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MLAB 1415-Hematology Keri Brophy-Martinez

MLAB 1415-Hematology Keri Brophy-Martinez. Chapter 13: Hypoproliferative Anemias. Overview. Disorders Acquired Congenital Anemia Normocytic or Macrocytic Normochromic Bone marrow hypocellularity Defect Depletion, damage or inhibition of stem and/or progenitor cells. APLASTIC ANEMIA.

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MLAB 1415-Hematology Keri Brophy-Martinez

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  1. MLAB 1415-HematologyKeri Brophy-Martinez Chapter 13: Hypoproliferative Anemias

  2. Overview • Disorders • Acquired • Congenital • Anemia • Normocytic or Macrocytic • Normochromic • Bone marrow hypocellularity • Defect • Depletion, damage or inhibition of stem and/or progenitor cells

  3. APLASTIC ANEMIA • Aplastic anemia • Disorder (or group of disorders) characterized by aplasia of bone marrow or its destruction by chemical agents or physical factors. All cell lines are affected. • Aplasia • Failure of a tissue or organ to develop normally

  4. Pathophysiology • Aplastic anemia is caused by an immune process, either antibodies directed against the stem cell or a cellular immune mechanism (T-lymphocyte) that suppresses stem cell prolieferation • Bone marrow fails due to the immunologically mediated tissue-specific destruction

  5. Phases • I: Onset • After an initiating event (i.e. viral infection) the hematopoietic compartment is destroyed by the immune system • Small numbers of surviving stem cells can support adequate hematopoiesis for some time, but eventually the circulating cell counts become very low and clinical symptoms appear

  6. Phases • II: Recovery • Either a partial response or a complete response can occur, initially, without increased number of stem cells • III: Late Disease • Years after recovery, blood counts may fall as pancytopenia occurs or as abnormal clones of stem cells emerge. • Often leads to PNH( paroxysmal noctural hemoglobinuria), AML (acute myelogenous leukemia) or MDS (myelodysplasia)

  7. Aplastic Anemia • Two Groups • Acquired • Surface after age 60 • Congenital • 2-5 years old • 15-25 years old

  8. APLASTIC ANEMIA • Acquired • Idiopathic or primary with no clear cause • Secondary due to • Chemical agents such as benzene, insecticides, weed killers • Drugs such as chloramphenicol (antibiotic) and phenylbutazone (anti-inflammatory), chemotherapy drugs (busulfan, vincristine), anticonvulsants (Dilantin) • Ionizing radiation from nuclear fallout, x-rays or radiation therapy, radium • Infections such as hepatitis, Epstein-Barr virus, CMV, HIV • Miscellaneous - pregnancy, malnutrition, immunologic dysfunction

  9. APLASTIC ANEMIA • Congenital/ Constitutional Aplastic Anemia • Fanconi’s anemia • Genetic predisposition to bone marrow failure • Chromosomal breaks, gaps, exchanges • 25- 30% cases of childhood aplastic anemia • Twice as common in males than females

  10. Clinical Features • Low birth weight • Skin hyperpigmentation • Short statue • Dyspnea • Bleeding • Infections • Skeletal disorders

  11. Aplastic anemia: Lab Features • Total Bone marrow failure • Hypocellular: 70% fat • Pancytopenia= all cell lines affected

  12. Aplastic Anemia:Lab Features • ↓ RBC, hgb (<7), hct (<20), retic (absolute and corrected) • ↓ WBC (<1.5) , absolute neutrophil count (<0.5) • Relative lymphocytosis due to the neutropenia • ↓ platelets (<20,000-60,000) • P.B • Normocytic,normochromic • Moderate anisocytosis, poikilocytosis • NRBCs, teardrops*

  13. Treatment • Treatment of choice is bone marrow transplantation. If this is not an option, immunotherapy is given. Spontaneous recovery may occur if the offending agent is removed. • Prognosis is poor, high mortality rate..2 years • Complications include • Bleeding • Iron overload due to transfusions

  14. Pure red cell aplasia • Rare condition • Red cell precursors in the bone marrow are decreased • Presence of peripheral blood anemia

  15. Pure Red Cell Aplasia • Congenital • Diamond-Blackfan anemia • Defect in BFU-E and CFU-E • Acquired • Hemolytic crises • Infection with parvovirus, EBV, viral hepatitis • Malnutrition • Certain drugs or neoplasms

  16. Other Hypoproliferative Anemias • Defective hormonal stimulation of erythroid progenitor cells • Renal Disease • Endocrine Disorders • Lab findings reflect anemia and pathologies of primary disorder

  17. References • Harmening, D. M. (2009). Clinical Hematology and Fundamentals of Hemostasis. Philadelphia: F.A Davis. • http://www.aamdsglossary.co.uk/glossary/b • http://blass.com.au/definitions/aplastic%20anemia • McKenzie, S. B., & Williams, J. L. (2010). Clinical Laboratory Hematology . Upper Saddle River: Pearson Education, Inc.

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