Preconstitución inmunológica mediante trasplante medular en un paciente portador de inmunodeficiencia combinada severa

Abstract

A 6 month old boy with severe combined immunodeficiency disease was treated successfully with a bone marrow 'ranspiantaiion (BMT) from his HLA-icntical dizyqotic female twin. Development of immune functions was achieved within 3 months with full reconstilulion of T-and B-lympnocyfc-mcdiated responses. The donor was Iransfused with irradiated self fresh whole blood, she showed good tolerance for the procedure, without complications and was discharged 24 hours later without changes in her hematological parameteis. The receptor showed clinical and bacteriological evidences of disseminated infection by bacillus of CalmeUe-Guerin before and after bone marrow transplantation, and was treated with rifanpicine, isoniazid, ethambutol. The clinical pre.sentclion of BCG infection was like that seen in similar cases and mimiked some aspects of local reactions at BCG vaccination site that have been described in patients with Kawasaki disease. No conditioning treatment was given to ihe patient and a mild graft versus host disease was seen one month after bone marrow infusion, consisting of rash, hepatomegaly, thrombocytopenia and increased scrum activity of liver enzymes. He was successfully treated with hydrocortisone and prcdnisonc for three months. One year after bone marrow trnsplantation the patient remains clinically well and free of any significant infection.