Clinical case of hemophagocytic lymphohistiocytosis: rare or undiagnosed syndrome?

Authors

DOI:

https://doi.org/10.14739/2310-1210.2023.1.261972

Keywords:

hemophagocytic syndrome, macrophage activation syndrome, cytokine storm, hyperferritinemia, coagulopathy

Abstract

Hemophagocytic lymphohistiocytosis (hemophagocytic syndrome, HLH) is a life-threatening hyperinflammatory condition associated with a high mortality rate; it is characterized by hyperstimulation of histiocytes and cytotoxic T-cells, which leads to cytokine storm and multisystemic injury.

Aim. To present our own clinical case of the HLH development at the key aspects of pathogenic mechanisms, differential diagnosis, and therapeutic management of this syndrome.

Materials and methods. This article provides information on the HLH development in a 69-year-old man with the onset of this syndrome prior to a diagnosis of the underlying disease – splenic marginal zone lymphoma. The article summarizes the current literature data on clinical manifestations, diagnosis, and treatment of HLH.

Results. This article describes a case of secondary HLH from our clinical practice. The most common causes of HLH are malignant neoplasm, infectious factors and rheumatic diseases (when associated with the latest, HLH is called “macrophage activation syndrome”). The main clinical symptoms are prolonged high fever and hepatosplenomegaly, typical laboratory changes such as cytopenia, hyperferritinemia, hypertriglyceridemia, elevated liver enzymes and low fibrinogen levels.

Conclusions. Despite typical clinical features, HLH is a condition that often remains unrecognized and it is characterized by a poor prognosis. Prompt prescription of adequate treatment can improve patients’ prognoses and increase the survival rate.

Author Biographies

O. B. Yaremenko, Bogomolets National Medical University, Kyiv, Ukraine

MD, PhD, DSc, Professor, Head of the Department of Internal Medicine 3

A. O. Sydorova, Bogomolets National Medical University, Kyiv, Ukraine

MD, Assistant of the Department of Internal Medicine 3, Bogomolets National Medical University; Rheumatologist, General Practitioner, Medical Center “Universal Clinic “Oberig”, Kyiv, Ukraine

O. Ya. Antoniuk, Bogomolets National Medical University, Kyiv, Ukraine

MD, Assistant of the Department of Social Medicine and Public Health, Bogomolets National Medical University; Endocrinologist, General Practitioner, Medical Center “Universal Clinic “Oberig”, Kyiv, Ukraine

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Published

2023-02-15

How to Cite

1.
Yaremenko OB, Sydorova AO, Antoniuk OY. Clinical case of hemophagocytic lymphohistiocytosis: rare or undiagnosed syndrome?. Zaporozhye Med J [Internet]. 2023Feb.15 [cited 2023Dec.5];25(1):81-6. Available from: http://zmj.zsmu.edu.ua/article/view/261972