The lymphoid infiltrate of the lamina propria of the intestinal mucosa results in villous atrophy, malabsorption, steatorrhea, diarrhea, and hypocalcemia. An abdominal mass of lymphoid tissue is often present. Serum a chains must be demonstrated for the diagnosis. Presenting symptoms are most often abdominal pain, diarrhea, and malabsorption. In time a HCD may progress to a large cell B - immunoblastic lymphoma.
Survival is variable from a few months to several years.
No lytic bone lesions are present but plasmacytoid lymphocytes, plasma cells, eosinophils and histiocytes diffusely infiltrate the marrow and lymph nodes. As many as one third of g HCD patients have an associated autoimmune disease including AIHA, Sjogren's syndrome, rheumatoid arthritis, SLE, thyroiditis, etc. As with a HCD, survival in g HCD varies greatly from months to years. Death is most often secondary to infection. Marrow plasma cells are characteristically vacuolated.