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Clinical Medicine: Hematology and Oncology > Special Consideration Hematology Disorders > Flashcards

Special Consideration Hematology Disorders Flashcards

1
Q

Megakaryocyte proliferation that leads to high platelet count with a high tendency for thrombosis or hemorrhage. Associated with a JAK2 mutation.

A

Essential Thrombocythemia

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2
Q

Sometimes the first sign of Essential Thrombocythemia is thrombosis, where may this occur at in the body?

A

Hepatic Veins

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3
Q

What is the mean age at diagnosis of Essential Thrombocytopenia?

A

64 - 73

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4
Q

Who has a higher incidence of Essential Thrombocythemia?

A

Women

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5
Q

A sign seen with Essential Thrombocythemia possibly caused by intravascular activation and aggregation of platelets with subsequent smudging or occlusion of the distal limb arterial microvasculature

A

Erythromelalgia
(burning of hands and feet)

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6
Q

How is Essential Thrombocytopenia usually discovered?

A

Routine labs in asymptomatic patients

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7
Q

What is the progression of Essential Thrombocytopenia?

A

Thrombosis
Hemorrhage
Progression to Myelofibrosis

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8
Q

What will a blood smear show in a patient with Essential Thrombocythemia?

A

Elevated Platelet Count
(large platelets)

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9
Q

What lab test should you perform on a patient you suspect to have Essential Thrombocythemia to rule out other causes?

A

Complete Metabolic Panel with Lactate Dehydrogenase

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10
Q

What else should you test for in a patient who you suspect might have Essential Thrombocythemia?

A

Philadelphia Chromosome
(CML)

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11
Q

What are the Major Criterion for diagnosing Essential Thrombocythemia?

A

Elevated Platelet Count
Bone Marrow Biopsy with Megakaryocytic Proliferation
Exclusion of Other Conditions
Presence of JAK2, CALR, or MPL

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12
Q

What is the Minor Criterion for Essential Thrombocytopenia?

A

Presence of a Clonal Marker
or
Absence of Evidence for Reactive Thrombocytosis

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13
Q

What Criterion must be met to make the diagnosis of Essential Thrombocythemia?

A

4 Major Criterion
or
First 3 Major Criterion + Minor Criterion

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14
Q

What is the main goal of treatment for a patient with Essential Thrombocythemia?

A

Reduce risk of Thrombosis and Hemorrhage

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15
Q

How do you treat Essential Thrombocythemia?

A

Hydroxyurea to maintain platelet count
Anagrelide (if anemia develops)
Aspirin

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16
Q

Erythrocytosis that causes overproduction of all three cell lines however, Red Blood Cells are predominant.

A

Polycythemia Vera

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17
Q

What acquired genetic mutation causes Polycythemia Vera?

A

JAK2

18
Q

What is the median age at presentation of Polycythemia Vera?

A

60

19
Q

What are the signs and symptoms of Polycythemia Vera?

A

Often Asymptomatic
Early Satiety and GI Discomfort
Pruritus following a warm shower or bath
- due to histamine release from basophilia
Bone Pain

20
Q

What are the clinical and physical exam findings of a patient with Polycythemia Vera?

A

Peptic Ulcer Disease - 4x more likely
Venous Engorgement (retinal veins)
Palpable Spleen

21
Q

What is a hallmark that is seen on a Complete Blood Count of a patient with Polycythemia Vera?

A

Hematocrit Over:
49% in Males
48% in Females
(at sea level)

22
Q

How do you confirm the diagnosis of Polycythemia Vera?

A

JAK 2 Mutation Screening

23
Q

What other two clinical findings may lead you towards the diagnosis of Polycythemia Vera?

A

Elevated B12
Hyperuricemia

24
Q

What are the Major Criterion of Polycythemia Vera?

A

Increased Red Blood Cell Volume
Bone Marrow Biopsy with Trilineage Growth
JAK 2 Mutation

25
Q

What are the Minor Criterion of Polycythemia Vera?

A

Low Serum EPO

26
Q

Which criterion must be met to make the diagnosis of Polycythemia Vera?

A

All 3 Major Criteria
or
First 2 Major Criteria + Minor Criteria

27
Q

What is the treatment for Polycythemia Vera?

A

Phlebotomy
Hydroxyurea
Aspirin
(avoid Iron supplementation)

28
Q

What is the median survival prognosis for patients with Polycythemia Vera?

A

15+ Years

29
Q

What is the number one cause of morbidity and mortality from Polycythemia Vera?

A

Arterial Thrombosis

30
Q

Iron overload and deposition disorder that affects the liver, pancreas, heart, adrenal glands, testes, pituitary, and kidneys.

A

Hemochromatosis

31
Q

What is the most common form of Hemochromatosis?

A

Autosomal Recessive Disorder

32
Q

What cells are defected and cause increased iron absorption in Hemochromatosis?

A

Duodenal Crypt Cells

33
Q

Who is Hemochromatosis most common in?

A

Men

34
Q

What is the classic triad of Hemochromatosis?

A

Cirrhosis
Bronze Skin
Type I Diabetes

35
Q

What comorbidities are associated with Hemochromatosis?

A

Hepatocellular Carcinoma
Intrahepatic Cholangiocarcinoma
Infection via Siderophilic Organisms

36
Q

Evaluation of a patient with Hemochromatosis might reveal with 3 things?

A

Elevated Plasma Iron
Low Unsaturated Iron Binding Capacity
Mildly Elevated AST and Alkaline Phosphatase

37
Q

Patients with what 4 diseases should be evaluated for Hemochromatosis?

A

Chronic Liver Disease
Erectile Dysfunction
Chondrocalcinosis (mineral deposits in cartilage)
Type I Diabetes (especially late onset)

38
Q

How do you treat Hemochromatosis?

A

Depletion of Iron stores via Phlebotomy
(takes 2 - 3 years of weekly phlebotomy)

39
Q

What can be used to decrease iron absorption and the need for maintenance phlebotomy?

A

Proton Pump Inhibitors
(PPI)

40
Q

What can be used as a chelating agent for patients with Hemochromatosis?

A

Deferoxamine

Clinical Medicine: Hematology and Oncology (8 decks)

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