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RBC and BLEEDING DISORDERS www.freelivedoctor.com
RBC and Bleeding Disorders ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
www.freelivedoctor.com Classical RBC’s and platelets,
www.freelivedoctor.com Classical features of peripheral white cells
www.freelivedoctor.com Adult Reference Ranges for Red Blood Cells     Measurement (units) Men Women Hemoglobin (gm/dL) 13.6–17.2 12.0–15.0 Hematocrit (%) 39–49 33–43 Red cell count (10 6  /µL) 4.3–5.9 3.5–5.0 Reticulocyte count (%) 0.5–1.5 Mean cell volume (µm 3  ) 82–96 Mean corpuscular hemoglobin (pg) 27–33 Mean corpuscular hemoglobin concentration (gm/dL) 33–37 RBC distribution width 11.5–14.5
www.freelivedoctor.com Bone marrow biopsy stained with H&E (left), and smear stained with Giemsa (right).
WHERE is MARROW? ,[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
MARROW FEATURES ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
MARROW “ DIFFERENTIATION” www.freelivedoctor.com
www.freelivedoctor.com
ANEMIAS* ,[object Object],[object Object],[object Object],[object Object],[object Object],*  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. www.freelivedoctor.com
Features   of ALL anemias ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
www.freelivedoctor.com Blood Loss Acute: trauma Chronic: lesions of gastrointestinal tract, gynecologic disturbances. The features of chronic blood loss anemia are the same as iron deficiency anemia, and is defined as a situation in which the production cannot keep up with the loss
HEMOLYTIC ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
IMPAIRED PRODUCTION ,[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
MODIFIERS ,[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
HEMOLYTIC ANEMIAS ,[object Object],[object Object],[object Object],www.freelivedoctor.com
HEMOLYSIS ,[object Object],[object Object],www.freelivedoctor.com
HEREDITARY SPHEROCYTOSIS Genetic defects affecting ankyrin, spectrin, usually autosomal dominant Children, adults Anemia, hemolysis, jaundice, splenomegaly, gallstones (what kind?) www.freelivedoctor.com
Glucose-6-Phosphate  Dehydrogenase (G6PD) Deficiency ,[object Object],www.freelivedoctor.com
FEATURES of G6PD Defic. ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
Sickle Cell Disease ,[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
Clinical features of HGB-S disease ,[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
www.freelivedoctor.com
THALASSEMIAS ,[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
Hemoglobin H Disease ,[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
HYDROPS FETALIS ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
Paroxysmal Nocturnal Hemoglobinuria  (PNH) ,[object Object],[object Object],[object Object],G lycosylphos P hatidyl I nositol  www.freelivedoctor.com
Immunohemolytic Anemia ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
IMMUNOHEMOLYTIC ANEMIAS ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
COOMBS TEST ,[object Object],[object Object],www.freelivedoctor.com
HEMOLYSIS/HEMOLYTIC ANEMIAS DUE TO RBC TRAUMA ,[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
NON-Hemolytic Anemias: i.e., DE-creased Production ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
MEGALOBLASTIC ANEMIAS ,[object Object],[object Object],[object Object],www.freelivedoctor.com
www.freelivedoctor.com Decreased intake Inadequate diet, vegetarianism Impaired absorption Intrinsic factor deficiency    Pernicious anemia      Gastrectomy      Malabsorption states    Diffuse intestinal disease, e.g.,   lymphoma, systemic sclerosis Ileal resection, ileitis    Competitive parasitic uptake    Fish tapeworm infestation      Bacterial overgrowth in blind loops and   diverticula of bowel Increased requirement Pregnancy, hyperthyroidism,   disseminated cancer
Vit-B12 Physiology ,[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
Please remember that ALL megaloblastic anemias are also MACROCYTIC (MCV>94  or  MCV~100), and that not only are the RBC’s BIG, but so are the neutrophils, and neutrophilic precursors in the bone marrow too, and even more so, HYPERSEGMENTED!!! www.freelivedoctor.com
PERNICIOUS ANEMIA ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
FOLATE DEFICIENCY MEGALOBLASTIC AMEMIAS ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
Fe Deficiency Anemia ,[object Object],[object Object],[object Object],www.freelivedoctor.com
