[Evans syndrome and rheumatoid arthritis as autoimmune manifestations of large granular T-cell leukemia]. / Síndrome de Evans y artritis reumatoidea como manifestaciones autoinmunes de la leucemia de células T grandes granulares.

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.

Síndrome de Evans y artritis reumatoidea como manifestaciones autoinmunes de la leucemia de células T grandes granulares / Evans syndrome and rheumatoid arthritis as autoimmune manifestations of large granular T-cell leukemia

Resumen 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.

Abstract 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 rheuma toid arthritis, which occurs with a severe Evans syndrome with a good initial and sustained response to gamma globulin, corticosteroid therapy, and rituximab.

Fragility fractures in psoriatic arthritis patients: a matched retrospective cohort study.

OBJECTIVE: To compare incidental fragility fractures in psoriatic arthritis (PsA) patients with matched controls from a university hospital. METHODS: Consecutive PsA patients were matched (age and sex) with controls (1:2). Follow-up began at index date, defined as the date of PsA diagnosis for cases and their respective controls, until the last hospital visit, death or the end of the study (31 December 2017). Electronic medical records were reviewed for osteoporotic fractures. Incidence rates per 100,000 persons-years (PY) of distinct types of fractures after index dates were calculated and compared between groups. A multivariate Cox regression analysis was performed to investigate determinants of fractures. RESULTS: Ninety-two PsA patients and 184 controls were included. No difference was found in the overall fracture incidence rate per 100,000 PY between PsA and controls (1020 95% CI 510-1930, vs 870 95% CI 520-1390, p = 0.36). Vertebral fractures were numerically more frequent in PsA patients with an incidence rate of 1020 (95% CI 510-1930) per 100,000 PY versus 460 (95% CI 240-920), per 100,000 PY in the control group but it did not reach statistical significance (p = 0.06). In the Cox regression analysis, after adjusting for bisphosphonate use, only age (HR 1.10, 1.05-1.16, p < 0.001) and female sex (HR 3.94, 1.11-13.91, p = 0.03) were associated with fractures while PsA diagnosis and use of glucocorticoids were not. CONCLUSION: In this cohort of PsA patients, no overall increased risk of fractures was found in comparison with matched controls. Key Points • PsA could have different effects on bone, leading to confusing results in bone densitometry readings contributing to the difficulty in establishing the real prevalence of OP in PsA. • Vertebral fractures were more frequent in PsA patients compared to controls, but it did not reach statistical significance. No difference was found in the overall fracture incidence rate.