Knowledge Check

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Sort the conditions (top) according to the type of polycythemia/erythrocytosis (bottom):

Gaisbock’s syndrome
EPOR mutations
Chronic ling disease
Testosterone use
Renal cyst
PV
Vomiting
Diarrhea
CO poisoning
Sleep apnea
Hepatoma
Diuretics
Relative
Primary absolute
Secondary absolute

Abbreviations: PV, polycythemia vera; EPOR, erythropoietin receptor; CO, carbon monoxide

Abbreviations: ODC, oxygen dissociation curve

Congenital left shift of oxygen dissociation curve (ODC). Left shift is caused by a mutation in Hb that increases oxygen affinity. The result is that for any level of pO2, oxygen saturation (HbO2 Sat [%]) is increased. There is less offloading of oxygen at the level of the tissues and this is sensed by the renal erythropoietin (EPO)-producing peritubular cell of the kidney as an hypoxic stimulus for EPO production, leading to compensatory erythrocytosis.

What tumor(s) is/are associated with secondary erythrocytosis?

a
Hepatoma
b
Atrial myxoma
c
Cerebellar hemangioblastoma
d
Uterine leiomyoma
e
Squamous cell carcinoma of skin

What is the difference between primary and secondary polycythemia/erythrocytosis?

a
Primary refers to high clinical relevance
b
Primary refers to cases occurring in childhood
c
Primary refers to cases in which the increased production of red cells is autonomous to the red cell progenitors
d
Primary refers to cases in which the increased production of red cells is driven by a factor external to the bone marrow.
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