Fe Transferrin Ferritin (GREAT test) Hemosiderin www.freelivedoctor.com
Clinical Fe-Defic-Anemia ,[object Object],[object Object],[object Object],www.freelivedoctor.com
www.freelivedoctor.com
2 BEST lab tests: ,[object Object],[object Object],www.freelivedoctor.com
Anemia of Chronic Disease ,[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
APLASTIC ANEMIAS ,[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
APLASTIC ANEMIAS www.freelivedoctor.com
APLASTIC ANEMIAS ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
MYELOPHTHISIC  ANEMIAS ,[object Object],www.freelivedoctor.com
POLYCYTHEMIA ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
P. VERA ,[object Object],[object Object],www.freelivedoctor.com
BLEEDING DISORDERS (aka, Hemorrhagic “DIATHESES”) ,[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
VESSEL WALL ABNORMALITIES (NON-thrombotic cytopenic purpuras ) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
THROMBOCYTOPENIAS ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
DE-CREASED PRODUCTION ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
IN-CREASED DESTRUCTION ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
THROMBOCYTOPENIAS ,[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
I.T.P. ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
ACUTE  ITP ,[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
DRUGS ,[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
HIV ,[object Object],www.freelivedoctor.com
Thrombotic  Microangiopathies ,[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
“ QUALITATIVE” platelet disorders ,[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
BLEEDING DISORDERS due to CLOTTING FACTOR DEFICIENCIES ,[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
von Willebrand Disease ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
HEMOPHILIA A ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
HEMOPHILIA B ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
DIC, Disseminated INTRA-vascular, Coagulation ,[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
DIC, Disseminated INTRA-vascular, Coagulation ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com
Common Coagulation TESTS ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],www.freelivedoctor.com

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Rbc

  • 1. RBC and BLEEDING DISORDERS www.freelivedoctor.com
  • 2.
  • 4. www.freelivedoctor.com Classical features of peripheral white cells
  • 5. www.freelivedoctor.com Adult Reference Ranges for Red Blood Cells Measurement (units) Men Women Hemoglobin (gm/dL) 13.6–17.2 12.0–15.0 Hematocrit (%) 39–49 33–43 Red cell count (10 6 /µL) 4.3–5.9 3.5–5.0 Reticulocyte count (%) 0.5–1.5 Mean cell volume (µm 3 ) 82–96 Mean corpuscular hemoglobin (pg) 27–33 Mean corpuscular hemoglobin concentration (gm/dL) 33–37 RBC distribution width 11.5–14.5
  • 6. www.freelivedoctor.com Bone marrow biopsy stained with H&E (left), and smear stained with Giemsa (right).
  • 7.
  • 8.
  • 9. MARROW “ DIFFERENTIATION” www.freelivedoctor.com
  • 11.
  • 12.
  • 13. www.freelivedoctor.com Blood Loss Acute: trauma Chronic: lesions of gastrointestinal tract, gynecologic disturbances. The features of chronic blood loss anemia are the same as iron deficiency anemia, and is defined as a situation in which the production cannot keep up with the loss
  • 14.
  • 15.
  • 16.
  • 17.
  • 18.
  • 19. HEREDITARY SPHEROCYTOSIS Genetic defects affecting ankyrin, spectrin, usually autosomal dominant Children, adults Anemia, hemolysis, jaundice, splenomegaly, gallstones (what kind?) www.freelivedoctor.com
  • 20.
  • 21.
  • 22.
  • 23.
  • 25.
  • 26.
  • 27.
  • 28.
  • 29.
  • 30.
  • 31.
  • 32.
  • 33.
  • 34.
  • 35. www.freelivedoctor.com Decreased intake Inadequate diet, vegetarianism Impaired absorption Intrinsic factor deficiency    Pernicious anemia      Gastrectomy      Malabsorption states    Diffuse intestinal disease, e.g., lymphoma, systemic sclerosis Ileal resection, ileitis    Competitive parasitic uptake    Fish tapeworm infestation      Bacterial overgrowth in blind loops and diverticula of bowel Increased requirement Pregnancy, hyperthyroidism, disseminated cancer
  • 36.
  • 37. Please remember that ALL megaloblastic anemias are also MACROCYTIC (MCV>94 or MCV~100), and that not only are the RBC’s BIG, but so are the neutrophils, and neutrophilic precursors in the bone marrow too, and even more so, HYPERSEGMENTED!!! www.freelivedoctor.com
  • 38.
