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Heme > Myeloproliferative Disorders > Flashcards

Myeloproliferative Disorders Flashcards

1
Q

Pathyphysiology of Myeloproliverative Disorders?

A

overproduction of cells in the bone marrow

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2
Q

What are the four main types of myeloproliferative disorders?

A
  • chronic myelogenous leukemia (CML)
  • polycythemia vera
  • myelofibrosis
  • Essential thrombocytosis
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3
Q

Polycythemia is mainly characterized by what?

A

elevated red cell mass, Hgb, & HCT

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4
Q

What are the 3 main causes of polycythemia?

A
  • appropriateely elevated erythropoietin: hypoxemia
  • Inappropriately elevated EPO: renal cell carcinoma, hepatocellular carcinoma
  • germline and somatic mutation: polycythemia vera
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5
Q

Polycythemia vera is most commmon among who?

A

most frequently found in men over age 60

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6
Q

Polycythemia vera sx

A

very non-specific

  • HA
  • Weakness
  • Dizziness
  • Excessive Sweating
  • pruritus **hallmark is itching post bathing
  • erythromelalgia: burning pain in feet and/or hands often with pallor, erythema, or cyanosis
  • may have palpable pulses

-sx may also be related to thrombosis; aterial and venous thrombosis, transient visual disturbances (amaurosis fugax, scintillating scotomata)

  • GI sx:
  • -epigastric discomfort
  • -hx of peptic ulcer disease
  • gastroduodenal erosions
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7
Q

Lab abnormalities in polycythemia

A

polycythemia, thrombocytosis, leukocytosis and others.

Hgb/HCT > 18.5/56 men
Hgb/HCT > 16.5/50 women
WBC >10.5
Platelets >450K

elevated serum lactate dehydrogenase in 50%

JAK2 mutation in 98%

abnormal low serum EPO in 81%

Hypercellular bone marrow

iron storage in bone marrow is absent (b/c its being consumed in making RBC)

low EPO

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8
Q

Polycythemia aka?

A

erythrocytosis

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9
Q

Physical exam findings in polycythemia vera?

A
  • splenomegaly
  • facial plethora (ruddy cyanosis; reddish complexion)
  • hepatomegaly
  • injection of conjunctival small vessels &/or engorged veins of the optic fundus
  • excoriation of skin (from pruritus)
  • gouty arthritis
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10
Q

Polycythemia Vera Tx

A

-targeted at lowering HCT and decreasing thrombosis

  • phlebotomy to keep HCT 60 or prior thrombotic episode)
  • -Interferon alpha: use instead of hydroxyurea in women of childbearing age or if hydroxyurea is ineffective.
  • pruritus: Antihistamines, H2 blockers, myelosuppression, interferon alpha, antidepressants (paxil, fluoxetine), photo chemotherapy
  • hyperuricemia(gout): allopurinol for prevention
  • erythromelagia: aspirin 75-100mg
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11
Q

Polycythemia vera can progress into much more serious disorders of bone marrow metabolism such as:

A

myelofibrosis

acute leukemia

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12
Q

Myelofibrosis, what is this?

A

where bone marrow is replaced by connective tissue.

unknown etiology,

**hematopoiesis will occur extramedullary to compensate

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13
Q

Myelofibrosis occurs among who most commonly?

A
  • primarily middle aged and elderly patients (67)

- more often in men than women

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14
Q

Major causes of morbidity and mortality of myelofibrosis

A
  • progression to acute leukemia
  • infection
  • bleeding
  • portal HTN
  • vascular complications
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15
Q

Clinical Manifestations of Myelofibrosis

A

most commonly severe fatigue and LUQ

  • splenomegaly(90%): LUQ pain(w/ or w/o radiation to left shoulder) and early satiety
  • hepatomegaly(40-70%): RUQ pain, early satiety, ascites, esophagearl and gastric varices, GI bleeding, hepatic encephalopathy, portal vein thrombosis, portal HTN
  • weight loss
  • low grade fever, nigh sweats
  • asymptomatic (15-30%)
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16
Q

How might extramedullary hematopoiesis present and why?

A
  • may present as splenomegaly, hepatomegaly, lymphadenopathy, pleural effusions, pericardial effusions, abdominal effusion, GI, GU, or respiratory (dyspnea)
  • production of RBC occurs outside the bone marrow in these areas.
17
Q

What does peripheral blood smear of Primary Myelofibrosis look like?

A
  • marked poikilocytosis
  • tear drop poikilocytes
  • anisocytosis (RBC of unequal size)
  • myelocyte (bone marrow cell)
18
Q

Bone marrow biopsy and blood results of Myelofibrosis

A

blood and bone marrow at the early stage: high white count w/ a left shift, and a hypercellular marrow.

as disease progresses, the marrow becomes replaced with fibrous tissue.

19
Q

Myelofibrosis Lab abnormalities

A

depends upon stage of disease

  • WBC can be low or high
  • Platelets can be low or high
  • Elevated alkaline phosphatase (d/t liver or bone diseases)
  • elevated lactate dehydrogenasee (d/t ineffective hematopoiesis)
  • elevated uric acid (d/t enhanced turn over of hematopoietic tissue)
  • Vit B12 (d/t increased neutrophil mass)
  • may have JAK2 mutation (for regulation of the production of cells in be bone marrow)
20
Q

Diagnosis of Myelofibrosis

A
  • bone marrow fibrosis
  • absence of philadelphia chromosome
  • splenomegaly
  • Tear drop erythrocytes
  • circulating myeloid cells
  • circulating erythroblasts
  • clusters of megakaryoblasts
21
Q

Tx for Myelofibrosis

A
  • stem cell transplant
  • hydroxyurea
  • chemotherapeutic agents
  • JAKK2 inhibitors: urxolitinib
  • splenectomy but may cause worsening hepatomegaly
  • radiation therapy
22
Q

What is thrombocytosis?

A

overproduction of platelets, these platelets may be abnormal and nonfunction therefore it may lead to thrombosis or bleeding.

*elevated platelet count without other blood cell abnormalities

23
Q

Who does thrombocytosis most commonly occur in?

A

Women over the age of 50

24
Q

Thrombocytosis:

  • are platelets affected?
  • are WBC affected?
  • what does a peripheral blood smear look like?
  • what does bone marrow biopsy reveal?
  • are there any JAK2 mutations?
A
  • platelet counts markedly elevated
  • WBC may be slightly elevated
  • peripheral smear shows normal RBC morphology
  • Bone marrow biopsy reveals increased numbers of megakaryocytes but otherwise normal
  • high frequecy of JAK2 mutaitons
25
Q

Thrombocytosis Sx

A
  • HA
  • lightheadedness
  • visual changes
  • numbness, tingling or burning in the feet
  • splenomegaly
  • hx of thrombosis
26
Q

WHere is thrombosis most likely to occur?

A

hands feet, brain, but may occur anywhere

venous thrombosis: mesenteric, hepatic, and portal vein, as well as deep vein thrombosis

27
Q

Tx of Thrombocytosis

A
  • low dose aspirin

- hydroxyurea (keep platelet count below 500,000)

28
Q

Thrombocytosis may progress to what other diseases?

A
  • fibrotic bone marrow
  • massive splenomegaly
  • myelofibrosis
  • 1-2% risk of acute myelogenous leukemia over 20years

Heme (13 decks)

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