RESUMEN
BACKGROUND: Richter's syndrome (RS) is a rare complication with an unfavorable prognosis, in which chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL) transform into a more aggressive type of lymphoma, most commonly into diffuse large B cell lymphoma (DLBCL) or less often into Hodgkin's lymphoma (HL). OBJECTIVES: The objective of this research paper was to present a retrospective analysis of patients with CLL/SLL whose disease transformed into RS. MATERIAL AND METHODS: The study included 217 patients (100 women and 107 men) with CLL/SLL diagnosed in the years 2006-2015 at the Department of Hematooncology and Bone Marrow Transplantation of the Medical University of Lublin, which transformed into RS. We analyzed clinical, laboratory, immunophenotypic (ZAP-70 and CD38 expression), histopathological, and genetic data (del(17p), del(11q)), which was collected at the time of CLL/SLL diagnosis, and some which was collected at the time of transformation. RESULTS: Richter's syndrome was diagnosed in 4.6% of all CLL and SLL patients. The group of patients with RS consisted of 9 patients with primary CLL and 1 patient with a diagnosis of SLL (8 patients with transformation into DLBCL and 2 patients with transformation into HL). Leukemic lymphocytes showed evidence of peripheral blood lymphocyte membrane expression of ZAP70+/CD38+ (1 patient), of ZAP-70+/CD38- (3 patients), of ZAP-70-/CD38- (1 patient), and of ZAP-70-/CD38+ (5 patients). The deletion of 11q (del(11q)) was documented in 2 patients. In 4 cases, the location of RS was extremely rare (the thyroid gland, liver, skin, bladder, and central nervous system). CONCLUSIONS: Richter's syndrome is a rare, but probable complication of CLL/SLL with an unfavorable prognosis, and it should be taken into account at every stage of the disease, particularly when the course of the disease is aggressive.
Asunto(s)
Enfermedad de Hodgkin/diagnóstico , Enfermedad de Hodgkin/genética , Leucemia Linfocítica Crónica de Células B/diagnóstico , Leucemia Linfocítica Crónica de Células B/genética , Linfoma de Células B Grandes Difuso/diagnóstico , Linfoma de Células B Grandes Difuso/genética , Proteína Tirosina Quinasa ZAP-70/genética , Adulto , Transformación Celular Neoplásica , Niño , Progresión de la Enfermedad , Femenino , Enfermedad de Hodgkin/patología , Humanos , Leucemia Linfocítica Crónica de Células B/patología , Linfoma de Células B Grandes Difuso/patología , Masculino , Pronóstico , Estudios RetrospectivosRESUMEN
Chronic nutrition deficiencies, economic or cultural in their nature are one of the most often reason ofanaemia. From the other hand various diet restrictions may lead to different nutrition deficiencies. The occurrence of anaemia caused by diet restrictions is relatively rare among Catholics in Poland. We present a case of 51 years old woman with severe anaemia (Hgb 2.48 mmol/l), occasionally diagnosed. Anaemia occurred 20 years after she took a pledge never to eat meat. The aim of her oath was the begging for grace and quitting alcoholism by her husband, a habitual alcoholic. The iron and vitamin B12 supplementation led to quick improvement of hematological parameters.