Chronic diarrhea and malabsorption due to hypogammaglobulinemia: a report on twelve patients.

ABSTRACT

Hypogammaglobulinemic sprue (HGS), which may predispose to infection, is uncommon. Twelve patients (all men; median age 29 years, 15–50) with HGS (4%) of 296 with chronic small bowel diarrhea and malabsorption syndrome (MAS) during a 10-year period were analyzed. Treatment of HGS was delayed due to misdiagnosis as intestinal tuberculosis (n=7) and diarrhea-predominant irritable bowel syndrome (n=1). All had diarrhea and weight loss (median loss 12 Kg). Associated conditions were clubbing, bronchiectasis, and seizure (2 patients each), and hypothyroidism (n=1). Laboratory parameters were urinary D-xylose median 0.46 g/5 g/5 h (range 0.2–1.6; normal ≥1), fecal fat 11.9 g/day (3.8–16.7; normal ≤7 g), serum IgA, IgG, and IgM 23.5 mg/dL (17–114; normal 90–450), 584 mg/dL (145–1051; normal 800–1800), and 23 (0–40.3; normal 60–280). IgA, IgG, and IgM were low in 10, 10, and 11, respectively. Duodenal biopsy was normal in 6 patients and showed partial villous atrophy in 6 and nodular lymphoid hyperplasia in two. Associated infections were giardiasis (n=1), disseminated strongyloidiasis (1), small intestinal bacterial overgrowth (3), septicemia (2), and septic arthritis (1). Two patients died of sepsis, five are well on immunoglobulin and specific antiinfective treatment, and five are lost to follow up. Approximately 4% patients with MAS have hypogammaglobulinemia, which is often associated with infection and is diagnosed late.