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

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Nội dung chi tiết: Ebook Board basics - An enhancement to MKSAP® 18: Part 2

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

HematologyAplastic Anemia and Paroxysmal Nocturnal HemoglobinuriaDiagnosisAplastic anemia is a disorder tn which hematopoietic stem cells are severely

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

thymoma. Patients with aplastic anemia are at increased risk of developing acute leukemia and MDS.Aplastic anemia. PNI I. and MDS arc all acquired de

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

fects of hematopoietic stem cells, so clinical overlap Is considerable. PNII results from a genetic mutation of membrane proteins that ameliorate comp

HematologyAplastic Anemia and Paroxysmal Nocturnal HemoglobinuriaDiagnosisAplastic anemia is a disorder tn which hematopoietic stem cells are severely

Ebook Board basics - An enhancement to MKSAP® 18: Part 2 (including Budd-Chiari syndrome)•pancytopeniaTestingThe basic evaluation of patients presenting with pancytopenia includes:•bone marrow aspirate and

biopsy (hypocelhilar with increased tat content)•cytogenetic analysis to exclude other bone marrow disorders (e.g.. MDS)•PNII screening flow cytometry Ebook Board basics - An enhancement to MKSAP® 18: Part 2

with cell surface markers CDSS and CDS9 absent• vitamin B12 and folate levels, hepatitis serologies, and 1Il V testingAplastic Anemia: Profound!/ hyp

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

ocellular bone mairoa t$ chiiactenstic, with the marrow space composed mostly of fat cells and marrow stroma.TreatmentInitial treatment of aplastic an

HematologyAplastic Anemia and Paroxysmal Nocturnal HemoglobinuriaDiagnosisAplastic anemia is a disorder tn which hematopoietic stem cells are severely

Ebook Board basics - An enhancement to MKSAP® 18: Part 2 leads to disease control in 70% of adult patients.Allogeneic 1 iscr is a potentially curative therapy and should be considered for those younger than

so years.In symptomatic patients with PN11, ecullzumab reduces Intravascular hemolysis, hemoglobinuria, and the need for transfusion. Allogeneic IISC Ebook Board basics - An enhancement to MKSAP® 18: Part 2

T can lead to long-term survival. Prophylactic anticoagulation and supplementation with iron and folic acid are indicated in all patients.193Hematolog

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

yDON'T BE TRICKED•Treatment of aplastic anemia with hematopoietic growth factors Is ineffective.•PN11 may present as a DAT-negative hemolytic anemia o

HematologyAplastic Anemia and Paroxysmal Nocturnal HemoglobinuriaDiagnosisAplastic anemia is a disorder tn which hematopoietic stem cells are severely

Ebook Board basics - An enhancement to MKSAP® 18: Part 2hrocyte production with normal leukocyte and platelet counts. The cause is predominately T cell autoimmunity (pregnancy. thymoma, malignancy) or direc

t toxicity to erythrocyte precursors (viral infection, drug toxicity).TestingBone marrow shows profound erythroid hypoplasia. Clonal CD57-positiveT ce Ebook Board basics - An enhancement to MKSAP® 18: Part 2

lls consistent with Large granular lymphocytosis are often found.The basic evaluation Is similar to that for pancytopenia but includes CT of the chest

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

to rule out thymoma.TreatmentPatients with pure red cell aplasia are treated with:•transfusion support and Immunosuppressive drugs (prednisone, cyclo

HematologyAplastic Anemia and Paroxysmal Nocturnal HemoglobinuriaDiagnosisAplastic anemia is a disorder tn which hematopoietic stem cells are severely

HematologyAplastic Anemia and Paroxysmal Nocturnal HemoglobinuriaDiagnosisAplastic anemia is a disorder tn which hematopoietic stem cells are severely

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