Hepatic veno-oclussive disease with immunodeficiency syndrome

Patients have Pneumocystis jerovici infection, enteroviral infection or mucocutaneous candidiasis. Hepatic vascular oclusion disease was verified by biopsy. B cell immunodeficiency include hypogamaglobulinemia, absent memory B cells and tonsilar lymph nodes. Absence of lymph node germinal centers and tissue plasma cells. T cell immunodeficiency include reduced number of memory T cells, and low or reduced intracellular T cell cytokine expression, IL2, IL4, and IL10.

Diagnostic laboratories



Therapeutic options

  • Intravenous immunoglobulin and Pneumocystis jerovici prophylaxis.

Research programs, clinical trials