RESUMO Antecedentes: O sangramento em hemofílicos causa complicações no sistema nervoso central e periférico (SNC e SNP). A incidência de hemorragia intracraniana diminuiu após a introdução da profilaxia com fator VIII ou IX, entretanto esse benefício ainda não foi avaliado no SNP. Objetivo: O objetivo deste estudo foi determinar a prevalência de complicações neurológicas em hemofílicos, verificando o efeito da terapia profilática também no SNP. Métodos: Avaliamos retrospectivamente a prevalência de complicações neurológicas causadas por sangramentos em hemofílicos atendidos no Hemocentro Regional Norte, Ceará, Brasil, de 1992 a 2018, comparando a incidência em diferentes períodos (antes e depois da introdução do tratamento profilático em 2011). Resultados: Foram avaliados 75 pacientes com hemofilia A (4,61/100 mil habitantes). Deles, 13,3% (n=10) tinham distúrbios do SNC (n=5) ou do SNP (n=5) secundários a hemorragias. Os pacientes submetidos à terapia profilática com fator VIII apresentaram menor probabilidade de eventos do SNC: de 1992 a 2011, cinco de 63 pacientes apresentaram hemorragia no SNC, enquanto de 2011 a 2018 não ocorreram novos casos (p=0,0181). De 2011 a 2018, cinco eventos no SNP ocorreram entre pacientes sem terapia profilática, e nenhum ocorreu entre aqueles cobertos pela profilaxia (5/20 × 0/29, p=0,0081). Conclusões: A prevalência de complicações neurológicas em hemofílicos em nossa coorte é similar à de outros estudos. Assim como no SNC, a terapia profilática também reduz o risco de complicações no SNP. Este é o primeiro relato na literatura a mostrar esse benefício.

ABSTRACT Background: Bleeding in hemophiliacs can cause complications in the central and peripheral nervous system (CNS and PNS). The incidence of intracranial hemorrhage has reduced after the introduction of prophylactic treatment with factor VIII or IX, but the benefits of this therapy have not yet been evaluated on PNS complications. Objective: The aim of this study was to determine the prevalence of neurological complications in hemophiliacs and verify the effect of prophylactic therapy in these patients, including PNS disorders. Methods: We retrospectively evaluated the prevalence of CNS and PNS disorders caused by bleeding in hemophiliacs seen at the Hemocentro Regional Norte, Ceará, Brazil, from 1992 to 2018, and we compared the incidence in different periods (before and after the introduction of prophylactic treatment in 2011). Results: Of 75 hemophilia A patients evaluated (4.61/100.000 population), 13.3% (n=10) had either CNS (n=5) or PNS (n=5) disorders secondary to bleeding. Patients submitted to factor VIII replacement prophylactic therapy were less likely to have CNS events: from 1992 to 2011, 5 of 63 patients had CNS disease, while from 2011 to 2018, there were no new cases (p=0.0181). From 2011 to 2018, 5 PNS events occurred in patients without prophylactic therapy, whereas none occurred in those covered by prophylactic therapy (5/20 versus 0/29, p=0.0081). Conclusions: The prevalence of neurological complications in hemophiliacs in our cohort is similar to other studies. Similar to CNS, prophylactic therapy also reduces the risk of PNS complications. This is the first report in the literature showing this benefit.

ABSTRACT Introduction: The management of adult (≥18 years) immune thrombocytopenia patients relies on platelet count, the risk of bleeding and presence of bleeding. Objective: Confirming the diagnosis of immune thrombocytopenia and the start of therapy, our hematology service, a referral center, favors the establishment of this algorithm to treat those patients. Results: Presentation, recently diagnosed or recurrence - group 1: life-threatening bleeding: high-dose intravenous immunoglobulins with methylprednisolone or dexamethasone. Hospitalization and platelet transfusion are considered. Group 2: Platelets <30 × 109/L with bleeding or risk factor for bleeding, or platelets <20 × 109/L: prednisone or dexamethasone. No response, platelets <20 × 109/L: replace corticoid or increase doses. If platelets continue <20 × 109/L: immunization and splenectomy. Investigation of Helicobacter pylori, if positive: treatment for H. pylori. Chronic immune thrombocytopenia with platelets <20 × 109/L we propose two new groups (A and B): Group A: <65 years, no or low surgical risk, patient declines maintenance therapy or patient intends to get pregnant: immunization and splenectomy. Group B: failure of splenectomy (refractory) or no splenectomy indication or history of exposure to malaria or babesiosis and no response to corticoids or corticoid dependence: choose thrombopoietin receptor agonists: eltrombopag or romiplostim. Patient at high risk for arterial or venous thrombosis: recommend rituximab. After rituximab or thrombopoietin receptor agonists, if platelets continue <20 × 109/L: indicate immunosuppressants (azathioprine or cyclophosphamide), dapsone or mycophenolate mofetil or vinca alkaloids. The goals of treatment for chronic or refractory immune thrombocytopenia are to keep platelets >20 × 109/L and stop bleeding.