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Clin Med III - Heme/Onc > Myeloproliferative > Flashcards

Myeloproliferative Flashcards

1
Q

How are myeloproliferative syndromes generally characterized?

A

Too many blood cells

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2
Q

General definition of polycythemia vera (PCV)

A

Too many RBCs

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3
Q

General definition of essential thrombocytosis (ET)

A

Too many platelets

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4
Q

General definition of chronic myelogenous or chronic myeloid leukemia (CML)

A

Too many neutrophils

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5
Q

Define myelofibrosis

A

Too many RBCs, platelets and neutrophils with prominent immature RBCs

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6
Q

Describe myeloproliferative syndromes

A
  • Neoplastic blood cell disorders
  • Insidious onset w/vague symptoms
  • Splenomegaly
  • Cure NOT possible
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7
Q

Describe polycythemia vera

A

Elevated RBC mass a/w a mild increase in WBCs and platelets

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8
Q

Etiology of PCV

A

JAK2 gene mutations in 90% of cases

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9
Q

PE findings of PCV

A
  • Ruddy complexion

- Mild splenomegaly

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10
Q

Diagnostic tests of PCV

A
  • Total body plasma volume
  • Total body RBC volume or mass
  • Difficult to obtain in practice
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11
Q

Lab findings of PCV

A
  • Normal ABG
  • Normal Fe/TIBC
  • Normal or slightly high B12
  • Normal folate
  • Low serum erythropoietin
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12
Q

Treatment of PCV

A
  • Decrease Hct via phlebotomy until Fe deficient
  • AVOID chemo drugs (Hydrea)
  • Anagrelide can be used to decrease platelets
  • Ruxolitinib can decrease platelets/splenomegaly but may cause severe anemia (pt must be JAK2 positive to work)
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13
Q

What are the lab findings of a patient with well controlled PCV?

A
  • Normal (or near normal) Hct

- Low MCV

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14
Q

Why should chemo drugs such as Hydrea be avoided in PCV patients?

A

Increase the risk of secondary leukemias

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15
Q

How can Anagrelide be used to treat PCV?

A

Decreases platelet count

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16
Q

How can Ruxolitinib be used to treat PCV?

A
  • Decreases platelets and splenomegaly
  • May cause severe anemia
  • Only works if pt is JAK2 positive
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17
Q

WHO definition of Essential Thrombocytosis (ET)

A

Platelet count over 600,000 without other etiologies (Fe deficiency, co-existent infection/inflamm process, bleeding, other myeloproliferative disorder)

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18
Q

Clinical presentation of ET

A
  • MC in females
  • HA, easy bruising/bleeding
  • PE usually normal (mild splenomegaly may be present, but massive splenomegaly should suggest another diagnosis)
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19
Q

Lab findings of ET

A
  • Normal Hgb and Hct
  • Normal or slightly high WBCs (normal diff)
  • Normal Fe/TIBC
  • High platelets w/giant plts
  • JAK2 V617F present
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20
Q

How can elevated platelets affect chem labs of a patient?

A

Increased K - released from platelets when clot forms in serum tube

21
Q

How can elevated platelets affect ABG results?

A

Decreased pO2 - due to hypermetabolic state in ABG syringe if not quickly cooled on ice

22
Q

Treatment of ET

A
  • Conservative and symptom based (lower platelets until pt is asymp)
  • Anagrelide or Hydrea are mainstays
23
Q

What is CML characterized by?

A
  • High WBC count w/immature blood cells normally seen in BM but now in peripheral blood
  • Thrombocytosis and anemia are common
  • Basophilia is almost diagnostic
24
Q

What is the most significant factor of CML?

A

Philadelphia chromosome creating uncontrolled WBC production

25
Q

Lab findings of CML

A
  • Normal ESR
  • Low leukocyte alkaline phosphatase
  • Bands, meylocytes on smear
  • Hypercellular BM w/less than 5% blasts
26
Q

Phases of CML

A
  1. Chronic (modest symptoms)
  2. Accelerated
  3. Blast Crisis (clinical picture looks like acute leukemia)
27
Q

Treatment of CML

A
  • Refer to hematology
  • Goal is complete molecular remission
  • Imatinib (oral TKI, minimal side effects)
28
Q

What is imatinib used to treat?

A

CML

29
Q

How does imatinib work?

A

Blocks ATP binding site of ABL kinase

30
Q

Characteristics of myelofibrosis w/myeloid metaplasia (MMM)

A
  • Increased marrow reticulum
  • Increased marrow fibrosis
  • Extramedullary hematopoiesis
  • Splenomegaly
31
Q

Why is early MMM sometimes difficult to distinguish from other myeloproliferative syndromes?

A

Increased marrow reticulum can be seen in most other myeloproliferative syndromes

32
Q

Classic lab finding of MMM

A

Leuko-erythroblastic peripheral smear (showing immature RBCs and WBCs)

33
Q

Treatment of MMM

A

Mainly supportive

  • Anemia treated w/transfusion
  • Splenectomy
  • Hydrea or Ruxolitinib to control hypermetabolic symptoms
  • BM transplant potentially curative
34
Q

Poor prognosis factors for MMM

A
  • Over 65 yo
  • WBC over 25,000
  • Blasts over 10%
  • Constitutional symptoms
  • Hgb less than 10
35
Q

What s/s are NOT seen in myelodysplastic syndromes (MDS)?

A

Splenomegaly and extramedullary hematopoiesis

36
Q

MDS is characterized by?

A
  • Cytopenias (usually of more than 1 cell line)

- Often a chromosomal abnormality but NOT always

37
Q

What is aplastic anemia characterized by?

A

Pancytopenia a/w bone marrow hypoplasia

38
Q

Treatment of aplastic anemia

A
  • Supportive until definitive treatment planned by heme
  • Transfusion
  • BM transplant potentially curative
39
Q

Describe pure red cell aplasia (PRCA)

A
  • Very rare
  • Only RBC precursors affected
  • Etiologies and associated diseases are similar to aplastic anemia
  • Treatment is immunosuppression, supportive, sometimes anti-virals
40
Q

Who is affected by MDS?

A

Primarily older adults (over 70)

NOT rare

41
Q

Splenomegaly suggests MDS OR MPD?

A

MPD

42
Q

Clinical presentation of MDS

A
  • Insidious onset of fatigue
  • SOB
  • Possibly angina
43
Q

Typical lab findings of MDS

A
  • Macrocytosis and abnormally formed RBCs/WBCs

- Hypercellular BM w/dysplastic changes

44
Q

Lab finding that indicates better prognosis for MDS

A

Ringed sideroblasts

45
Q

Lab finding that indicates worse prognosis for MDS

A

Increased percentage of blasts

46
Q

What does RAEB-2 indicate in MDS?

A

Poor prognosis - survival less than 6 months

47
Q

Treatment of MDS

A
  • Mainly supportive

- BM transplant for younger patients

48
Q

What type of treatment should be avoided in MPS if possible?

A

Chemo

Clin Med III - Heme/Onc (8 decks)

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