medigraphic.com
ISSN 1561-2996 (Electronic)
ISSN 0864-0289 (Print)

2021, Number 4

<< Back Next >>

Rev Cubana Hematol Inmunol Hemoter 2021; 37 (4)

Burkitt's lymphoma as a complication of Bruton's Agammaglobulinemia

de la Guardia POM, Machín GS, Arce HA, Casado HI

Language: Spanish
References: 18
Page: 1-12
PDF size: 299.64 Kb.


ABSTRACT

Introduction: Bruton's Agammaglobulinemia is a primary immunodeficiency (PID) caused by a mutation in the gene that encodes Bruton's tyrosine kinase (BTK). It is suspected mainly in men with frequent respiratory tract infections and has, among other complications, tumors, mainly lymphoproliferative. Autosomal recessive agammaglobulinemias with similar clinical characteristics have been reported in both sexes.
Objective: To present the first pediatric case reported in Cuba, with a diagnosis of Burkitt's lymphoma associated with PID and that also used combined immunoglobulin replacement and antitumor therapy.
Case report: 2-year-old male diagnosed with Bruton's disease. With the replacement treatment with intravenous immunoglobulin (Intacglobin), he maintained three years without serious infections. At 5 years of age, he presented Burkitt's lymphoma, treated with polychemotherapy according to the AEIOP scheme, associating Rituximab. Although do not have molecular biology detection of the BTK gene mutation, the decrease of CD19 + B cells to below 2% and the absent values of IgG, IgA and IgM allowed the diagnosis.
Conclusion: Antitumor treatment and intravenous immunoglobulin replacement therapy coexisted with satisfactory clinical results. The patient remains in good general condition.


REFERENCES

  1. Urriza Ripa I, Villareal Calvo M, Álvarez García J, Duran Urdaniz G, Ramos Arroyo MA, Herranz Aguirre M. Enfermedad de Bruton de diagnóstico precoz: importancia de una adecuada historia clínica y los antecedentes familiares. Rev Pediatr Aten Primaria [Internet]. 2016 Sep [acceso 09/06/2016];18(71):e111-14. Disponible en: http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1139-76322016000300013&lng=es

  2. Seoane ME, Muñoz-Fernández MA. Agammaglobulinemia ligada al cromosoma X. Acta Pediatr Esp. 2006;64(11):531-5.

  3. Matos-Benavides E, García-Gomero D, Inocente-Malpartida R, Córdova-Calderón W, Aldave-Becerra J. Diagnóstico genético de pacientes conagammaglobulinemia primaria atendidos en centros peruanos de tercer nivel. Rev Peru Med Exp Salud Publica. 2019;36(4):664-9. DOI: https://10.17843/rpmesp.2019.364.4311

  4. Bousfiha A, Jeddane L, Al-Herz W, Ailal F, Casanova J, Chatila T, et al. The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies. J Clin Immunol. 2015;35(8):727-38. DOI: https://doi.org/10.1007/s10875-015-0198-5

  5. Notarangelo LD (International Union of Immunological SocietisExpers Committee of Primary Immunodeficiency). Primary Immunodeficiency disease: 2009 update. J Allergy ClinImmunol 2009:124:1161-78.

  6. Kuijpers TW, Eldering E, van Lie RAW. CD20 deficiency in humans results in impaired T cell-independent antibody responses.J Clin Invest. 2010;120(1):214-22. DOI: https://doi.org/10.1172/JCI40231

  7. Rezaei N, Hedayat M, Aghamohammadi A, Nichols KE. Primary immunodeficiency diseases associated with increased susceptibility to viral infections and malignancies. J Allergy Clin Immunol. 2011;127:1329-41 DOI: http://dx.doi.org/10.1016/j.jaci.2011.02.047

  8. Shiramizu B, Goldman S, Smith L, et al.: Impact of persistent minimal residual disease post-consolidation therapy in children and adolescents with advanced Burkittleukaemia: A Children's Oncology Group Pilot Study Report. Br J Haematol. 2015;170(3):367-71.

  9. Quintero Sierra Y, Hernández Padrón C. Linfoma no hodgkiniano parecido al Burkitt: presentación de un caso. Rev Cubana Hematol Inmunol Hemoter [Internet]. 2016 [acceso 14/04/2020];32(2):[aprox. 0 p.]. Disponible en: http://www.revhematologia.sld.cu/index.php/hih/article/view/405

  10. Sánchez Segura M, Marsán Suárez V, Macías Abraham C, Pino Blanco D, Socarrás Ferrer BB, de Valle Pérez LO, Arce Hernández AA, et al. Agammaglobulinemia ligada al X o de Bruton. Rev Cubana Hematol Inmunol Hemoter [Internet]. 2014 [acceso 08/01/2020];30(4):[aprox. 0 p.]. Disponible en: http://www.revhematologia.sld.cu/index.php/hih/article/view/174

  11. Vaca CL, Hidalgo JL. Agammaglobulinemia ligada al cromosomaX, lo crucial del diagnóstico y tratamiento oportunos. Cambios rev. méd. 2019;18(1):90-5.

  12. Merchant AH, Parekh D, Ahmad N, Madkaikar M, Ahmed J. X-linked Agammaglobulinemia: A single centre experience from India Indian J Pediatr. 2014;81:92-4. DOI: http://dx.doi.org/10.1007/s12098-013-1113-9

  13. Chun JK, Lee TJ, Song JW, Linton JA, Kim DS. Analysis of clinical presentations of Bruton disease: a review of 20 years of accumulated data from pediatric patients at Severance Hospital. Yonsei Med J. 2008 Feb 29;49(1):28-36. DOI: https://10.3349/ymj.2008.49.1.28

  14. Fernández A, Guedes M, Vasconcelos J, Neves E, Fernández S, Marques L. Agammaglobulinemia ligada al cromosoma X: experiencia en un hospital portugués. An Pediatr (Barc) 2015;82:166-71

  15. Percy CL, Smith MA, Linet M, Gloeckler Ries LA, Friedman DL. Lymphomas and reticuloendothelial neoplasms. In: Ries LAG, Smith MA, Gurney JG, Linet M, Tamra T, Young JL, et al (eds). Cancer Incidence and Survival among Children and Adolescents: United States SEER Program 1975-1995, National Cancer Institute, SEER Program. NIH Pub. No. 99-4649. Bethesda, MD, 1999.

  16. Vela E, Cruz O. Linfomas. En: Cruz M. Tratado de Pediatría.11naEdición t-2, Parte XX. España: Médica Panamericana; 2013. p. 372.

  17. Yanik EL, Shiels MS, Smith JM, et al.: Contribution of solid organ transplant recipients to the pediatric non-hodgkin lymphoma burden in the United States. Cancer. 2017;123(23):4663-71.

  18. Landmann E, Oschlies I, Zimmermann M, Moser O, Graf N, Suttorp M, et al. Secondary non-Hodgkin lymphoma (NHL) in children and adolescents after childhood cancer other than NHL. Br J Haematol. 2008 Nov;143(3):387-94. DOI: https://10.1111/j.1365-2141.2008.07356.xx




2020     |     www.medigraphic.com