MPNs Flashcards
What are the hematologic parameters that define P. Vera?
Hgb of 16.5 for men and 16 for women.
What platelet count is needed for ET?
A platelet count of 450K or higher
In ET what is the one thing to keep in mind about platelet morphology?
The platelets are large and mature appearing and form in loose clusters. In other words there are no major abnormalities present, if there are you should consider another diagnosis.
What should make you consider congenital erythrocytosis?
Laboratory workup will show chronically elevated EPO levels or normal levels in addition to chronic polycythemia. p50 testing will be normal. There will be a family history.
What are some congenital conditions that can cause chronically elevated Hgb? What additional workup can be done and what is the EPO level for each?
For all of these conditions you can do a p50 test which will be normal. VPL-EPO level will be normal. HIF2a-EPO will be increased. PHD2-EPO will be increased.
If the p50 is shifted to the left indicating an increased affinity for O2 what causes of erythrocytosis could this indicate?
Abnormal hemoglobin variants such as Hemoglobin M disease. It could also indicate 2,3 bisphosphoglycerate def
When the EPO is very low what congenital cause of erythrocytosis could this indicate? (You want to keep this mind when trying to differentiate this from PV where EPO is subnormal).
This could indicate autosomal dominant EPO disease where they have a signal defect and the receptor is always turned on.
What are some medications that cause elevated EPO levels?
Testosterone (androgen) use and SGLT inhibitors
In JAK2 unmutated erythrocytosis how do you treat these patients?
Only give phlebotomy if they have symptoms. DO NOT give cytoreductive therapy for these patients. If they have a history of thrombosis then you give AC or ASA for cardiac event.
What determines very low risk and low risk ET? And what do you do?
In those patients younger than age 60, no thrombosis, and no JAK2 mutation these patients are at very low risk and require no tx (give ASA only if they have vasomotor symptoms). Patients younger than age 60 and JAK2 w/no hx of thrombosis are at low risk-you start ASA 81mg daily for these patients per NCCN.
What determines intermediate and high risk ET and what do you do?
Intermediate risk patients are older than 60 but do not have JAK2 mutation and no hx of thrombosis. You just monitor, GW lecture they mentioned you can start ASA. High risk-anyone with history of thrombosis OR older than 60 with JAK2. These patients need cytoreduction therapy.
Who gets aspirin once daily and twice daily in ET?
For those patients who have low risk disease and intermediate risk disease with CV risk factors you give these patients twice daily dosing. Those with high risk and arterial event. Everyone else gets once daily dosing.
Besides Hydrea what other options can you use for cytoreduction in ET? What do you do in pregnancy or those desiring to get pregnant?
Interferon alpha or anegralide. For those that do not respond to Hydrea or Interferon or cannot tolerate you give you Busulfan (per ASH, not in NCCN-they recommend Jakafi). Pregnancy-interferon alpha.
What is defined as low risk PV? What is the management?
No hx of thrombosis and age less than 60. You can use twice daily ASA if CV risk factors, leukocytosis, and microvascular symptoms. Also all patients should get phlebotomy to get Hct to less than 45%. And every patient should get ASA!
For those with low risk disease who still have symptoms from PV despite aggressive phlebotomy what do you do?
You can give interferon alpha-if they didn’t respond to Hydrea. For those that don’t respond completely to Hydrea or Interferon you can use Jakafi. Busulfan is also an option-third line option per ASH.
