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Heme - Wier/Pallera > Myeloproliferative Diseases > Flashcards

Myeloproliferative Diseases Flashcards

1
Q

What defines a myeloproliferative Disease?

A

Myeloproliferative Neoplasms
• hematopoietic neoplasms
• Single clone of cells is mutated early in the differentiation of blood cells
• cells can differentiate fully into red cells, platelets and neutrophils,
• longer are well controlled in number, but still function normally

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2
Q

What reason might cause Hemoglobin and Hematocrit to be artificially increased?
• what specific conditions would do this OTHER than Polycythemia Vera?

A

Decrease in Plasma Volume

Other Conditions:
Acute
• HUGE fluid loss from the body via vomiting, diarrhea, severe burns, diabetic ketoacidosis

Chronic
• Long and inappropriate use of diuretics

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3
Q

***What is the mechanism of action of the JAK-2 V617F mutation?

A

*This is the EPO receptor that is constantly stimulated no matter if EPO is there or not

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4
Q

***Diagnostic Criteria/Process of Polycythemia Vera.

A
  1. High RBC >18.5 (male) or >16.5 (female)
  2. Check JAK2V617F and EPO (MUST BE LOW)
  3. If EPO is low and not JAC2V617F, then check JAK2 Exon12
  4. Do bone marrow biopsy
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5
Q

***Diagnostic Criteria/Process of Essential Thrombocythemia.

A
  1. Sustained Platelet Count ≥ 450,000
  2. NO ELEVATED RED COUNT
  3. Look for 2˚ causes like iron deficiency, inflammation, or malignancies
  4. JAK2V617F check, then Calrectin and MLP if JAK is (-)
  5. BONE Marrow Biopsy to Confirm Dx. showing lots of Megs.
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6
Q

***Diagnostic Criteria/Process of Primary Myelofibrosis

A
  1. Hepatosplenomegaly, Constitutional Symptoms
  2. Bone Marrow Biopsy
  3. JAK2V617F check, Calrectin, and MLP check
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7
Q

***Diagnostic Criteria/Process of Chronic Myeloid Leukemia

A

BCR-ABL fusion protein with t(9,22) detected using FISH or PCR

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8
Q

***Complications of Polycythemia Vera

A

COMPLICATIONS:

  • THROMBOSIS
  • Acute Myeloid Leukemia
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9
Q

***Treatment of Polycythemia Vera

A

Treatment:
• Phlebotomy - target hct 45%
• Asprin - 100mg/day

Second Line Treatment:
• Hydroxyurea - bone marrow suppressant

3rd Line Treatment (if hydroxyurea fails)
• Interferon - good for pregnant women
• JAK2 inhibitors

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10
Q

***Causes of Increased Neutrophils

A

Neoplastic Causes:
• MF, PV, CML, ET

Drugs:
• CORTICOSTERIODS, LITHIUM
• Infection

Others: 
• Smoking Cigarettes 
• Obesity
• Inflammation
• Malignancy
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11
Q

***Causes of Increased Eosinophils

A
  • Idiopathic Eosinophilia
  • Reactive Eosinophilia
  • Hypereosinophilia Sydrome - Idopathic, Chronic Eosinophilic Leukemia, Hematopoietic Neoplasma accompanied by eosinophilia
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12
Q

***Mutation and MOA of Philadelphia Chromosome

A

t(9,22) BCR-ABL mutation

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13
Q

***MOA of present best therapy for CML

A

Imatinib is the main drug used to bind in the ATP binding site of the mutant BCR-ABL Tyrosine Kinase

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14
Q

***What does the Peripheral Blood Smear Look like in CML?

A
  • Agranular, Early Neutrophils

* Lots of Myelocytes (very early neurophil precursor)

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15
Q

What will your EPO levels look like in PV?

A

They should be low because the JAK2 V617F mutation activates the EPO receptor in the absence of EPO

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16
Q

What are some potential causes of Increased Platelets?

*Other than neoplasms

A
  • Splenectomy
  • Trauma
  • Malignancy
  • Iron Deficiency
17
Q

What is the major difference between a myelodysplastic syndrome and a myeloproliferative neoplasm?

A

In a myelodysplastic syndrome most cells die in the bone marrow so you actually gets cytopenias rather than cyotosis

18
Q

What are the possible driver mutations causing polycythemia vera?

A
  • Jak2-V617F (90%)

* Jak2 Exon12

19
Q

What are the driver mutations that can lead to Essential Thrombocytosis or Myelofibrosis?

