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Good Morning! Welcome Applicants! 

Good Morning! Welcome Applicants! . Morning Report: Friday, December 2 nd. A Little Pathology, Anyone??. Schistocytes. Microangiopathic hemolytic anemia (TTP, HUS, HELLP, DIC, vasculitis ), severe burns, and valve hemolysis. Target Cells.

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Good Morning! Welcome Applicants! 

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  1. Good Morning! Welcome Applicants! Morning Report: Friday, December 2nd

  2. A Little Pathology, Anyone??

  3. Schistocytes Microangiopathic hemolytic anemia (TTP, HUS, HELLP, DIC, vasculitis), severe burns, and valve hemolysis

  4. Target Cells Liver disease, thalassemia, and other hemoglobinopathies

  5. Teardrop Cells Myelofibrosis/ myeloid metaplasia, thalassemia

  6. A Comparison… Burr Cells (Echinocytes) Spur Cells (Acanthocytes) Uremia Liver disease

  7. Howell-Jolly Bodies Splenectomy or functional asplenia

  8. Sickle Cells

  9. Elliptocytes

  10. Spherocytes

  11. Hereditary Spherocytosis

  12. Epidemiology and Genetics • Most common red cell membrane disorder • Incidence in the US: 1/5000 • Increased numbers among persons of northern European descent • Most cases inherited in the AD fashion • 25% with no FHx spontaneous mutation or recessive forms of the disease

  13. Pathogenesis • Spectrin deficiency unstable red cell membrane loss of cell surface area shift in form from a biconcave disc to a sphere decreased ability to circulate freely through the narrow capillaries RBC trapping in spleen

  14. Signs and Symptoms • Jaundice • Anemia • Mild (HgB 11-15 g/dL, retic <5%) • Moderate (HgB 8-12 g/dL, retic 5-10%) • Severe (HgB 6-8 g/dL, retic> 10%) • Splenomegaly

  15. Laboratory Evaluation • CBC • Low HgB • High reticulocyte ct • MCH high • Peripheral smear • Spherocytes (round RBC lacking central clearing) • Polychormasia (larger bluish cells)

  16. Laboratory Evaluation • Osmotic fragility • Unstable RBC membrane increased osmotic fragility

  17. General Management • Annual PE • Document growth and development • Measure spleen size • CBC and retic ct • Family education

  18. Management of Complications • Red Cell Transfusion • Splenectomy

  19. Management of Complications • Cholecystectomy • Chronic hemolysis gallstones • Cholecystectomy recommended for painful, symptomatic gallstones or bile duct obstruction

  20. Aplastic Crisis in HS

  21. A Little Background… • Caused by Parvovirus B19 • Transient cessation of erythropoiesis • Characterized by an absence of erythroid precursors in the BM and absence of reticulocytes in the peripheral circulation • Can result in a potentially life-threatening decrease in HgB • Can be seen in ANY chronic hemolytic condition

  22. An Important Distinction… • Aplastic crisis • Patients with chronic hemolytic anemia must maintain an increased rate of erythropoiesis to account for shortened RBC survival • Dramatic arrest of erythropoiesis precipitous drop in HgB • Relative erythroidhypoplasia • Can be caused by infectious, metabolic, or nutritional disorders • More indolent progression

  23. Clinical Features Clinical Appearance Laboratory Findings Profound anemia Low retic ct <1% Bilirubin at baseline Lymphocytosis, eosinophelia or neutropenia Thrombocytopenia • Fever • GI complaints • HA • URI symptoms • Pallor • Weakness • Lethargy

  24. Management and Course • Recovery of erythropoiesis occurs spontaneously in 6-10 days • Until then, SUPPORTIVE CARE!! • Oxygen • Transfusion therapy • Folic acid • Respiratory and contact isolation

  25. Thanks for your attention!! Noon conference: Dr. Liacouras, EosinophilicEsophagitis (LUNCH PROVIDED!!)

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