A UNIQUE CLINICAL PRESENTATION OF COMMON VARIABLE IMMUNODEFICIENCY: MALAKOPLAKIA OF RECTUM.

Abstract

<h3>Introduction</h3> Common variable immunodeficiency (CVID) is the most common primary immunodeficiency in adults and present with a broad range of clinical manifestations including granulomatous diseases. Malakoplakia is a rare chronic granulomatous disease (CGD) that can affect multiple organs and shown as a manifestation in immunocompromised individuals. In this case report, we present rectal malakoplakia as a clinical manifestation of a CVID in patient for the first time. <h3>Case Description</h3> This is a 62-year-old man with no significant past medical history except hypertension. He did not report recurrent infections, autoimmune disorders or malignancy. Recently, the patient underwent first screening colonoscopy, had a resection of one tubular polyp of rectum and the biopsy of polyp contained malakoplakia. Underlying etiology for rectal malakoplakia was investigated and all results were unremarkable except serum protein electrophoresis showing hypogammaglobulinemia without monoclonal gammopathy. Serum immunoglobulin levels were low (IgG: 538, IgA: 65 and IgM: 14, mg/dL). Flow cytometry analyses of lymphocytes showed markedly decreased absolute cell counts of CD4⁺ (221/ul) and CD19⁺ (28/ul) cells with low memory and switched-memory B-cells. <h3>Discussion</h3> This is a unique case of a completely asymptomatic patient who was incidentally found to have rectal malakoplakia. Work up for malakoplakia led to the diagnosis of CVID. CVID present with a broad range of clinical manifestations including granulomatous disease, however, rectal malakoplakia - a rare chronic granulomatous disease, was never reported as a manifestation of CVID. Although this patient is currently asymptomatic, it will be crucial to closely monitor his immune profile and clinical manifestations.