RESUMO
Paroxysmal nocturnal hemoglobinuria is a rare disease with the incidence ranging from 0.08 to 0.57 per 100,000 person-years. Up to 25% of cases in women are detected during pregnancy. We report two cases of successful pregnancy outcomes in patients treated with eculizumab, pointing out the importance of interdisciplinary approach in these high-risk pregnancies.
Assuntos
Nitrilas , Resultado da Gravidez , Primeiro Trimestre da Gravidez , Mielofibrose Primária , Pirazóis , Pirimidinas , Humanos , Mielofibrose Primária/tratamento farmacológico , Mielofibrose Primária/diagnóstico , Pirazóis/uso terapêutico , Feminino , Nitrilas/uso terapêutico , Pirimidinas/uso terapêutico , Gravidez , Adulto , Resultado do TratamentoRESUMO
CASE REPORT: A 41-year-old man presented with anemia, lymphocytosis and splenoniegaly. T-cell large granular lymphocyte leukemia was diagnosed based on lymphocytosis of T-cell large granular lymphocytes, characteristic inimunophenotype (CD)3⺠CD8âº, CD16âº, CD57âº) of the lymphocytes and clonally rearranged T-cell receptor genes. Therapy indication was transfusion-dependent anemia. Initial cyclosporine therapy and low-dose oral methotrexate failed to control anemia and lymphocytosis. Yet, a complete clinical and hematological. response (without molecuIlar remission) was achieved and sustained when cyclosporine was reintroduced into the therapy. CONCLUSION: Our case confirms that cyclosporine therapy is effective in treating T-celI large granular lymphocyte leukemia and suggests that indefinite treatment may not be needed to maintain the response.