  • 39.
  • 40.
  • 41. Fe Transferrin Ferritin (GREAT test) Hemosiderin www.freelivedoctor.com
  • 42.
  • 44.
  • 45.
  • 46.
  • 48.
  • 49.
  • 50.
  • 51.
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Editor's Notes

  1. RBC 7.4 microns in diameter
  2. Topics
  3. Classical RBC’s and platelets, as in in the lab or your office with WRIGHT’s stain.
  4. Classical features of peripheral white cells
  5. Bone marrow biopsy stained with H&E (left), and smear stained with Giemsa (right). BOTH have special advantages!
  6. This is a very intense slide, you may have to write a lot of stuff down and listen well!
  7. This is also a very INTENSE slide!
  8. Platelet analogy.
  9. Note lack of a central pallor and a microcytosis, i.e., low MCV
  10. G6PD converts glucose-6-phosphate into 6-phosphoglucono-δ-lactone and is the rate-limiting enzyme of the pentose phosphate pathway .
  11. Heinz bodies, precipitated hemoglobin are seen quite well on a methylene blue stain.
  12. At first the spleen may be enlarged (left) because of HYPERPLENISM due to hemolysis, later it may infarct itself due to small vessel occlussive disease and be quite small (right), perhaps only 1/10 its normal size.
  13. H= High affinity
  14. PIGA makes GPI, defective PIGA makes defective or inadequate GPI. Why does the term hemoglobinuria imply hemolysis? What does “paroxysmal” mean” Ans: Sudden, UN-controllable
  15. What is the difference between an “agglutinin” and a “hemolysin”? Ans: “-lysis” implies complement fixation
  16. The Coombs is a routine test used in the workup of just about ALL kinds of hemolytic anemias
  17. Anemias of diminished erythropoesis. Doesn’t this just really boil down to three items?
  18. Megaloblasts on top, macrocytes on bottom. What is the difference between a megaloblast and a macrocyte? What is the difference between a megaloblast and an erythroblast?
  19. This is the HARD way to remember megaloblastic anemias
  20. This is the EASY way
  21. A great diagram of the iron cycle. Know what heme, transferrin, ferritin, and hemosiderin are in the iron cycle
  22. Relate hypochromia, microcytosis, anisocytosis to the Wintrobe indices: Ans: MCHC, MCV, RDW, respectively
  23. Most are hypochromic (low MCHC), and microcytic (low MCV) like Fe deficiency anemias but have NORMAL iron stores (i.e., hemosiderin).
  24. Fanconi syndrome and Fanconi anemia are two completely different disorders, but named after the same guy, even though the “syndrome was NOT described by him. Fanconi’s Anemia is characterized by  short stature , skeletal anomalies, increased incidence of solid tumors and leukemias, bone marrow failure ( aplastic anemia ), and cellular sensitivity to DNA damaging agents such as  mitomycin C . If you understand the cell differentiation concept, why would an aplastic anemia be less likely to involve lymphocytes?
  25. The NORMAL adult RED bone marrow in the axial skeleton should be about 50% cells and 50% fat. What is this? Perhaps around 90:10?
  26. Does this sound like the usual suspects again?
  27. What do you think the most serious consequence might be for a person with increased RBCs and platelets?
  28. Doesn’t this really boil down to TWO things? 1) Reduced platelet function, and 2) everything else?
  29. Normal platelets, but DAMAGED vessel walls
  30. At what platelet count level does SPONTANEOUS bleeding generally occur? Ans: 20K Platelets normally 150K-300K
  31. Note the last three items are ALL in the same category
  32. At what platelet count level does SPONTANEOUS bleeding generally occur? Ans: 20K Platelets normally 150K-300K
  33. Any thrombocytopenia of increased destruction should have INCREASED megakaryocytes in the marrow! JUST LIKE a hemolytic anemia has an erythroid HYPER-plasia, same principle!
  34. For all practical purposes, the same as Hemophilia A. How to differentiate? Factor assays! Note the AMAZING similarities between Hemophilia A and B
  35. What is a “consumptive” coagulopathy? Ans: the platelets and many clotting factors are “consumed”, i.e., used up!