ET1
What cell line is affected in essential thrombocythemia?
Platelets
What cell line is affected in polycythemia vera?
Red blood cells
What cell line is affected in myelofibrosis?
Red, white, and platelets -- Neutrophils & monocytes
True or False: All patients with MF regardless of risk category should be taken to transplant.
False.
Risk for thrombosis is highest in patients with ET older than _______.
60 years old
What are 3 related common symptom of polycythemia vera?
Pruritus, night sweat, fatigue
Enlargement of this organ is very common in primary myelofibrosis
Spleen
True or False. Splenectomy is no longer performed for patients with MF.
False. Splenectomy can be done for palliation, but has many complications such as extramedullary hematopoiesis
JAK2 is mutated in ______% of patients with ET.
~50 – 55%
Many polycythemia patients present with _____ as a serious complication.
Thrombotic episode
Primary myelofibrosis is difficult to differentiate from ______ myelofibrosis.
Secondary
Which stage of PV should we begin to consider to treat with upfront hydrea?
High Risk
Paradoxically, _______, can occur in ET patients
Bleeding
Polycythemia vera patients can live on average of 14 years, however, their disease can transform into ________?
Leukemia or myelofibrosis
On an MF blood smear, how do we characterize the MF cells?
Leukoerythroblastic
If using ruxolitinib for PV, how do you titrate?
In increments of 5 mg BID, max 25 mg BID no more frequently than q2 weeks (and not within the first month)
A 75 yo patient presents with plt 1.25 million and a CVA. His serum ferritin is normal. No infection is present. Spleen is intact. Besides starting aspirin, you should start ______, and how do you dose it?
Hydroxyurea 500 mg. Titrate to keep platelets < 600k
Low risk polycythemia vera is best treated by ______.
Phlebotomy to maintain a HCT < 45k + aspirin daily
True or False. A patient presents with profound fatigue, splenomegaly and pancytopenia. BM Bx is consistent with primary myelofibrosis. JAK2 (-), BCR-ABL (-), and no metastatic disease. The patient has a lower risk of transformation to AML than a patient with ET?
Name 2 therapies used to treat patients PMF or the fibrosis phase of MF disorders prior to ruxolitinib.
Androgens, steroids, thalidomide, interferon