alpha-chain disease

Occurs mainly in young adults, most commonly in Mediterranean area. Characterized by massive inflitration of the intestinal lamina propria & abdominal lymph nodes by lymphocytes, plasma cells, & histiocytes. Villous atrophy, malabsorption, diarrhea, steatorrhea, & hypocalcemia result from the infiltration. May evolve into immunoblastic B cell lymphoma.

Properties

PREVALENCE: EXTREMELY-RARE

References

Cotran et al Robbins Pathologic Basis of Disease, W.B. Saunders Co, Philadelphia, PA 1989 pg 744