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Robert E. Richard, MD, PhD Assistant Professor Division of Hematology University of Washington School of Medicine rrichard@u.washington.edu faculty.washington.edu/rrichard
Objectives
Review red blood cell disorders for which transfusions are therapeutic. Discuss controversial areas of transfusion therapy in red blood cell disorders. Understand the risks related to long term transfusion therapy (non-infectious).
Definition:
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 dont order that test!)
Anemia ?
Production? Survival/Destruction?
Morphological Approach
(big versus little)
First, measure the size of the RBCs:
Use of volume-sensitive automated blood cell counters, such as the Coulter counter. The red cells pass through a small aperture and generate a signal directly proportional to their volume. Other automated counters measure red blood cell volume by means of techniques that measure refracted, diffracted, or scattered light By calculation from an independently-measured red blood cell count and hematocrit: MCV (femtoliters) = 10 x HCT(percent) RBC (millions/L)
Underproduction
Normocytic Anemia of chronic disease Mixed deficiencies Renal failure Microcytic Iron deficiency Thal. trait Anemia of chronic disease (30-40%) sideroblastic anemias
Acquired
Immunological Toxins Benzene Drugs methotrexate, chloramphenicol Viruses EBV, hepatitis
Hereditary
Fanconi, Diamond-Shwachman
All lineages affected. Most patients require red cell transfusions. Transplant when possible. Transfusions should be used selectively to avoid sensitization (no family donors!).
Preleukemia, most commonly in the elderly. Supportive care that involves transfusion therapy is an option. Poor response to growth factors
Barosi G. Inadequate erythropoietin response to anemia: definition and clinical relevance. Ann Hematol. 1994;68:215-223 (early review)
Anemia associated with marrow infiltration teardrops Cancer, infections Treatment is aimed at the underlying disease Supportive transfusions as needed.
Hemolytic Anemias
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.
episodic, acute transfusions to stabilize or reverse complications. long-term, prophylactic transfusions to prevent future complications.
Controversial uses:
Prior to contast media exposure Sub-clinical neurological damage Priapism Leg Ulcers Pregnancy
Thalassemias
Genetic defect in hemoglobin synthesis
synthesis of one of the 2 globin chains ( or b) Imbalance of globin chain synthesis leads to depression of hemoglobin production and precipitation of excess globin (toxic)
Ineffective erythropoiesis
Ranges in severity from asymptomatic to incompatible with life (hydrops fetalis)
Thalassemias
Dx:
Smear: microcytic/hypochromic, misshapen RBCs b-thal will have an abnormal Hgb electrophoresis (HbA2, HbF) The more severe -thal syndromes can have HbH inclusions in RBCs Fe stores are usually elevated
Thalassemias
The only treatments are stem cell transplant and simple transfusion. Chelation therapy to avoid iron overload has to be started early.
Conclusions
Transfuse for any severe anemia with physiologic compromise. Decide early whether transfusion will be rare or part of therapy. Avoid long-term complications by working with your blood bank and using chelation theraoy.