1 / 23

RBC and BLEEDING DISORDERS

This article explores the normal anatomy and physiology of red blood cells (RBC) and bleeding disorders, including anemias, polycythemia, and hemolytic disorders. It also discusses the clinical features and causes of RBC disorders. (500 characters)

karruda
Download Presentation

RBC and BLEEDING DISORDERS

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. RBC and BLEEDING DISORDERS

  2. RBC and Bleeding Disorders • NORMAL • Anatomy, histology • Development • Physiology • ANEMIAS • Blood loss: acute, chronic • Hemolytic • Diminished erythropoesis • POLYCYTHEMIA • BLEEDING DISORDERS

  3. WHERE is MARROW? • Yolk Sac: very early embryo • Liver, Spleen: NEWBORN • BONE • CHILDHOOD: AXIAL SKELETON & APPENDICULAR SKELETON BOTH HAVE RED (active) MARROW • ADULT: AXIAL SKELETON RED MARROW, APPENDICULAR SKELETON YELLOW MARROW

  4. MARROW FEATURES • CELLULARITY 50% • MEGAKARYOCYTES at least 1-2/hpf • M:E RATIO  3:1 • MYELOID MATURATION  1/3 bands or more • ERYTHROID MATURATION  nucleus/cytoplasm • LYMPHS, PLASMA CELLS  small percentage • STORAGE IRON, i.e., HEMOSIDERIN present • “FOREIGN CELLS”

  5. ANEMIAS* • BLOOD LOSS • ACUTE • CHRONIC • IN-creased destruction (HEMOLYTIC) • DE-creased production * A good definition would be a decrease in OXYGEN CARRYING CAPACITY, rather than just a decrease in red blood cells, because you need to have enough blood cells THAT FUNCTION, and not just enough blood cells.

  6. Featuresof ALL anemias • Pallor, where? • Tiredness • Weakness • Dyspnea • Palpitations • Heart Failure (high output)

  7. HEMOLYTIC • HEREDITARY • MEMBRANE disorders: e.g., spherocytosis • ENZYME disorders: e.g., G6PD deficciency • HGB disorders (hemoglobinopathies) • ACQUIRED • MEMBRANE disorders (PNH) • ANTIBODY MEDIATED, transfusion or autoantibodies • MECHANICAL TRAUMA • INFECTIONS • DRUGS, TOXINS • HYPERSPLENISM

  8. IMPAIRED PRODUCTION • Disturbance of proliferation and differentiation of stem cells: aplastic anemias, pure RBC aplasia, renal failure • Disturbance of proliferation and maturation of erythroblasts • Defective DNA synthesis: (Megaloblastic) • Defective heme synthesis: • Deficient globin synthesis: (Thalassemias)

  9. MODIFIERS • MCV, microcytosis, macrocytosis • MCH • MCHC, hypochromic • RDW, anisocytosis

  10. HEMOLYTIC ANEMIAS • Life span LESS than 120 days • Marrow hyperplasia (M:E), (erythropoitin (EPO) + • Increased catabolic products, e.g., bilirubin, hemosiderin, haptoglobin-HGB

  11. HEMOLYSIS • INTRA-vascular (vessels) • EXTRA-vascular (spleen)

  12. M:E Ratio normally 3:1

  13. HEREDITARY SPHEROCYTOSIS Genetic defects affecting ankyrin, spectrin, usually autosomal dominant Children, adults Anemia, hemolysis, jaundice, splenomegaly, gallstones (what kind?)

  14. Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency • A- and Mediterranean are most significant types

  15. FEATURES of G6PD Defic. • Genetic: Recessive, X-linked • Can be triggered by foods (fava beans), oxidant substances drugs (primaquine, chloroquine), or infections • HGB can precipitate as HEINZ bodies • Acute intravascular hemolysis can occur: • Hemoglobinuria • Hemoglobinemia • Anemia

  16. Sickle Cell Disease • Classic hemoglobinopathy • Normal HGB is α2 β2: β-chain defects (Val->Glu) • Reduced hemoglobin “sickles” in homozygous • 8% of American blacks are heterozygous

  17. Clinical features of HGB-S disease • Severe anemia • Jaundice • PAIN (pain CRISIS) • Vaso-occlusive disease: EVEREWHERE, but clinically significant bone, spleen (autosplenectomy) • Infections: Pneumococcus, Hem. Influ., Salmonella osteomyelitis

More Related