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Chapter 058. Anemia and Polycythemia (Part 12)

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Approach to the Patient: Polycythemia As shown in Fig. 58-18, the first step is to document the presence of an increased red cell mass using the principle of isotope dilution by administering 51 Cr-labeled autologous red blood cells to the patient and sampling blood radioactivity over a 2-h period. If the red cell mass is normal (36 mL/kg in men, 32 mL/kg in women), serum EPO levels should be measured. If EPO levels are low or unmeasurable, the patient most likely has polycythemia vera. Ancillary tests that support this diagnosis include elevated white blood cell count, increased absolute basophil count, and thrombocytosis. A mutation in...

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  1. Chapter 058. Anemia and Polycythemia (Part 12) Approach to the Patient: Polycythemia As shown in Fig. 58-18, the first step is to document the presence of an increased red cell mass using the principle of isotope dilution by administering 51 Cr-labeled autologous red blood cells to the patient and sampling blood radioactivity over a 2-h period. If the red cell mass is normal (32 mL/kg in women), serum EPO levels should be measured.
  2. If EPO levels are low or unmeasurable, the patient most likely has polycythemia vera. Ancillary tests that support this diagnosis include elevated white blood cell count, increased absolute basophil count, and thrombocytosis. A mutation in JAK-2 (Val617Phe), a key member of the cytokine intracellular signaling pathway, can be found in 70–95% of patients with polycythemia vera. Figure 58-18
  3. An approach to diagnosing patients with polycythemia. RBC, red blood cell; EPO, erythropoietin; COPD, chronic obstructive pulmonary disease; AV, atrioventricular; IVP, intravenous pyelogram; hct, hematocrit. If serum EPO levels are elevated, one attempts to distinguish whether the elevation is a physiologic response to hypoxia or is related to autonomous production.
  4. Patients with low arterial O2 saturation (
  5. Dr. Robert S. Hillman wrote this chapter in the 14th edition, and elements of his chapter were retained here. Further Readings Hillman RS et al: Hematology in Clinical Practice, 4th ed. New York, McGraw-Hill, 2005
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