TAILIEUCHUNG - Ebook Board basics - An enhancement to MKSAP® 18: Part 2

(BQ) Part 1 book “Board basics - An enhancement to MKSAP® 18” has contents: Hematology, infectious disease, nephrology, pulmonary and critical care medicine, rheumatology, oncology, and other contents. | Hematology Aplastic Anemia and Paroxysmal Nocturnal Hemoglobinuria Diagnosis Aplastic anemia is a disorder in which hematopoietic stem cells are severely diminished, resulting in hypocellular bone marrow and pancytopenia. All cell lines are involved. Autoimmune attack on stem cells is the most common identifiable cause. Other causes include toxins, ionizing radiation, drugs, nutritional deficiencies, and infections. Some patients have an associated thymoma. Patients with aplastic anemia are at increased risk of developing acute leukemia and MDS. Aplastic anemia, PNH, and MDS are all acquired defects of hematopoietic stem cells, so clinical overlap is considerable. PNH results from a genetic mutation of membrane proteins that ameliorate complement-mediated destruction of erythrocytes. PNH is characterized by: • chronic hemolytic anemia • iron deficiency through urinary losses • venous thrombosis (including Budd-Chiari syndrome) • pancytopenia Testing The basic evaluation of patients presenting with pancytopenia includes: • bone marrow aspirate and biopsy (hypocellular with increased fat content) • cytogenetic analysis to exclude other bone marrow disorders (., MDS) • PNH screening flow cytometry with cell surface markers CD55 and CD59 absent • vitamin B12 and folate levels, hepatitis serologies, and HIV testing Aplastic Anemia: Profoundly hypocellular bone marrow is characteristic, with the marrow space composed mostly of fat cells and marrow stroma. Treatment Initial treatment of aplastic anemia involves withdrawal of any potentially causative agents. Immunosuppression with cyclosporine and antithymocyte globulin is first-line therapy and leads to disease control in 70% of adult patients. Allogeneic HSCT is a potentially curative therapy and should be considered for those younger than 50 years. In symptomatic patients with PNH, eculizumab reduces intravascular hemolysis, hemoglobinuria, and the need for transfusion. Allogeneic HSCT can lead to long-term .

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