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2005, Número 1

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Gac Med Mex 2005; 141 (1)


Evolución clínica en dos pacientes con Síndrome de Wiskott-Aldrich

Baptista-González, Alvarez-Amaya C

Idioma: Español
Referencias bibliográficas: 27
Paginas: 47-52
Archivo PDF: 57.56 Kb.


FRAGMENTO

El síndrome de Wiskott-Aldrich (WAS) fue descrito original-mente por Wiskott en 1937,1 en dos varones de una misma familia alemana con los datos de púrpura trombo-citopénica crónica, eccema e infecciones recurrentes. En 1954, Aldrich,2 en los EUA reportó una familia con la tríada característica y el rasgo de transmisión ligado al cromosoma X.


REFERENCIAS (EN ESTE ARTÍCULO)

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  2. Aldrich RA, Steinberg AG, Campbell DC. Pedigree demonstrating a sex-linked recessive condition characterized by draining ears, eccematoid dermatitis and bloody diarrhea. Pediatrics 1954;13:133-39.

  3. Perry GS, Spector BD, Schuman LM, Mandel JS, Anderson VE, McHugh RB et al. The Wiskott-Aldrich syndrome in the United States and Canada (1892-1979). J Pediatr 1980;97:72-8.

  4. Conley ME, Saragoussi D, Notarangelo L, Etzioni A, Cazzanova JL, PAGID; ESID. An International study examining therapeutic options used in treatment of Wiskott-Aldrich syndrome. Clin Immunol 2003;109:272-7.

  5. Alvarez AC, Dorantes MS, Toro HA, Bello A, Cuellar J. Forma atenuada del síndrome de Wiskott-Aldrich y la trascendencia de su identificación en el pronóstico de la esplenectomía. Bol Med Hosp Infant Mex 1969;26:633-47.

  6. Dorantes MS. Diagnóstico de los problemas hematológicos en pediatría. Segunda edición. México: Ed Med Hosp Infant Mex 1997.

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  9. García ML, Camacho R, Ortega JA, Berrón PR, Espinoza RF, Hernández BV, Rojas GA. Registro de inmunodeficiencias primarias en pacientes mexicanos en una institución de tercer nivel: experiencia de 30 años. Alerg Asma Inmunol Pediatr 2001;11:48-66.

  10. Notarangelo LD, Ochs HD. Wiskott-Aldrich syndrome: A model for defective actin reorganization, cell trafficking and synapse formation. Curr Opin Immunol 2003;15:585-91.

  11. Dupuis-Girod S, Medioni J, Haddad E, Quartier P, Cavazzana-Calvo M, Le Deist F, et al. Autoimmunity in Wiskott-Aldrich syndrome: risk factors, clinical features, and outcome in a single–center cohort of 55 patients. Pediatrics 2003;111(5 Pt 1):e622-7.

  12. Imai K, Nonmoyama S, Ochs HD. WASP (Wiskott-Aldrich syndrome protein) gene mutations and phenotype. Curr Opin Allergy Clin Immunol 2003;3:427-36.

  13. Zhu Q, Watanabe C, Liu T, Hollenbaugh D, Blaese RM, Kanner SB, et al. Wiskott-Aldrich syndrome/X-linked thrombocytopenia: WASP gene mutations, protein expression, and phenotype. Blood 1997;90:2680-89.

  14. Chinen J, Puck JM. Successes and risks of gene therapy in primary immunodeficiencies. J Allergy Clin Immunol 2004;113:595-604.

  15. Thomson BG, Robertson KA, Gowan D, Heilman D, Broxmeyer HE, Emanuel D, et al. Analysis of engraftment, graft-versus-host disease, and immune recovery following unrelated donor cord blood transplantation. Blood 2002;96:2703-11.

  16. Yamada M, Ariga T, Kawamura N, Yamaguchi K, Ohtsu M, Nelson D, et al. Determination of carrier status for the Wiskott-Aldrich syndrome by flow cytometric analysis of Wiskott-Aldrich syndrome protein expression in peripheral blood mononuclear cells. J Immunol 2000;165:1119-22.

  17. Imai K, Morio T, Zhu Y, Itoh S, Kajiwara M, Yata J et al. Clinical course of patients with WASP gene mutations. Blood 2004;103:456-64.

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  20. Akman IO, Ostrov BE, Neudorf S. Autoimmune manifestations of the Wiskott-Aldrich syndrome. Sem Arthritis Rheum 1998;27:218-25.

  21. Bradley M, Soderhall C, Wahlgren CF, Luthman H, Nordenskjold M, Kockum I. The Wiskott-Aldrich syndrome gene as a candidate gene for atopic dermatitis. Acta Derm Venereol 2001;81:340-2.

  22. Litzman J, Jones A, Hann I, Chapel H, Strobel S, Morgan G. Intravenous immunoglobulin, splenectomy, and antibiotic prophylaxis in Wiskott-Aldrich syndrome. Arch Dis Child 1996;75:436-9.

  23. Mullen CA, Anderson KD, Blaese RM. Splenectomy and/or bone marrow transplantation in the management of the Wiskott-Aldrich syndrome: long-term follow-up of 62 cases. Blood. 1993;82:2961-6.

  24. Lum LG, Tubergen DG, Corash L, Blaese RM. Splenectomy in the management of the thrombocytopenia of the Wiskott-Aldrich syndrome. N Engl J Med. 1980;302:892-6.

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  27. Strom TS, Turner SJ, Andreansky S, Liu H, Doherty PC, Srivastava DK, et al. Defects in T-cell-mediated immunity to influenza virus in murine Wiskott-Aldrich syndrome are corrected by oncoretroviral vector-mediated gene transfer into repopulating hematopoietic cells. Blood. 2003;102:3108-16.




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