Successful treatment of agammaglobulinemia by HLA-mismatched unrelated cord blood stem cell transplantation--the first case report / 中华血液学杂志
Chinese Journal of Hematology
; (12): 401-403, 2005.
Article
em Zh
| WPRIM
| ID: wpr-255869
Biblioteca responsável:
WPRO
ABSTRACT
<p><b>OBJECTIVE</b>To evaluate cord blood stem cell transplantation (CBT) in the treatment of X-linked agammaglobulinemia, and observe the courses of the hematopoietic and immune reconstitution.</p><p><b>METHODS</b>A 14-year-old male patient with agammaglobulinemia received CBT from a 1/6 HLA-mismatched unrelated cord blood. The conditioning regimen was Bu/Cy/anti-CD3 antibody. CsA was given together with MMF and MTX for prophylaxis of GVHD. The patient received 0.42 x 10(8) nucleated cells/kg, containing 0.35 x 10(6) CD34(+) cells/kg.</p><p><b>RESULTS</b>The recipient showed hematopoietic reconstitution on day 30 post-transplantation when ANC was 0.5 x 10(9)/L and BPC 20 x 10(9)/L. Sex chromosome analysis showed engraftment (donor 46, XX/recipient 46, XY = 4:1) on day 45. The recipient's blood group changed from AB to O, IgG from 1.1 g/L to 3.5 g/L, sex chromosome from 46, XY to full 46, XX, and mature B cells in peripheral blood from 0 to 5% on day 100, indicating immune reconstitution. At the last follow-up of 360 days, the patient without acute or chronic GVHD showed normal hemogram and myelogram, IgG 13.5 g/L and 10% mature B cells in peripheral blood, indicating the hematopoiesis and immune persistent reconstitution. No acute or chronic GVHD was developed.</p><p><b>CONCLUSION</b>This is the first case report of successful treatment of X-linked agammaglobulinemia by HLA-mismatched unrelated CBT.</p>
Texto completo:
1
Índice:
WPRIM
Assunto principal:
Cirurgia Geral
/
Resultado do Tratamento
/
Condicionamento Pré-Transplante
/
Agamaglobulinemia
/
Transplante de Células-Tronco de Sangue do Cordão Umbilical
/
Alergia e Imunologia
/
Doença Enxerto-Hospedeiro
/
Antígenos HLA
/
Métodos
Limite:
Adolescent
/
Humans
/
Male
Idioma:
Zh
Revista:
Chinese Journal of Hematology
Ano de publicação:
2005
Tipo de documento:
Article