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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.