Aplastic Anemia, Histiocytosis and Erythrodermia in Immunologically Deficient Children

IMMUNOLOGICALLY tolerant animals that accept a graft of foreign hematopoietic tissue frequently manifest a syndrome characterized by generalized wasting, dermatitis, diarrhea, anemia, aplasia of the bone marrow, lymphoid tissue depletion, histiocytosis and death. This disorder has been called graft-versus-host (GVH) reaction,1 secondary syndrome2 or runt disease.3 To our knowledge the human counterpart of this syndrome has not previously been reported.The purpose of this paper is to describe 2 children with severe progressive vaccinia necrosum who were treated with multiple fresh transfusions containing leukocytes and in whom erythrodermia, hepatomegaly and fatal aplastic anemia subsequently developed. The pathological findings in these . . .