Inborn errors of immunity in pediatric oncohematological patients from Pinar del Río

Authors

  • Carlos Alfredo Miló Valdés Universidad de Ciencias Médicas de Pinar del Río. Hospital Pediátrico Provincial Docente “Pepe Portilla”. Pinar del Río, Cuba https://orcid.org/0000-0003-1527-4541
  • Odalys Orraca-Castillo Universidad de Ciencias Médicas de Pinar del Río. Facultad de Ciencias Médicas “Dr. Ernesto Che Guevara de la Serna”. Pinar del Río, Cuba. https://orcid.org/0000-0002-1742-610X
  • Madelayne Valdés-Acosta Universidad de Ciencias Médicas de Pinar del Río. Hospital Pediátrico Provincial Docente “Pepe Portilla”. Pinar del Río, Cuba https://orcid.org/0000-0002-1849-9554
  • Daniel Barreras-Sixto Universidad de Ciencias Médicas de Pinar del Río. Hospital Pediátrico Provincial Docente “Pepe Portilla”. Pinar del Río, Cuba https://orcid.org/0000-0003-3464-0500
  • Cesar Valdés-Soho Universidad de Ciencias Médicas de Pinar del Río. Hospital Pediátrico Provincial Docente “Pepe Portilla”. Pinar del Río, Cuba https://orcid.org/0000-0001-7000-2288
  • Jorge Luis Hernández-González Universidad de Ciencias Médicas de Pinar del Río. Hospital Pediátrico Provincial Docente “Pepe Portilla”. Pinar del Río, Cuba https://orcid.org/0000-0003-4195-9739

Keywords:

DIAGNOSIS, HEMATOLOGY, ONCOLOGY.

Abstract

Introduction: increased susceptibility to infectious processes is considered the main sign suggestive of immunodeficiency; however, other manifestations such as those of hematological and/or oncological nature may be part of the evolution and even the onset of an inborn error of immunity.

Objective: to identify patients with probable diagnosis of inborn errors of immunity in the pediatric oncohematology service at Pepe Portilla Pediatric Provincial Teaching Hospital in Pinar del Rio during the period from January 1979 to March 2021.

Methods: an observational, descriptive, cross-sectional study was carried out with a sample of 103 patients. The following variables were studied: diagnosed oncohematological disease, probable diagnosis of inborn error of immunity and alarm signs of primary immunodeficiency.

Results: the diagnosis of some oncological disease reached 87,4 %. Five patients were found with a probable diagnosis of an inborn error of immunity (Fanconi's anemia and Medullary Aplasia); 54,5 % of non-leukemic patients and only 24,1 % of leukemic patients had at least one and up to three alarm signs for immunodeficiency.

Conclusions: the Jeffrey Modell Foundation alarm signs for the diagnosis of primary immunodeficiencies were not useful in patients with hematopathies; therefore other criteria for the identification of these disorders could be implemented. These patients should be re-evaluated by the provincial immunology group with a view to the diagnosis of inborn errors of immunity and their comprehensive treatment.

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Published

2022-04-10

How to Cite

1.
Miló Valdés CA, Orraca-Castillo O, Valdés-Acosta M, Barreras-Sixto D, Valdés-Soho C, Hernández-González JL. Inborn errors of immunity in pediatric oncohematological patients from Pinar del Río. Rev Ciencias Médicas [Internet]. 2022 Apr. 10 [cited 2025 Aug. 26];26(2):e5466. Available from: https://revcmpinar.sld.cu/index.php/publicaciones/article/view/5466

Issue

Vol. 26 No. 2 (2022)

Section

ORIGINAL ARTICLES

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