Macrophage Activation Syndrome as a Complication of Chronic Granulomatous Disease: A Case Report

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Liquidano Perez Eduardo; Carmona Berron Mariana; Carrillo Nieto Rosa Itzel; Corcuera Delgado Celso Tomas; Blancas Galicia Lizbeth; Scheffler Mendoza Selma Cecilia

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Journal Paper

Paper Information

Journal: Iranian Journal of Allergy, Asthma and Immunology Year:2023 | Volume:22 | Issue:6 Page(s): 600-603

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Title

Macrophage Activation Syndrome as a Complication of Chronic Granulomatous Disease: A Case Report

Author(s)

Keywords

Chronic granulomatous disease

Dihydrorhodamine 123

Immunologic deficiency syndromes

Macrophage activation syndrome

Abstract

Chronic granulomatous disease (CGD) presents with granuloma formation and lethal infections. It is inherited in an autosomal or X-linked recessive pattern. We describe a 10-month-old patient with a fatal secondary HLH as a CGD primary manifestation. We carried out an autopsy and found noncaseating granulomas, an aspergilloma in the lung, and hemophagocytosis. We performed a DHR assay on the patient’s mother and grandmother, showing a bimodal pattern conclusive of X-linked CGD. Thus, our definitive diagnosis was CGD complicated by Macrophage activation syndrome. CGD is caused by phagocytes’ inability to control pathogens, resulting in granulomas. Secondary HLH is a severe complication and could be characterized by the proliferation of macrophages and T lymphocytes and the production of proinflammatory cytokines. The early suspicion of this presentation helps establish a specific treatment, and the study of the carriers helps determine the etiology.

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