Test 7

Match the following types of Polycythemia.

• increased erythropoiesis independent of erythropoietin (EPO) production

• secondary polycythemia

• looks like it is increased

  dehydration, hemoconcentration, Gaisbock's syndrome HBP

• Relative Polycythemia

• primary more marrow issues

• Polycythemia vera

• marrow to produce high RBCs because of something else (tissue hypoxia, high altitude, renal ischemia

• Pathophysiology

Pathophysiology of Polycythemia Vera (PV) continued

›All ​ (a)   may be ​ (b)   , but RBC is ​ (c)   feature

›Cell ​ (d)   and ​ (e)   are normal

• lineages
• increased
• outstanding
• maturation
• function
• decreased
• issues
• lowest
• divison
• seperation

Clinical features of Polycythemia Vera (PV) include all of the following except

• Hypotension

• headache, weakness, fatigue

• CVD, Thrombotic episodes

• Hypertension

This is another clinical feature of Polycythemia Vera (PV) severe itching of the skin

• Pruritis

• Plethora

• Hypertension

• Thrombotic episodes

Polycythemia Vera (PV) Lab findings

›​ (a)   erythrocytosis 6-10 million

›Hgb ​ (b)   and Hct >52%

›​ (c)   iron in BM

›EPO normal or decreased

›JAK2 mutation causes​ (d)  

​ (e)   used to screen if PV suspected

›Various cytogenetic abnormalities; Trisomy 8

›Elevated LAP score

• Absolute
• >18 g/dL
• Decreased
• “gain of function”
• PCR
• Increased
• Probable
• PCH
• <18 g/dL

Polycythemia Vera (PV) Lab findings

›​ Absolute erythrocytosis ​ (a)  

›Hgb >18 g/dL​ and ​ (b)  

›​ Decreased ​ (c)   in BM

›​ (d)   normal or decreased

›JAK2 mutation causes​ "gain of function"

PCR used to ​ (e)   if PV suspected

›Various cytogenetic abnormalities; Trisomy 8

›Elevated LAP score

• 6-10 million
• Hct >52%
• iron
• EPO
• screen
• diagnose
• EPA
• magnesium
• Hct <52%
• 80 million

Which of these is not a prognosis of Polycythemia Vera (PV)

• without treatment: 18 months

• with phlebotomy: up to 14 yrs

• with myelosuppression: 12-14 yrs

• JAK2 inhibitors

• Patients will not need iron supplements

Choose all of the treatments that could help a patient with Polycythemia Vera (PV).

• Phlebotomy

• Myelosuppressive/Cytoreductive therapy

• patients often on anti-PLT therapy and may need iron supplements

• transfusion of blood

A myeloproliferative syndrome primarily affecting the megakaryocytic lineage (bone marrow defect) is

• Essential Thrombocythemia (ET)

• Polycythemia

• Plethura

• Acute Thrombosis

Essential Thrombocythemia (ET)

​ (a)   normal

​ (b)   overproduction of ​ (c)   in BM results in ​ (d)  

• TPO
• Sustained
• megakaryocytes
• defective PLT
• TPK
• thrombocytes
• Constant
• sufficient PLT