Case Report
Giant Cell Myocarditis in a 12-Year-Old Girl With Common Variable Immunodeficiency

https://doi.org/10.4065/77.1.92Get rights and content

Giant cell myocarditis (GCM) is a rare and often fatal disease that infrequently affects children. Common variable immunodeficiency (CVID) describes a heterogeneous group of disorders characterized by hypogammaglobulinemia and poor specific antibody responses. To our knowledge, CVID and GCM have not been reported together in 1 patient. We describe a 12-year-old girl with CVID who developed acute severe GCM that necessitated cardiac transplantation. Histopathological and immunohistochemical studies of the endomyocardial biopsy specimen and the explanted heart revealed numerous histiocytes, eosinophils, T cells, and multinucleated giant cells. Both CVID and GCM are thought to involve dysregulation of T-cell function and have been associated with a similar spectrum of autoimmune conditions. The coincidence of CVID and GCM in a single patient may reflect a pathophysiologic connection.

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REPORT OF A CASE

A 12-year-old girl with a history of CVID presented to the emergency department after 3 days of fatigue, chest pain, mild exertional dyspnea, and an episode of near-syncope while showering. No viral prodrome had been noted. She was tachycardic with a heart rate of 150 beats/min, her blood pressure was 90/54 mm Hg, and she had an oxygen saturation of 99% by pulse oximetry. Findings on physical examination were remarkable for hepatomegaly and a third heart sound. Electrocardiography revealed

PATHOLOGIC FINDINGS

The initial endomyocardial biopsy confirmed the diagnosis of diffuse acute myocarditis, with a marked mixed inflammatory infiltrate of lymphocytes, eosinophils, and histiocytes associated with extensive myocyte damage and necrosis. Eosinophils were numerous and formed small focal cellular aggregates. The cardiectomy specimen exhibited a softened myocardium with increased thickness of the ventricular walls. On cut section, the myocardium appeared mottled with widespread light tan nodules

DISCUSSION

Giant cell myocarditis is an often fulminant form of myocardial inflammation with a high mortality rate. Most re ported cases have occurred in adults. The largest series described 63 patients ranging in age from 15 to 69 years (median, 43 years), with equal incidence in males and females.1 Although rare, GCM accounted for 22% of all cases of biopsy-verified myocarditis diagnosed at 1 major academic center during a 13 72-year period.3 No data are available about the epidemiology of GCM in

CONCLUSION

To our knowledge, this is the first case of GCM reported in a patient with CVID, and this patient is one of the youngest described with GCM. Since both disorders are thought to represent disordered T-cell function and have been encountered with similar autoimmune conditions, it may be that their association in this case reflects an underlying pathophysiologic connection. Confirmation of this hypothesis will require the identification of other examples of the association of these 2 disorders and

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    The Children's Hospital and Harvard Medical School, Boston, Mass.

    1

    Mr Laufs, a medical student at the Medical University of Lübeck, Germany, contributed to this work while participating in a student clerkship.

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