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Familial Autohemolytic Anemia and Runting Syndrome with Rh o ‐specific Autoantibody
Author(s) -
Shapiro Maurice
Publication year - 1967
Publication title -
transfusion
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.045
H-Index - 132
eISSN - 1537-2995
pISSN - 0041-1132
DOI - 10.1111/j.1537-2995.1967.tb05519.x
Subject(s) - hypergammaglobulinemia , autoantibody , clone (java method) , anemia , immunology , autopsy , medicine , lymphatic system , biology , antibody , pathology , genetics , gene
A case of autohemolytic anemia with hypergammaglobulinemia and specific anti‐Rh o autoantibody is reported. The patient had the clinical syndrome of simulated runt disease as it occurs in experimental animals. Thymic atrophy and generalized lymphocytic depletion were found at autopsy. Four siblings of the proposita died in infancy of similar affections. The sera of the two living siblings and the parents disclose abnormal serum globulin patterns. It is suggested that dysglobulinemia favors the survival of lymphoid cells acquired trans‐placentally from the mother and that the autohemolytic anemia and other pathologic manifestations in this case are the result of a graft‐versus‐host reaction. The anti‐Rh o specificity of the autoantibody is ascribed to isosensitization of a clone of grafted lymphoid cells which have sustained a loss of Rh genes in the process of somatic mitosis.