[Erythrophagocytic histiocytosis syndrome in a child with a primary immunodeficit].

The authors described a case of the syndrome of erythrophagocytic histiocytosis in an infant with primary immune deficiency who died at the age of 11 months and 20 days. Microscopic examination revealed focal and diffuse histiocyte proliferation in the bone marrow, lymph nodes, liver, and lung. Histiocytes were found to actively phagocytize erythrocytes and hemosiderin. The changes in the thymus were regarded as congenital primary unclassifiable immunodeficiency. The differential diagnosis of the syndrome was made in comparison with histiocytosis, histiocytosis X and familial erythrophagocytic lymphohistiocytosis.