Preconstitución inmunológica mediante trasplante medular en un paciente portador de inmunodeficiencia combinada severa
| Autor: | Benito González M, Esperanza Marzouka B, Soledad Godoy P, Patricia Dal Borgo A |
|---|---|
| Jazyk: | Spanish; Castilian |
| Rok vydání: | 1993 |
| Předmět: |
medicine.medical_specialty
Severe combined immunodeficiency business.industry Isoniazid medicine.disease Rash Surgery inmunodeficiencia combinada severa medicine.anatomical_structure Immune system Graft-versus-host disease Pediatrics Perinatology and Child Health medicine enfermedad de Kawasaki Kawasaki disease Bone marrow medicine.symptom Trasplante medular business Ethambutol medicine.drug |
| Zdroj: | Revista chilena de pediatría v.64 n.2 1993 SciELO Chile CONICYT Chile instacron:CONICYT |
| Popis: | 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 within3 months with full reconstilulion of T-and B-lympnocyfc-mcdiated responses. The donor was Iransfused with irradiated selffresh whole blood, she showed good tolerance for the procedure, without complications and was discharged 24 hourslater without changes in her hematological parameteis. The receptor showed clinical and bacteriological evidences ofdisseminated infection by bacillus of CalmeUe-Guerin before and after bone marrow transplantation, and was treatedwith rifanpicine, isoniazid, ethambutol. The clinical pre.sentclion of BCG infection was like that seen in similar cases andmimiked some aspects of local reactions at BCG vaccination site that have been described in patients with Kawasakidisease. No conditioning treatment was given to ihe patient and a mild graft versus host disease was seen one monthafter bone marrow infusion, consisting of rash, hepatomegaly, thrombocytopenia and increased scrum activity of liverenzymes. He was successfully treated with hydrocortisone and prcdnisonc for three months. One year after bone marrowtrnsplantation the patient remains clinically well and free of any significant infection.(Key words: severe combined immunodeficiency, disseminated Bacillus Calmcttc-Guerin, bone marrow transplantation,Kawasaki disease.! |
| Databáze: | OpenAIRE |
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