Aplastic anemia | Anatomy2Medicine
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Aplastic anemia

    • Aplastic Anemia
      • syndrome of chronic primary hematopoietic failure and attendant pancytopenia (anemia, neutropenia, and thrombocytopenia).
      • Persistent marrow aplasia occur most commonly viral hepatitis of the non-A, non-B, non-C, non-G type (MCQ)
      • Fanconi anemia
        • autosomal recessive disorder  (MCQ)

 

  • caused by defects in a multiprotein complex that is required for DNA repair  (MCQ)

 

        • Marrow hypofunction
        • accompanied by multiple congenital anomalies
          • hypoplasia of the kidney and spleen

 

  • Bone anomalies, which most commonly involve the thumbs or radii. (MCQ)

 

      • Inherited defects in telomerase are found in 5% to 10% of adult-onset aplastic anemia  (MCQ)

 

  • about 65% of cases fall into this idiopathic category. (MCQ)

 

      • Antithymocyte globulin and cyclosporine are used in treatment
      • they suppress or kill autoreactive T-cell clones. (MCQ)
      • Morphology

 

  • markedly hypocellular bone marrow is largely devoid of hematopoietic cells;

 

        • often only fat cells, fibrous stroma, and scattered lymphocytes and plasma cells remain. (MCQ)
        • Marrow aspirates often yield little material (a “dry tap”);
        • aplasia is best appreciated in marrow biopsies
        • If the anemia necessitates multiple transfusions, systemic hemosiderosis can appear.
        • Splenomegaly is characteristically absent (MCQ)
          • if  splenomegaly is present, the diagnosis of aplastic anemia should be seriously questioned.
        • The red cells are usually slightly macrocytic and normochromic.
        • Reticulocytopenia is the rule. (MCQ)
      • The diagnosis rests on examination of a bone marrow biopsy.
      • other causes of pancytopenia (MCQ)

 

  • “aleukemic” leukemia

 

        • myelodysplastic syndromes
      • In aplastic anemia, the marrow is hypocellular (and usually markedly so), myeloid neoplasms are associated with hypercellular marrows filled with neoplastic progenitors. (MCQ)
      • Bone marrow transplantation  is treatment of choice in those with a suitable

 

  • Pure Red Cell Aplasia

 

      • a primary marrow disorder
      • only erythroid progenitors are suppressed
      • may occur in association with

 

  • thymoma  (MCQ)

 

        • large granular lymphocytic leukemia
        • drug exposures
        • autoimmune disorders,

 

  • parvovirus infection  (MCQ)

 

    • Myelophthisic anemia
      • a form of marrow failure
      • caused by space-occupying lesions replace normal marrow elements
      • Causes
        • commonest cause is metastatic cancer (MCQ)
          • most often carcinomas arising in the breast, lung, and prostate. (MCQ)
        • any infiltrative process (e.g., granulomatous disease) involving the marrow can produce identical findings.

 

  • Leukoerythroblastosis is seen  (MCQ)

 

        • abnormal release of nucleated erythroid precursors and immature granulocytic forms (leukoerythroblastosis) into peripheral smears
      • teardrop-shaped red cells is seen (MCQ)

 

  • which are believed to be deformed during their tortuous escape from the fibrotic marrow.

 

    • Major Causes of Aplastic Anemia
      • ACQUIRED
        • Idiopathic
          • Acquired stem cell defects
          • Immune mediated
        • Chemical Agents
          • Dose related
            • Alkylating agents (MCQ)
            • Antimetabolites
            • Benzene
            • Chloramphenicol (MCQ)
            • Inorganic arsenicals
          • Idiosyncratic
            • Chloramphenicol (MCQ)
            • Phenylbutazone
            • Organic arsenicals
            • Methylphenylethylhydantoin
            • Carbamazapine (MCQ)
            • Penicillamine
            • Gold salts
        • Physical Agents
          • Whole-body irradiation
        • Viral Infections
          • Hepatitis (unknown virus)
          • Cytomegalovirus infections
          • Epstein-Barr virus infections
          • Herpes zoster (Varicella zoster)
      • INHERITED
        • Fanconi anemia (MCQ)
        • Telomerase defects