Test 8 myeloproliferative neoplasms

Choose all that apply to the clinical findings of Essential Thrombocythemia (ET).

• often asymptomatic; neurologic involvement

• internal circulatory problems (brain stem, optic nerve, etc.)

• thrombotic or hemorrhagic complications

• Circulatory problems in extremities

• Autosplenectomy

Lab findings for Essential Thrombocythemia (ET)

›Thrombocytosis, with​ (a)   bizarre plts, plt aggregates, and ​ (b)  

›Anemia if bleeding

›​ (c)   : <40,000

›Normal to increased LAP score

›Increase in ​ (d)  

›JAK2+ ​ (e)   mutation

• giant
• microMKs
• Leukocytosis
• RBC defects
• “gain of function”
• "loss of function"
• RBCs adequate
• macroMKs
• minature

Choose all the therapies for Essential Thrombocythemia (ET).

• Blood Transfusion

• Plateletpheresis

• Anticoagulants

• Chemotherapy

• JAK2 inhibitors

The prognosis for Essential Thrombocythemia (ET) is >75% - 10 yrs and better in older patients.

• True

• False, younger

• False, adults over 30

This is the least common but most aggressive of the MPNs ___.

• Polycythemia

• Primary Myelofibrosis (PMF)

• Secondary Myelofibrosis

  (SMF)

• Tertiary Myelofibrosis

  (TMF)

Primary Myelofibrosis (PMF)

Often occurs as a consequence of ​ (a)   , but can occur spontaneously

Pathology

›Begins with ​ (b)   in BM, then fibroblast ​ (c)  

›​ (d)   hypoplasia

›Extramedullary ​ (e)  

• PV or ET
• MK hyperplasia
• over-proliferation
• BM
• hematopoiesis
• WBC
• RBC
• PU or EO
• MK hypoplasia
• hemorrhagic

Lab findings for Myelofibrosis (PMF)

›Leukoerythroblastic ​ (a)   (poikilo/anisocytosis)

›Dacryocytes

›​ (b)   anomaly

›PLT ​ (c)  

›“Dry tap”

›JAK2+ ​ (d)  

›LAP: ​ (e)   to normal

• anemia
• Pseudo-Pelger Huet
• abnormalities
• in 50%
• elevated
• decreased
• in 75%
• normal

In Primary Myelofibrosis (PMF) clinical course you will find which of these.

• anemia

• thrombocytosis

• marked hepatosplenomegaly

• autosplenectomy

Choose all the treatments for Primary Myelofibrosis (PMF).

• treatments to improve cytopenias

• Irradiation of spleen

• JAK2 inhibitors

• ellective surgery

Match the following Prognosis for Primary Myelofibrosis (PMF)

• 10-15% develop

• ALL

• 4-5 yrs

• Main causes of Death

• infection, hemorrhage, thrombosis and heart failure

• acute leukemia

• a few cases develop

• Average survival