Myeloproliferative Disorders Flashcards
Myeloproliferative
Overproduction of cells in the bone marrow Chronic myelogenous leukemia Polycythemia vera Myelofibrosis Essential thrombocystosis
Polycythemia vera (PV)
Characterized by elevated H/H
Elevated red cell mass
3 causes of polycythemia
Appropriately elevated EPO hypoxemia
Innapropriately elevated EPO (carcinoma)
Germline and somatic mutation (Pvera)
PV occurs most frequently in ___ over the age of ____.
Men over the age of 60.
most common 70 - 79
PV survival
untreated: 6 - 18 months
treated: >10 yrs
PV Symptoms
HA, weakness, dizziness, sweating. Pruritis post-bathing*** Erythromelalgia (burning in feet/hands) Thrombotic events GI discomfort
PV lab findings
H/H > 18.5/56 men, 16.5/50 women WBC: > 10.5 Platelets > 450k Elevated LDH JAK-2 mutation abnormal serum epo Absent iron storage in marrow
PV exam findings
splenomegaly facial plethora hepatomegaly injection of conjunctiva excoriation of skin gouty arthritis and tophi
PV Tx
Phlebotomy to keep HCT < 45 or 42 (fem) Myelosuppressive agents Antihistamines (pruritis) Allopurinol (gout) Aspirin (erythromelalgia)
Myelosuppressive agents
Hydroxyurea
Alpha interferon
What can PV progress to?
Myelofibrosis and Acute Lukemia
Myelofibrosis (PMF)
Marrow is replaced with collagenous connective tissue fibers.
Myelofibrosis facts
Least frequent of chronic myeloproliferative disorders
Middle aged and elderly (median age 67)
Rare in childhood
More often in men than women
Myelofibrosis Signs
Severe fatigue * LUQ Pain * radiating to L shoulder Early satiety Weight loss low grade fever/night sweats Spleno/hepatomegaly
Myelofibrosis leads to?
Acute leukemia Infection Bleeding Portal HTN Vascular complications
Myelofibrosis morphology
Tear drop RBC’s
Poik
Anisocytosis
PMF lab findings
WBC can be low or high Platelets can be low or high Elevated alkaline phosphotase Elevated LDH Elevated Uric acid Mat have JAK-2 mutation
PMF Tx
Stem cell transplant Hydroxyurea Chemotherapy JAK-2 inhibitors Splenectomy Radiation
Extramedullary Hematopoesis
Caused by myeloproliferative disorder
Other organs than the marrow producing RBC’s
Can occur in almost any organ, rendering it useless
Treated with radiation
Is PMF Ph (philadelphia chromosome) +?
Nope, its negative. boom.
Essential Thrombocytosis (ET)
Overproduction of platelets
Abnormal platelet count
Isolated thrombocytosis
Occurs in ages over 50
ET lab findings
Elevated platelet count, but normal values
Elevated megakaryocytes
JAK-2 present in many cases
ET symptoms
HA, lightheadedness, visual changes
Numbness, tingling, burning
splenomegaly
Thrombosis
ET Tx
Low dose aspirin
Hydryxyurea: keep platelets below 500,000
ET can progress to…
Myelofibrosis and acute leukemia
massive splenomegaly
