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IHO Week 4 > CML Module - Krafts > Flashcards

CML Module - Krafts Flashcards

1
Q

What is CML?

A

Chronic Myeloproliferative Leukemia

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2
Q

What type of cells are found in the chronic leukemias?

A

Mature Cells with a WBC Left Shift of Immature Cells

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3
Q

Define Chronic Myeloproliferative Disorders

A

Malignant proliferation of myeloid cells (not blasts, but maturing cells) in blood and bone marrow

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4
Q

What are a couple of characteristics of Chronic Myeloproliferative Disorders?

A

ONLY in Adults
LONG course
- Can potentially live years without treatment

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5
Q

What is PV?

A

Polycythemia Vera

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6
Q

What is ET?

A

Essential Thrombocythemia

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7
Q

What is MF?

A

Myelofibrosis

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8
Q

What cells are proliferating most in CML?

A

Neutrophils

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9
Q

What cells are proliferating most in PV?

A

Red Cells

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10
Q

What cells are proliferating most in ET?

A

Platelets

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11
Q

What cells are proliferating most in MF?

A

Everything

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12
Q

What clinical features are associated with Chronic Myeloproliferative Disorders?

A

Hypercellular Marrow
Large Spleen (no room in marrow for hematopoiesis)
Evolves to Acute Leukemia (more than 20% blasts)
Mutated Tyrosine Kinases

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13
Q

What mutation is associated with CML?

A

Philadelphia chromosome translocation t(9;22)

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14
Q

What cell types are associated with CML?

A

Neutrophilic Leukocytosis and Basophilia

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15
Q

What would the lab findings be in CML?

A 
HIGH WBC
Neutrophilia with Left Shift
Basophilia
Low Hemoglobin
Low LAP
Increased Platelet Count (at first)
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16
Q

What are the symptoms of CML?

A

Slow onset
Fever, Fatigue, Night sweats
Abdominal Fullness

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17
Q

What is one of the key clinical signs of CML?

A

Organomegaly
Huge Spleen
Big Liver

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18
Q

What are the 3 phases of CML?

A

Chronic Phase
Accelerated Phase
Blast Crisis

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19
Q

Describe the Chronic Phase of CML

A

Stable counts
Easily controlled
Live for 3-4 years untreated

20
Q

Describe the Accelerated Phase of CML

A

Unstable counts

Blast crisis within 6-12 months

21
Q

Describe the Blast Crisis of CML

A

Acute Leukemia
High Mortality
50% of patients will go directly to the Blast Crisis and not enter the Accelerated Phase

22
Q

What are the 3 types of remission in CML?

A

Hematologic Remission
Cytogenetic Remission
Molecular Remission

23
Q

Describe Hematologic Remission

A

No splenomegaly

WBC

24
Q

Describe Cytogenetic Remission

A

No metaphases with t(9;22)

25
Q

Describe Molecular Remission

A

No BCR/ABL transcripts by PCR

26
Q

What makes the blood sludgy in Polycythemia Vera?

A

High RBC Count

27
Q

What mutation is associated with Polycythemia Vera?

A

JAK-2 mutation causing the cells to grow on their own

28
Q

What is the issue in Primary Polycythemia Vera?

A

Intrinsic Myeloid Cell Problem

29
Q

What is the issue in Secondary Polycythemia Vera?

A

Due to increased erythropoietin

30
Q

What are the clinical signs of Polycythemia Vera?

A

Headache, Pruritis, Dizziness
Thrombosis
Infarction

31
Q

What are the clinical symptoms of Polycythemia Vera?

A

Organomegaly (spleen and liver)

Plethora (flushing)

32
Q

What is the treatment for Polycythemia Vera?

A

Phlebotomy

Possibly Myelosuppressive drugs

33
Q

What is the prognosis for Polycythemia Vera?

A

Median Survival with death occurring from thrombosis or hemorrhage

34
Q

In what patients can Essential Thrombocythemia be seen?

A

Young Women

35
Q

What is the diagnostic criteria for Essential Thrombocythemia?

A

Platelet count over 600,000 (usually a million)
Hemoglobin of less than 13
Normal RBC Mass

None of the following:
Philadelphia Chromosome
Marrow Fibrosis

36
Q

What are the clinical symptoms of Essential Thrombocythemia?

A

Bleeding

Thrombosis

37
Q

What are the clinical signs of Essential Throbocythemia?

A 
Purpura
Bruising
Pallor
Tachycardia
Enlarged Spleen
38
Q

What is the treatment of Essential Thrombocythemia?

A

Platelet Pheresis
Myelosuppressive Drugs
Aspirin

39
Q

What is the prognosis of Essential Thrombocythemia?

A

Median Survival with death occurring from thrombosis or hemorrhage

40
Q

What chronic myeloproliferative disorders can have spontaneous leukemia transformation rarely in some patients?

A

Polycythemia Vera
Essential Thrombocythemia
Chronic Myelofibrosis

41
Q

What defines Chronic Myelofibrosis?

A

Panmyelosis (all of the myeloid cell lines are proliferating) with Marrow Fibrosis
Extramedullary Hematopoiesis
Teardrop RBC

42
Q

What are the clinical symptoms in Chronic Myelofibrosis?

A

Upper Left Quadrant Fullness

Weakness, Fatigue, Palpitations

43
Q

What are the clinical signs in Chronic Myelofibrosis?

A

HUGE spleen
Pallor
Tachycardia

44
Q

What is the treatment of Chronic Myelofibrosis?

A

Supportive

Myelosuppressive Drugs early on

45
Q

What is the prognosis of Chronic Myelofibrosis?

A

Median Survival with death from bone marrow failure

IHO Week 4 (15 decks)

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