune modulation, chemotherapy, and biological response modification, followed by hematopoietic stem-cell transplant (bone marrow transplant). A number of recent studies have contributed to the understanding of HLH pathophysiology, leading to alternate treatment options; however, much work remains to raise awareness and improve the high morbidity and mortality of these complex conditions.

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Hemophagocytic lymphohistiocytosis: an update on diagnosis and pathogenesis.

Flavia G N Rosado, Annette S Kim (2013)

Hemophagocytic lymphohistiocytosis (HLH) is a frequently fatal and likely underdiagnosed disease involving a final common pathway of hypercytokinemia, which can result in end-organ damage and death. Although an early diagnosis is crucial to decrease mortality, the definitive diagnosis is often challenging because of the lack of specificity of currently accepted diagnostic criteria and the absence of confirmatory gold standards. Because of the wide range of laboratory assays involved in the diagnosis of HLH, practicing pathologists from a broad spectrum of clinical specialties need to be aware of the disease so that they may appropriately flag results and convey them to their clinical counterparts. Our article summarizes these new advances in the diagnosis of HLH and includes a review of clinical findings, updated understanding of the pathogenesis, and promising new testing methods.

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Author and article information

Journal

Journal ID (nlm-ta): Cureus

Journal ID (iso-abbrev): Cureus

Journal ID (issn): 2168-8184

Title: Cureus

Publisher: Cureus (Palo Alto (CA) )

ISSN (Electronic): 2168-8184

Publication date (Electronic, pub): 20 August 2021

Publication date (Electronic, collection): August 2021

Volume: 13

Issue: 8

Electronic Location Identifier: e17328

Affiliations

[1 ] Pathology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, IND

[2 ] Internal Medicine, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, IND

[3 ] Paediatrics, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, IND

Author notes

Biswajit Dey drbish25@ 123456rediffmail.com

Article

DOI: 10.7759/cureus.17328

PMC ID: 8450015

PubMed ID: 34557370

SO-VID: b5d3be1b-11e1-4006-a999-c3fc5dfab68c

License:

This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

History

Date accepted : 20 August 2021

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