Differential Diagnosis

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Differential Diagnosis

• ARCILLA, Juan Martin E.

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Causes of Erythrocytosis Causes of Erythrocytosis
Relative Erythrocytosis Hemoconcentration secondary to dehydration, androgens, or tobaco abuse Relative Erythrocytosis Hemoconcentration secondary to dehydration, androgens, or tobaco abuse
Absolute Erythrocytosis Hypoxia Carbon monoxide intoxication High affinity hemoglobin High altitude Pulmonary disease R-L shunts Sleep apnea syndrome Neurologic disease Renal disease Renal artery stenosis Focal sclerosing or membranous glomerulonephritis Renal transplantation Tumors Hypernephroma Hepatoma Cerebellar hemangioblastoma Uterine fibromyoma Drugs Androgens Recombinant erythropoietin Familial (with normal Hgb function, Chuvash, erythropoietin receptor mutations)
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Absolute polycythemia vera apparent polycythemia vera
Either a clonal myeloproliferative disorder (polycythemia vera) or a nonclonal increase in red blood cell mass that is often mediated by erythropoietin (secondary polycythemia) Either a decrease in plasma volume (relative polycythemia) or a misperception of what constitutes the upper limit of normal values for either hemoglobin or hematocrit
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Apparent Polycythemia

• Relative Polycythemia
• conditions that cause acute depletion of volume
  e.g., severe dehydration, diarrhea, vomiting, use
  of diuretics, capillary leak syndrome, severe
  burns
• the existence of chronic contraction of the
  plasma volume
• Gaisbocks syndrome
• Stress/spurious polycythemia

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Absolute Polycythemia

• Polycythemia vera
• Secondary polycythemia
• Congenital
• Associated with high or normal serum
  erythropoietin level   
• Associated with low serum erythropoietin level
• Acquired
• Erythropoietin mediated
• Hypoxia-driven
• Hypoxia-independent (pathologic erythropoietin
  production)
• Drug associated
• Unknown mechanism

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Secondary Polycythemia

• Secondary polycythemia
• Congenital
• Associated with high or normal serum
  erythropoietin level   
• Chuvash and other polycythemias associated with
  von values Hippel-Lindau (VHL) gene mutation
• Highoxygen affinity hemoglobinopathy
• 2,3-Diphosphoglycerate mutase deficiency
• Associated with low serum erythropoietin level
• Activating mutation of the erythropoietin receptor

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Secondary Polycythemia

• Secondary polycythemia
• Acquired
• Erythropoietin mediated
• Hypoxia-driven

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Acquired Secondary Polycythemia
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Secondary Polycythemia

• Secondary polycythemia
• Acquired
• Erythropoietin mediated
• Hypoxia-independent (pathologic erythropoietin
  production)

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Acquired Secondary Polycythemia
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Secondary Polycythemia

• Secondary polycythemia
• Acquired
• Drug associated
• Erythropoietin doping
• Treatment with androgen preparations

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Secondary Polycythemia

• Secondary polycythemia
• Acquired
• Unknown mechanism
• Postrenal transplant erythrocytosis