Red Cell Disorders Robert E. Richard, MD, PhD Assistant Professor Division of Hematology University of Washington School of Medicine firstname.lastname@example.org faculty.washington.edu/rrichard Objectives Review red blood cell disorders for which transfusions are therapeutic.
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Robert E. Richard, MD, PhD
Division of Hematology
University of Washington School of Medicine
Anemia is operationally defined as a reduction in one or more of the major RBC measurements:
hemoglobin concentration, hematocrit, or RBC count
Keep in mind these are all concentration measures
…most accurately measured by obtaining a RBC mass via isotopic dilution methods!
(Please don’t order that test!)
1. Biologic or kinetic approach.
The key test is the …..
(Maturation time = 1 for Hct=45%, 1.5 for 35%, 2 for 25%, and 2.5 for 15%.)
(big versus little)
Barosi G. Inadequate erythropoietin response to anemia: definition and clinical relevance. Ann Hematol. 1994;68:215-223 (early review)
Hemolytic anemias are either acquired or congenital. The laboratory signs of hemolytic anemias include:
1. Increased LDH (LDH1) - sensitive but not specific.
2. Increased indirect bilirubin - sensitive but not specific.
3. Increased reticulocyte count - specific but not sensitive
4. Decreased haptoglobin - specific but not sensitive.
5. Urine hemosiderin - specific but not sensitive.
The indirect bilirubin is proportional to the hematocrit, so with a hematocrit of 45% the upper limit of normal is 1.00 mg/dl and with a hematocrit of 22.5% the upper limit of normal for the indirect bilirubin is 0.5mg/dl. Since tests for hemolysis suffer from a lack of sensitivity and specificity, one needs a high index of suspicion for this type of anemia.
A) Deoxyhemoglobin S
B) Paired strands of
C) Hydrophobic pocket
for 6b Val
D) Charge and size prevent
6b Glu from binding.
Dykes, Nature 1978; JMB 1979
Crepeau, PNAS 1981
Wishner, JMB 1975
Vichinsky et al., NEJM 1995
Inappropriate uses of transfusion: