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Case Reports
. 2021;81(6):1060-1064.

[Evans syndrome and rheumatoid arthritis as autoimmune manifestations of large granular T-cell leukemia]

[Article in Spanish]
Agustín M Muñoz  1 , José A Orozco Niño  2 , Cristian M Seehaus  2 , Alberto D Giménez Conca  2 , Fernando A Chuliber  2 , Luciano F Lo Giudice  2 , Georgina E Bendek  2
Affiliations
  • PMID: 34875608
Free article
Case Reports

[Evans syndrome and rheumatoid arthritis as autoimmune manifestations of large granular T-cell leukemia]

[Article in Spanish]
Agustín M Muñoz et al. Medicina (B Aires). 2021.
Free article

Abstract
in English, Spanish

Large granular T-cell leukemia is a rare cytotoxic lymphocyte disorder. These cells play an integral role in the immune system and are divided into 2 lineages: CD3 T positive and natural killer. Its proliferation and uncontrolled cytotoxicity can generate autoimmunity or malignancy. Rheumatoid arthritis is the most common autoimmune disease in individuals with this type of leukemia, however, it has been associated with a wide spectrum of other autoimmune diseases and hematological conditions including hemolytic anemia, pure red blood cell aplasia, and neutropenia, leading to recurring bacterial infections. The following is a case of a 72-year-old female with a history of large granular T-cell leukemia and manifestations compatible with rheumatoid arthritis, which occurs with a severe Evans syndrome with a good initial and sustained response to gamma globulin, corticosteroid therapy, and rituximab.

La leucemia de células T grandes granulares es un trastorno poco frecuente de linfocitos citotóxicos. Estas células juegan un rol integral en el sistema inmunológico y se dividen en 2 linajes: T CD3 positivas y natural killer. Su proliferación y citotoxicidad descontrolada puede generar autoinmunidad o malignidad. La artritis reumatoide es la enfermedad autoinmune más común en individuos con este tipo de leucemia, sin embargo, se ha asociado a un amplio espectro de otras enfermedades autoinmunes y afecciones hematológicas incluyendo anemia hemolítica, aplasia pura de glóbulos rojos y neutropenia, que conducen a infecciones bacterianas recurrentes. Se presenta a continuación una paciente de 72 años con antecedentes de leucemia de células T grandes granulares y manifestaciones compatibles con artritis reumatoidea, que intercurre con un Síndrome de Evans grave con buena respuesta inicial y sostenida a gammaglobulina, corticoterapia, y rituximab.

Keywords: Evans syndrome; large granular T-cell leukemia; rheumatoid arthritis.

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