TAILIEUCHUNG - Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 10)

Definition and Differential Diagnosis PRCA is characterized by anemia, reticulocytopenia, and absent or rare erythroid precursor cells in the bone marrow. The classification of PRCA is shown in Table 102-4. In adults, PRCA is acquired. An identical syndrome can occur constitutionally: Diamond-Blackfan anemia, or congenital PRCA, is diagnosed at birth or in early childhood and often responds to glucocorticoid treatment; a minority of patients have etiologic mutations in a ribosomal RNA processing gene called RPS19. Temporary red cell failure occurs in transient aplastic crisis of hemolytic anemias due to acute parvovirus infection (Chap. 177) and in transient erythroblastopenia of. | Chapter 102. Aplastic Anemia Myelodysplasia and Related Bone Marrow Failure Syndromes Part 10 Definition and Differential Diagnosis PRCA is characterized by anemia reticulocytopenia and absent or rare erythroid precursor cells in the bone marrow. The classification of PRCA is shown in Table 102-4. In adults PRCA is acquired. An identical syndrome can occur constitutionally Diamond-Blackfan anemia or congenital PRCA is diagnosed at birth or in early childhood and often responds to glucocorticoid treatment a minority of patients have etiologic mutations in a ribosomal RNA processing gene called RPS19. Temporary red cell failure occurs in transient aplastic crisis of hemolytic anemias due to acute parvovirus infection Chap. 177 and in transient erythroblastopenia of childhood which affects normal children. Table 102-4 Classification of Pure Red Cell Aplasia Self-limited Transient erythroblastopenia of childhood Transient aplastic crisis of hemolysis acute B19 parvovirus infection Fetal red blood cell aplasia Nonimmune hydrops fetalis in utero B19 parvovirus infection Hereditary pure red cell aplasia Congenital pure red cell aplasia Diamond-Blackfan syndrome Acquired pure red cell aplasia Thymoma and malignancy Thymoma Lymphoid malignancies and more rarely other hematologic diseases Paraneoplastic to solid tumors Connective tissue disorders with immunologic abnormalities Systemic lupus erythematosus juvenile rheumatoid arthritis rheumatoid arthritis Multiple endocrine gland insufficiency Virus Persistent B19 parvovirus hepatitis adult T cell leukemia virus Epstein- Barr virus Pregnancy .

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• Aplastic Anemia
• Myelodysplasia
• Related Bone Marrow Failure Syndromes
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• Harrisons Internal Medicine
• Severe aplastic anemia
• Hematopoietic stem cell transplantation
• Conditioning regimen
• Iron overload
• Matched sibling donor
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