MICROANGIOPATIA TROMBÓTICA E SHU ATÍPICA: DESAFIOS DIAGNÓSTICOS E TERAPÊUTICOS: RELATO DE CASO

Thrombotic microangiopathies (MATs) are characterized by generalized microvascular occlusion, which culminate in microangiopathic hemolytic anemia, thrombocytopenia and organ damage. Phenotypically, they may manifest as Thrombotic Thrombocytopenic Purpura and Hemolytic-Uremic Syndrome, clinically serious conditions with high morbidity and mortality. The objective of this study is to report the case of a young patient, admitted to the emergency department of the Complexo Hospitalar Prefeito Edvaldo Orsi, in the city of Campinas/SP, with dyspnea and arterial hypertension secondary to hypervolemia, associated with severe acute renal dysfunction and need for emergency dialysis. After evaluating the laboratory tests, the hypothesis of Hemolytic-Uremic Syndrome (HUS) was suggested, which was later confirmed with a renal biopsy performed in an outpatient service. The information contained in this report was transmitted through a review of medical records, an interview with the medical team, registration of complementary exams and a review of the literature. The resource restrictions of the service where the patient was admitted with installed renal dysfunction, added to the fact that Eculizumab, a monoclonal antibody used to treat cases of atypical Hemolytic Uremic Syndrome (aHUS) is not made available by the Ministry of Health in Brazil for HUS treatment culminated, respectively, in late diagnosis and impossibility of adequate treatment. The patient evolved with endstage Chronic Kidney Disease and required intermittent chronic renal replacement therapy.