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There were 11 patients, 8 women and 3 men (sex ratio M/F: 0.375). Age ranged from 19 to 74 years, with an average of 43 years. Diagnostic delay averaged 4.1 years. On admission, the majority of patients (63.64%) presented with associated digestive and extra-digestive signs. Whereas 36.36% of patients had isolated extra-digestive signs, and only 9% had exclusively digestive signs. Biologically, hypoalbuminemia and hypoprotidemia were the most frequent biological abnormalities. Anemia was observed in all patients. Hypocholesterolemia was associated in 8 cases and hypocalcemia in 5. Etiological investigation revealed Crohn's disease in 1 case, celiac disease in 2 cases, lymph node tuberculosis in one case and CVID in 1 case. It was negative in 7 cases. Symptomatic measures, with or without specific treatment, were indicated. However, infectious and thrombotic complications were noted, leading to death in 3 patients.