Hemophagocytic syndrome in systemic lupus erythematosus

Agata Liszewska; Ewa Robak; Małgorzata Bernacka; Anna Woźniacka; Jarosław Bogaczewicz; Krzysztof Chojnowski

doi:10.5114/dr.2019.86913

eISSN: 2084-9893
ISSN: 0033-2526

Dermatology Review/Przegląd Dermatologiczny

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3/2019
vol. 106

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abstract:

Case report

Hemophagocytic syndrome in systemic lupus erythematosus

Agata Liszewska

¹

,

Ewa Robak

¹

,

Małgorzata Bernacka

¹

,

Anna Woźniacka

¹

,

Jarosław Bogaczewicz

¹

,

Krzysztof Chojnowski

²

Department of Dermatology and Venereology, Medical University of Lodz, Poland/Katedra Dermatologii i Wenerologii Uniwersytetu Medycznego w Łodzi, Polska
Department of Blood Coagulation Disorders, Medical University of Lodz, Poland/Zakład Zaburzeń Krzepnięcia Krwi Uniwersytetu Medycznego w Łodzi, Polska

Dermatol Rev/Przegl Dermatol 2019, 106, 302–309

DOI: https://doi.org/10.5114/dr.2019.86913

Online publish date: 2019/08/24

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Introduction
A hemophagocytic syndrome is a life-threatening disorder, which develops as a result of the activation of macrophages. It may be divided into two distinct types. A primary hemophagocytic syndrome is caused by genetic mutations while secondary may occur in the course of infections, malignancies, or autoimmune disorders. When constituting a complication of autoimmune diseases, the hemophagocytic syndrome is commonly referred to as macrophage activation syndrome.

Objective
We report a case of secondary hemophagocytic syndrome in the course of systemic lupus erythematosus to draw attention to this condition, which may often be under-diagnosed in patients with autoimmune disorders.

Case Report
A 27-year-old woman was admitted to the Department of Dermatology with symptoms resembling an exacerbation of systemic lupus erythematosus. The hemophagocytic syndrome was diagnosed by clinical manifestation, i.e., fever, hemophagocytosis in bone marrow aspirate, neutropenia, increased ferritin level, and hypertriglyceridemia.

Conclusions
Hemophagocytic syndrome should be taken into consideration in systemic lupus erythematosus patients as it can mimic an acute flare of the disease.

keywords:

Hemophagocytic syndrome in systemic lupus erythematosus

Agata Liszewska 1 , Ewa Robak 1 , Małgorzata Bernacka 1 , Anna Woźniacka 1 , Jarosław Bogaczewicz 1 , Krzysztof Chojnowski 2

systemic lupus erythematosus, hemophagocytic syndrome, macrophage activation syndrome, hemophagocytosis

Agata Liszewska

¹

,

Ewa Robak

¹

,

Małgorzata Bernacka

¹

,

Anna Woźniacka

¹

,

Jarosław Bogaczewicz

¹

,

Krzysztof Chojnowski

²