[Evans syndrome in infants]. / Síndrome de Evans en lactantes.

BACKGROUND: Evans syndrome is characterized by the reduction of at least two blood cell lineages in the absence of other diagnoses; it was previously described as the simultaneous or sequential development of autoimmune hemolytic anemia and immune thrombocytopenia with unknown etiology. An incidence of 37% and mortality rate of 10% were reported for Evans syndrome. CLINICAL CASES: We report the clinical presentation and evolution of Evans syndrome in two infants who were initially diagnosed with immune thrombocytopenia. The clinical diagnosis was supported on complementary studies, where hematological disorders were corroborated. Both cases received treatment with steroids and intravenous immunoglobulin. CONCLUSIONS: For the management of children with thrombocytopenia, the pediatrician must analyze for other cell lineage disorders. In the cases that we report here, we found the presence of autoimmune hemolytic anemia and monocytosis. Therefore, infectious and immunological studies must be included. The first-line treatment of choice are steroids, and intravenous immunoglobulin can be considered if severe immune thrombocytopenia is associated, as observed in these cases.

Rituximab in refractory autoimmune diseases: Brazilian experience with 29 patients (2002-2004).

OBJECTIVE: Rituximab, a monoclonal antibody against B-lymphocytes that express CD 20, is already available for the treatment of non-Hodgkin's lymphoma. Due to the increased relevance of B-cell regulation in the pathogenesis of autoimmune diseases, rituximab is being used in the treatment of patients whose condition is refractory to conventional therapy. METHODS: We retrospectively evaluated the short-term efficacy and tolerance of rituximab in patients with various autoimmune diseases who were treated at the Hospital Israelita Albert Einstein in the city of Sao Paulo. RESULTS: During the period 2002-2004, 29 patients with various autoimmune diseases were treated with rituximab 375 mg/m2 for 4 consecutive weeks, or two doses of 1 g 2 weeks apart. We observed remarkable short-term results in all cases, except for one patient with thrombocytopenic purpura. Of note, we describe the results in two patients with diseases not previously treated with rituximab (hypergammaglobulinemic purpura of Waldenstrom and eosinophilic fasciitis with hypergammaglobulinemia). Treatment was well tolerated, with no unexpected adverse events. We also observed a marked reduction in steroid dosage. CONCLUSION: Rituximab seems to be safe and effective in the treatment of patients with a variety of autoimmune diseases that are refractory to other modalities of treatment.

Erythrophagocytosis assay in patients with autoimmune hemolytic anemia.

The aim of this paper is to evaluate the erythrophagocytosis assay (EA) in patients with autoimmune hemolytic anemia (AIHA). Direct antiglobulin test (DAT), indirect antiglobulin test (IAT) and EA were performed in blood samples from 46 patients with presumed AIHA. The EA was carried out incubating patients' erythrocytes and peripheral blood monocytes. A total of 200 monocytes were analysed to determine the percentage of active phagocytic cells (% APC). In 9 of these patients the applied treatment was evaluated by DAT, IAT and EA. In 14 transfusion requirements, the compatibility tests and EA were performed. For EA, patients' monocytes were incubated with erythrocytes from previously selected units sensitized with patients' sera. The % of APC was 32.1 +/- 1.7 in 35 patients with positive DAT and 17.8 +/- 1.3 in 11 patients with negative DAT. This last value was significantly higher than that with negative controls (3.7 +/- 0.3)(p < or = 0.01). As regards the applied treatment, patients with a successful response (n = 6) showed a significant decrease in the initial % APC (31.8 +/- 1.6 to 15.3 +/- 2.4; p < or = 0.05) while DAT and IAT remained positive. In those patients who required blood transfusion the compatibility tests were positive with all the units to be transfused, whereas the % APC varied for each one. Blood units were selected according to the lower % APC.

Anemia peerniciosa associada a anemia hemolitica auto-imune - relato de um caso / Pernicious anaemia associated to auotimmune haemolytic

Relatamos o caso de uma paciente de 60 anos com anemia perniciosa que, 10 meses apos seu diagnostico, apresentou episodio de anemia hemolitica, com teste de Coombs direto positivo. A anemia perniciosa foi diagnosticada por aspirado e biopsia de medula ossea, biopsia de fundo gastrico e teste terapeutico. Nesta ocasiao, o teste de Coombs direto foi negativo. O episodio de anemia hemolitica foi tratado com corticoide, com subsequente melhora clinica e laboratorial. Um segundo episodio, 4 meses apos o primeiro, tambem foi controlado com corticoterapia. E discutida a associacao entre anemia perniciosa e fenomenos auto imunes. Como evento raro , torna-se de interesse o relato de mais um caso de anemia perniciosa associada a anemia hemolitica auto-imune