Myeloproliferative Disorders Flashcards
(28 cards)
What is Polycythemia Vera (P Vera)?
Unregulated production of all 3 cells types: RBC overproduction is the most prominent
What is the cause of Polycythemia Vera (P Vera)?
Mutation in JAK2 Protein that is responsible for regulating marrow production leads to wild RBC growth despite a low Erythropoietin (EPO) level
What are Symptoms of P Vera related to?
Hyperviscosity from Increased RBC mass
What are common symptoms in P Vera?
Headache, Blurry vision, Tinnitus
Hypertension, Fatigue
SPlenomegaly
Bleeding from engorged blood vessels
Thrombosis from Hyperviscosity
What is the #1 Cause of Death in P Vera?
Thrombosis from Hyperviscosity
What is the most accurate test in P Vera?
JAK2 mutation testing
What other diagnostic testing is available for P Vera?
Increased Hct>60%
Elevated PLT and WBC
Elevated total RBC mass: reduced iron because its been used (dec MCV)
Normal Oxygen
Reduced Erythropoietin
Elevated B12
Elevated basophils (all myeloproliferative disorders)
How do you treat P Vera?
Phlebotomy and Aspirin: prevent thrombosis: target hct<45%
Hydroxyurea: help lower cell count
Ruxolitinib: JAK inhibitor if hydroxyurea does not help
Allopurinol or Rasburicase: protect against uric acid increase
Antihistamines
Spleen removal
What is Budd-Chiari Syndrome?
Occlusion of the hepatic vein that drains the liver
What are common causes of Budd-Chiari Syndrome?
Polycythemia Vera, PNH, Hypercoagulable states (Pregnancy)
APL syndrome
or cancer
How does Budd-Chiari Syndrome present?
Chronic or acute fulminant liver failure: liver is blocker
Pain, Jaundice, ascites with variceal bleeding
How do you diagnose Budd-Chiari Syndrome?
US, CT or MRI
How do you treat Budd-Chiari Syndrome?
Shunt placement using TIPS procedure
What is Essential Thrombocytosis?
Markedly elevated PLT count >1 million which leads to thrombosis and bleeding
How do you Treat Patients with Essential Thrombocytosis who are <60, Asymptomatic or have a platelet count <1.5 million?
No treatment needed
How do you treat patients with essential thrombocytosis who are >60, have thromboses, or platelet count >1.5 million?
Best initial treatment is Hydroxyurea
-if RBC suppression occurs use Anagrelide
Aspirin for Erythromelalgia
What mutation distinguishes Essential Thrombocytosis from Reactive Thrombocytosis?
CALR mutation
How does Hypereosinophilic Syndrome Present?
Rashes: Eczema, urticaria, cough and SOB > 6months without cancer or parasitic infection present
What does testing for Hypereosinophilic syndrome include?
Total Eosinophil Count >1,500
Negative ANA
Normal Cosyntropin stimulation test
No blasts
How do you treat Hypereosinophlic syndrome?
steroids
What does untreated Hypereosinophlic syndrome lead to?
Death from Cardiomyopathy or Thromboembolic disease
What is Systemic Mastocytosis?
Mast Cells proliferate abnormally in the skin and marrow: infiltrating the liver, spleen and nodes
What triggers Systemic Mastocytosis?
Narcotis, Asprin and NSAIDS
What are common symptoms of Systemic Mastocytosis?
Itchy skin lesions, abdominal pain
nausea, vomit, diarrhea
Urticaria pigmentosa and Darier sign
Possible flushing, hypotension and Anaphylaxis
