1064 Severe combined immunodeficiency: Case report of alogenic, haploidentical bone marrow transplantation

Sunday, 7 December 2014
Exhibition Hall-Poster Area (Sul America)

Fernanda Silveira , Allergy and Immunology, Instituto Nacional De Saude Da Mulher, Da Criança e Do Adolescente Fernandes Figueira - IFF/ Fiocruz, Rio de Janeiro, Brazil

Flavia Anisio , Allergy and Immunology, Instituto Nacional De Saude Da Mulher, Da Criança e Do Adolescente Fernandes Figueira - IFF/ Fiocruz, Rio de Janeiro, Brazil

Liziane Nunes , Allergy and Immunology, Instituto Nacional De Saude Da Mulher, Da Criança e Do Adolescente Fernandes Figueira - IFF/ Fiocruz, Rio de Janeiro, Brazil

Paula Lauria , Allergy and Immunology, Instituto Nacional De Saude Da Mulher, Da Criança e Do Adolescente Fernandes Figueira - IFF/ Fiocruz, Rio de Janeiro, Brazil

Abelardo Neto , Allergy and Immunology, Instituto Nacional De Saude Da Mulher, Da Criança e Do Adolescente Fernandes Figueira - IFF/ Fiocruz, Rio de Janeiro, Brazil

Patricia Lima , Allergy and Immunology, Instituto Nacional De Saude Da Mulher, Da Criança e Do Adolescente Fernandes Figueira - IFF/ Fiocruz, Rio de Janeiro, Brazil

Franciane Silva , Allergy and Immunology, Instituto Nacional De Saude Da Mulher, Da Criança e Do Adolescente Fernandes Figueira - IFF/ Fiocruz, Rio de Janeiro, Brazil

Juliana Fernandes , Hospital Israelita Albert Einstein, São Paulo, Brazil

Celso Ungier , Allergy and Immunology, Instituto Nacional De Saude Da Mulher, Da Criança e Do Adolescente Fernandes Figueira - IFF/ Fiocruz, Rio de Janeiro, Brazil

Sandra Bastos , Allergy and Immunology, Instituto Nacional De Saude Da Mulher, Da Criança e Do Adolescente Fernandes Figueira - IFF/ Fiocruz, Rio de Janeiro, Brazil

Background: Severe combined immunodeficiency (SCID) is characterized by important impairment in differentiation of T and/or B lymphocytes and occasionally Natural Killer cells, representing a pediatric emergency. A case of immunodeficiency is described emphasizing symptoms, diagnosis and answer after bone marrow transplantation.

Methods: Case report of a 2 years old male patient with severe combined immunodeficiency (SCID), diagnosed at 9 months after hospitalization due to failure to thrive, chronic diarrhea and pneumonia. Evolved with recurrent respiratory and gastrointestinal infections although using prophylaxis and immunoglobulin infusion. Alogenic, haploidentical transplantation was carried out with positive selection of CD34+, at 18 months of age due no compatible donor been found. 

Results: Satisfactory answer after transplantation keeping infusion of IV immunoglobulin with clinical and laboratorial favorable evolution.

Conclusions: Bone marrow transplantation when succeeds is supposed to restore lymphocyte system diminishing risks of severe and fatal infections.