A
  • Jak2-V617F
  • Calreticulin
  • MPL (TPO receptor)
20
Q

Most common physical findings and symptoms of Polycythemia Vera.

A

Physical Findings:
• Splenomegaly (MOST COMMON SYMPTOM)
• Skin/conjunctival Plethora (2nd Most common)
• Hepatomegaly

Symptoms:
• Headache
• Weakness
• Pruritus

21
Q

What is the principle cause of death in polycythemia vera?
• 2nd most common?
• Median survival from initiation of Phlebotomy?

A
  • THROMBOSIS
  • Acute Myeloid Leukemia (2nd most common)

*14 year survival from intitiation of phlebotomy

22
Q

What is often seen in the peripheral smear of a person with Polycythemia Vera?

A
  • Lots of Platelets
  • Hypochromic RBCs
  • Increased Platelets
23
Q

What are 5 predictors of thrombosis in a patient with Polycythemia vera?

A 
Age > 65
Previous thrombosis
Elevated Leukocyte Count (15,000)
Cardio vascular risk factors
Elevated Hematocrit
24
Q

What conditions should be on your differential diagnosis for a high platelet count?

A
  • Inflammation
  • Trauma
  • Malignancy
  • Iron Deficiency
  • Splenectomy
  • Myeloproliferative Neoplasm
25
Q

What liver problems might result from having a JAK2 V617F mutation?

A

Budd-Chiari Symdrome (40-58% have JAK2 V617F)
Portal Vein Thrombosis (33% have JAK2 V617F)

**Pts. with JAK2 V617F may also be susceptible to SVT

26
Q

What secondary complication may result when a patient has a platelet count over 1.5 million?
• What neoplasm is most likely the cause?

A

Von Willebrand’s Disease
**Loss of vWF multimers causing issues with bleeding

*Essential Thrombocythaemia is often the cause

27
Q

What can you do for a patient with major bleeding problems due to Essential Thrombocythaemia?
• What is likely the root cause of this major bleeding problem?

A

Von Willibrand’s disease can cause excessive bleeding in ET patients with platelets above 1.5 million

  1. Correct Thrombocytosis
  2. Withdraw ASPRIN and anti-thrombotics
  3. Plasma Products that contain vWF, antifibrinolytics, DDVAP (diargininedivasopressin, but not with platelets above 1.5 million)
28
Q

What therapies can you give to someone with Essential Thrombocytopenia?

A
  • HIGH RISK patients need platelet Reduction

1st line
• Hydroxyurea (like in PV)

2nd line
• Anagrelide
• Inferferon for Pregnant Women

29
Q

**What make a patient with ET “high risk and thus in need of platelet reduction”?

A
  • Over 60 years old
  • Previous Thrombosis
  • Platelet count above 1.5 million
  • Previous Hemorrhage Related to ET
  • Diabetes or Hypertension
30
Q

What 2 myeloproliferative disorders historically causes Splenomegaly?
• What is the root cause of this?

A

Primary Myelofibrosis
Chronic Myelocytic Leukemia

**Hematopoeitic Cells are pushed out of the marrow and must try to make it up in the spleen

31
Q

What are some clinical findings you might expect with Primary Myelofibrosis?

A
  • hepatoSPLENOMEGALY
  • Fatigue
  • Anemia
  • Weight Loss
  • Night Sweats
  • Portal Hypertension
32
Q

What are you looking for in the peripheral smear of a person with myelofibrosis?

A
  1. Teardrop RBCs are KEY
  2. Nucleated RBCs
  3. Giant Platelets
33
Q

What should you look for in the blood smear of a patient with Chronic Myelogenous Leukemia?

A
  1. Agranular Neutrophils
  2. Promyelocytes

**Overall, lots of early white cells in the periphery

34
Q

How do patients often present with CML?

A
  • Not current illness, but have ELEVATED neutrophils
  • Palpable Spleen
  • Smear showing early neutrophils, mainly MYELOCYTES
35
Q

What do myelocytes and promyelocytes look like?

A

Blasts with Granular Cytoplasm

36
Q

What 2 myeloproliferative conditions can imatinib be used to treat?
• Associated Mutation

A
  1. Chronic Myelocytic Leukemia (CML)
    • BCR-ABL mutation
  2. Chronic Eosinophilic Leukemia (CEL)
    • FIP1L1-PDGFRA mutation

Heme - Wier/Pallera (12 decks)

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