THYMIC ALYMPHOPLASIA AND CONGENITAL ALEUKOCYTOSIS

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Abstract
Thymic alymphoplasia has been found to be a part of the histopathological picture of congenital aleukocytosis. As in children with congenital agammaglobulinemia who have thymic alymphoplasia, the thymic anomaly is accompanied by lymphopenia and a marked generalized paucity of lymphocytes in the tissues or lymphocytic hypoplasia. The patient described in this report, a girl who succumbed at 15 days to overwhelming bacterial infection, also had agranulocytosis and no granulocytes or granulocyte precursors could be discerned in her bone marrow; megakaryocytes were present in the marrow in normal numbers and hematocytoblasts, normoblasts, and monocytes were present as well. Hence, the defect in this syndrome, whether intracellular or conveyed humorally, would appear to be highly selective. On the basis of the normal development of the human thymus and other lymphoid tissues including the bone marrow, it would seem that the defect in the development of the lymphocyte was manifest at or before the 10th week of gestation. The possible relations between lymphocytes, plasma cells, and granulocytes are discussed.

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