RESUMO
Among patients with systemic lupus erythematosus (SLE) there is an increased risk of haematological malignancies, especially non-Hodgkin lymphoma. However, the association of SLE with aggressive CD3 negative natural killer (NK)-cell leukaemia has not been reported so far. We present a case of a 39-year-old woman with SLE, aggressive NK-cell leukaemia and tuberous sclerosis complex. The prior probability of developing the combination of these three rare diseases by coincidence is extremely low (<10(-13)). Possible underlying immunological, genetic and toxic/environmental pathways are discussed.
Assuntos
Leucemia Linfocítica Granular Grande/diagnóstico , Lúpus Eritematoso Sistêmico/complicações , Esclerose Tuberosa/diagnóstico , Adulto , Medula Óssea/patologia , Feminino , Humanos , Rim/patologia , Leucemia Linfocítica Granular Grande/complicações , Lúpus Eritematoso Discoide/complicações , Imageamento por Ressonância Magnética , Esclerose Tuberosa/complicaçõesAssuntos
Síndrome Antifosfolipídica/sangue , Inibidor de Coagulação do Lúpus/sangue , Reação de Fase Aguda/sangue , Adulto , Idoso , Idoso de 80 Anos ou mais , Anticorpos Antifosfolipídeos/análise , Síndrome Antifosfolipídica/diagnóstico , Feminino , Humanos , Ensaio de Proficiência Laboratorial , Masculino , Programas de Rastreamento/métodos , Pessoa de Meia-Idade , Padrões de Prática Médica , Trombose/sangueRESUMO
Glomerular function of all long-term survivors who underwent hemopoietic stem cell transplantation (HSCT) from 1991 to 1998 (study I, n=121) was studied retrospectively. In addition, we prospectively analyzed glomerular and tubular function of all long-term surviving children who received an HSCT between 1998 and 2000 (study II, n=41). We found a lower prevalence of children with chronic renal failure (CRF) post-HSCT in our more recent cohort (study II: 10%) as compared to the older cohort (study I: 24%) 5.0 (0.7 s.d.) and 7.6 (2.4 s.d.) year's post-HSCT, respectively. Furthermore, it seems that renal function may stabilize after 1-year post-HSCT. None of the patients required dialysis or antihypertensive medication at long-term follow-up. The sole predictor of CRF in our study was high serum creatinine pre-HSCT (P=0.007), while acute renal failure within 3 months after HSCT (P=0.08) only showed a trend towards predicting CRF. We could not confirm a relation of conditioning with irradiation with CRF post-HSCT, as was shown in several other pediatric and adult studies. Proximal and distal tubular dysfunction only occurred in a minority of long-time survivors of HSCT (3-12 and 9-13%, respectively) and had no clinical consequences.