RESUMO
INTRODUCTION: Acquired hemophilia A is a rare disease. The incidence has been estimated to be 1.3-1.5 cases per 1 million persons per year. The etiology of acquired hemophilia A varies. It may develop in patients with autoimmune disorders, hematologic and solid cancers or in women during pregnancy or following childbirth. In about half of the cases no underlying disease can be found. The clinical picture is dominated by severe soft tissue hematomas especially in the cases of pregnancy-related acquired hemophilia A. Unlike congenital hemophilia A, bleeding into joints is rare. Pregnancy-related acquired hemophilia A may develop following any pregnancy but is observed more often in primigravidas. In most cases it arises in the postpartum period, most commonly 1-4 months after delivery. If factor VIII inhibitors develop during pregnancy or labor, they are frequently associated with severe uterine bleeding. The prognosis of pregnancy-related acquired hemophilia A is good with a high percentage of spontaneous remissions especially if the inhibitor was detected postpartum. Patients with acquired inhibitors do not usually have personal or family history of bleeding tendency, thus it is the presence of bleeding at multiple sites with prolonged activated partial thromboplastin time not corrected by incubation with normal plasma (mixing study) that raises the suspicion of inhibitor. Prompt diagnosis and treatment achieved by close collaboration among gynecologists and hematologists may improve the prognosis and prevent severe bleeding.
Assuntos
Doenças Autoimunes , Hemofilia A , Complicações Hematológicas na Gravidez , Autoanticorpos , Fator VIII , Feminino , Hemofilia A/diagnóstico , Hemofilia A/terapia , Hemorragia , Humanos , Gravidez , Complicações Hematológicas na Gravidez/diagnóstico , Complicações Hematológicas na Gravidez/terapiaRESUMO
Metastatic adenocarcinoma presenting as microangiopathic hemolytic anemia (MAHA) is a rare finding. We report the case of a young patient who presented with MAHA as the initial symptom of metastatic gastric adenocarcinoma. Cancer-related microangiopathic hemolytic anemia is a paraneoplastic syndrome characterized by Coombs-negative hemolytic anemia with schistocytes and thrombocytopenia. A high index of suspicion and prompt diagnosis can prevent ulterior deterioration, especially when chemotherapy is the optimal treatment in these cases, which can stop the hemolysis.
Assuntos
Adenocarcinoma/diagnóstico , Anemia Hemolítica/etiologia , Neoplasias Gástricas/diagnóstico , Adenocarcinoma/patologia , Adulto , Humanos , Masculino , Metástase Neoplásica , Neoplasias Gástricas/patologiaRESUMO
Several studies showed ethnic disparities in multiple myeloma (MM) incidence and prognosis. In order to compare prognosis and overall survival between different ethnic groups, a multicenter retrospective study was conducted in Northern Israel. A total of 145 patients suffering from MM were included (72% Jewish, and 28% Arabs) who were treated between 2008-2018. A difference was found in the stage of the disease at the time of diagnosis, patients of Arab origin were diagnosed at a more advanced stage (III), (53.7% vs. 33.7%, respectively). A mortality rate of 48.9% was found in the study, regardless of population ethnic origin. No significant differences in rates of MGUS, MM symptoms, treatments, or progression-free survival (PFS) and overall survival (OS) were observed between ethnic groups. This suggests that raising awareness of MM may result in an earlier diagnosis, especially among patients of Arab origin, preventing unnecessary suffering from these patients.