Characterization of Neutropenic Pediatric Patients sent to a referral centre
Versión traducida del artículo "Caracterización de pacientes pediátricos con neutropenia enviados a un hospital de referencia" DOI: https://doi.org/10.51481/amc.v52i2.643
DOI:
https://doi.org/10.51481/amc.v52i2.720Palabras clave:
neutropenia, granulocyte colony stimulation factor, recurrent infection, immunodeficiency, childrenResumen
Aim: Neutropenia is a relatively common cause of patient referral to the Immunology and Pediatric Rheumatology Department of the National Children’s Hospital. The present study characterizes the cases of neutropenia referred to this department between November 1988 and June 2008.
Methods: Eighty four patients between 0 and 12 years of age, were referred from November 6th, 1988 and June 1st, 2008. We performed a comprehensive descriptive analysis of the characteristics exhibited by these patients in terms of clinical course, pattern of infection, most common causative germs, complications and treatment applied.
Results: Neutropenia resolved spontaneously in 52.2% of the patients, and they were classified as transient neutropenia, 21.7% of the cases developed cyclic neutropenia, 13% of were categorized as benign chronic neutropenia, 7.2% developed severe chronic symptomatic neutropenia, 2.9% had neutropenia associated with type 1B glycogenosis and 2.9% of the cases were not classifiable in any of the proposed categories. More than 50% of the cases were associated with an abnormal pattern of infection in terms of frequency, severity, multiplicity of systems involved, or the presence of opportunistic microorganisms. The upper respiratory tract was the most commonly affected system with infection. Prophylactic antibiotics were used in 39.1% of the cases and granulocyte colony stimulating factor was required in 11.6% at some stage of the course. The most frequently involved pathogens in infection were Pseudomonas aeruginosa, Staphylococcus sp and E. coli.
Conclusion: The vast majority of neutropenic patients had a benign clinical course. The same germs described in other series, caused infections in our patients. The absolute neutrophil count at diagnosis has no effect on the incidence of recurrent infection, but the type or clinical course of neutropenia does.
Descargas
Citas
Lakshman R, Finn A. Neutrophil disorders and their management. J Clin Pathol. 2001; 54:7-19.
Bernini JC. Diagnosis and management of chronic neutropenia during childhood. Pediatr Clin North Am. 1996; 43: 773-792.
Berliner N, Horwitz M, Loughran T. Congenital and adquired neutropenia. Hematology. 2004: 63-79.
Boxer LA, Blackwood RA. Leukocyte disorders: Quantitative and qualitative disorders of the neutrophil, Part 2. Pediatr Rev. 1996; 17: 47-51.
James RM, Kinsey SE. The investigation and management of chronic neutropenia in children. Arch Dis Child. 2006; 91: 852-858.
Capsoni F, Sarzi-Puttini P, Zanella A. Primary and secondary autoimmune neutropenia. Arthritis Res Ther. 2005; 7: 208-214.
Horwitz M, Duan Z, Korkmaz B, Lee HH, Meallife ME, Salipante SJ.
Neutrophil elastase in cyclic and severe congenital neutropenia. Blood. 2007; 109:1817-1824.
Slatter MA, Gennery AR. Clinical Immunology Review Series: An approach to the patient with recurrent infections in childhood. Clin Exp Immunol. 2008; 152: 389-396.
Rosensweig S, Holland S. Phagocyte immunodeficiencies and their infections. J Allergy Clin Immunol. 2004; 113: 620-626.
Roskos RR, Boxer LA. Clinical disorders of neutropenia. Pediatr Rev. 1991;12: 208-213.
Dale D, Person RE, Bolyard AA, Aprikyan AG, Bos C, Bonilla MA et al. Mutations in the gene encoding neurophil elastase in congenital and cyclic neutropenia. Blood. 2000; 93: 2317-2322.
Online Mendelian Inheritance in Man. Elastase 2. Consultado el 26 de agosto de 2008 En: http://www.ncbi.nlm.nih.gov/entrez/dispomim.
cgi?id=130130
Ancliff PJ. Congenital neutropenia. Blood Rev 2003; 17: 209-216.
Klein C, Grudzien M, Appaswamy G, Germeshausen M, Sandrock I, Schäffer AA et al. HAX1 deficiency causes autosomal recessive severe congenital neutropenia (Kostmann disease). Nat Genet. 2007; 39: 8692.
Boxer LA. Neutrophil abnormalities. Pediatr Rev. 2008; 24: 52-61.
Zaromb A, Chamberlain D, Schoor R, Almas K, Blei F. Periodontitis as a manifestation of chronic benign neutropenia. J Periodontol. 2006; 77:1921-1926.
Kuritzkes DR. Neutropenia, neutrophil dysfunction, and bacterial infection in patients with human immunodeficiency virus disease: the role of granulocyte colony-stimulating factor. Clin Infect Dis. 2000; 30: 256-260.
Newburger PE. Disorders of neutrophil number and function. Hematology. 2006; 7: 104-110.
Segel GB, Halterman JS. Neutropenia in pediatric practice. Pediatr Rev. 2008; 29: 12-25.
Boxer LA, Blackwood RA. Leukocyte disorders: Quantitative and qualitative disorders of the neutrophil, Part 1. Pediatr Rev 1996; 17: 19-29.
Lehrnbecher T, Welte K. Haematopoietic growth factors in children with neutropenia. Br J Haematol. 2002; 116: 28-56.
Lima C, Paula E, Takahashi T, Saad ST, Lorand-Metze S, Costa FFl. Causes of incidental neutropenia in adulthood. Ann Hematol. 2006; 85: 705-709.
Palmblad J, Papadaki HA. Chronic idiopathic neutropenias and severe congenital neutropenia. Curr Opin Hematol. 2008; 15: 8-14.
Papadaki H, Eliopoulos A, Kosteas T, Gemetzic C, Damianaki A, Kontala H et al. Impaired granulocytopoiesis in patients with chronic idiopathic neutropenia is associated with increased apoptosis of bone marrow progenitor cells. Blood. 2003; 101: 2591-2600.
Descargas
Publicado
Cómo citar
Número
Sección
Licencia
Derechos de autor 2010 Acta Médica Costarricense
Esta obra está bajo una licencia internacional Creative Commons Atribución-NoComercial-SinDerivadas 4.0.
Los autores que publican en la revista Acta Médica Costarricense pueden distribuir, copiar, remezclar, retocar, leer, descargar, imprimir, buscar y crear a partir de su obra de modo no comercial, indicando los créditos a la revista y sus autores y compartir su obra en las mismas condiciones. Para ello se aplica la licencia Creative Commons Reconocimiento-NoComercial-CompartirIgual 4.0 Internacional(CC BY-NC-SA 4.0)