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MDTI > Myeloproliferative Neoplasms > Flashcards

Myeloproliferative Neoplasms Flashcards

1
Q

Chronic Myeloproliferative Neoplasms

A

typically involve an expansion of mature, terminally differentiated cells. Characterized by too many mature cells in the blood.

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2
Q

CMNs are proliferative diseases of…

A

stem/progenitor cells

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3
Q

All CMNs are characterized by what type of mutation?

A

tyrosine kinase dysregulation

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4
Q

CMNs are predisposed to transformation into what?

A

acute leukemia

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5
Q

how are CMNs identified?

A

asymptomatic patients with abnormal blood counts

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6
Q

consequence of CMNs on bone marrow cavity

A

cause fibrosis and therefore lead to extra medullary sites of hematopoiesis (splenomegaly & hepatomegaly)

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7
Q

are CMNs curable?

A

no, indolent growth prevents sensitivity to current therapy

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8
Q

another name for chronic myelogenous leukemia (CML)

A

BCR-ABL positive MPN

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9
Q

CML

A

abnormal growth and proliferation of white (myeloid) blood cells. Neutrophils initially accumulate, followed by basophils and eosinophils.

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10
Q

Chronic phase of CML

A

least severe phase. characterized by presence of differentiation in peripheral blood smear and hyper cellular marrow biopsy. often no symptoms, 4-6yrs

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11
Q

epidemiology of CML

A

rare, increases with age, majority diagnosed at chronic phase, so rarely proceeds to AML

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12
Q

sign/symptoms of CML

A

most commonly asymptomatic, fatigue, weight loss, splenomegaly

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13
Q

lab findings of CML

A

high WBC, left shift, basophilia, high LDH & B12

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14
Q

accelerated phase CML

A

increased blasts and platelets. genetic evolution, increased symptoms, drop in healthy blood cells. 1yr

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15
Q

blast crisis of CML

A

3-6mos survival, blasts>20%, resistant to chemo, speed of growth resembles acute leukemia. More likely to be AML, but 1/3 are ALL

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16
Q

molecular basis of CML

A

t(9;22) Philadelphia chromosome. creates fusion gene BCR/ABL. ABL is tyrosine kinase that is over activated, leading to abnormal proliferation and genetic instability

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17
Q

treatment for CML

A

Imatinib (Gleevec)

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18
Q

Imatinib

A

kinase inhibitor. competitive inhibitor for ATP binding site. leads to death of neoplastic cells.

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19
Q

ways to monitor CML

A

WBC count, Cytogenic studies (karyotype), FISH, PCR

20
Q

complete hematologic response (CHR)

A

normalized CBC and peripheral blood after CML treatment, has limited prognostic significance.

21
Q

Complete cytogenetic response

A

0% philadelphia+ cells after CML treatment. MOST PREDICTIVE OF SURVIVAL

22
Q

major molecular response (MMR)

A

1000x reduction in bcr-abl transcripts after CML treatment. presence at 1yr predicts 100% progression free survival at 5yrs!

23
Q

how can we tell if imatinib stops working?

A

increased WBC (hematologic relapse), increasing Ph+ cells (cytogenetic relapse), increased bcr-abr transcripts (molecular progression)

24
Q

why would imatinib stop working?

A

most commonly due to non-adherence. sometimes due to drug resistance

25
Q

what do we do when imatinib stops working

A

increase dose, switch to second or third generation tyrosine kinase inhibitors, bone marrow/stem cell transplantation

26
Q

what mutation is present in most classical philadelphia chromosome negative MPDs?

A

gain of function point mutation in JAK2

27
Q

polycythemia vera

A

elevation in red blood cell mass, somatic/acquired mutation of JAK2. up regulation of Bcl (antiapoptotic machinery)

28
Q

clinical features of polycythemia vera

A

high Hb, can be asymptomatic, clot, hyper metabolism, hyperviscositiy, erythromelalgia, bleeding, splenomegagly,

29
Q

differential diagnosis for erythrocytosis

A

congenital mutations, polycythemia vera, chronic hypoxia, ectopic EPO production, high oc=xygen affinity hemoglobinopathies

30
Q

clinical symptom of PV in the eye

A

congested retinal veins due to hyperviscosity

31
Q

at what hematocrit does hyper viscosity become a problem?

A

when it exceeds 55%, thrombotic complications become an issue

32
Q

treatment for polycythemia vera

A

therapeutic phlebotomy, aspirin, hydroxyurea (decreases level of all blood cells), interferon, JAK2 inhibitors

33
Q

diagnostic criteria for polycythemia vera

A

elevated red blood cell mass, presence of JAK 2 mutation. (bone marrow trilineage myeloproliferation, low serum EPO as minor criteria)

34
Q

essential thrombocytosis

A

JAK2 and Mpl mutations that causes elevation in platelets via megakaryocytosis in bone marrow

35
Q

clinical features of essential thrombocytosis

A

asymptomatic, clots, bleeding

36
Q

treatments for essential thrombocytosis

A

observation, aspirin, hydroxyurea, interferon

37
Q

primary myelofibrosis (PMF)

A

leukoerythroplastic smear and presence of fibrosis. most likely of the non Ph+ CMNs

38
Q

clinical features of PMF

A

anemia, abnormal blood counts, B symptoms (fatigue, weight loss, fever), splenomegaly, leukoerythroblastic peripheral blood smear, marked bone marrow fibrosis

39
Q

leukoerythroblastic blood smear

A

teardrop RBC forms, nucleated red blood cells, left shifted granulocytes, blasts

40
Q

differential diagnosis for leukoerythroblastic blood smear

A

metastatic disease, AIDS, granulomatous disease, myeloproliferative disorder (myelofibrosis), lipid storage disease

41
Q

bone marrow biopsy in primary myelofibrosis

A

abnormal megakaryocytic, intrasinusoidal hematopoiesis, osteosclerosis –> all of which lead to frequent fractures and bony pain

42
Q

spleen of primary myelofibrosis

A

extramedullary hematopoiesis

43
Q

treatment for PMF

A

observation, EPO transfusions, thalidomide, JAK2 inhibitors (ruxolitinib)

44
Q

only curative treatment for PMF

A

donor stem cell transplantation

45
Q

why is there bone marrow fibrosis in PMF?

A

reactive, polyclonal expansion of marrow fibroblasts in response to cytokines derived from both marrow monocytes and ineffective megakaryocyteopoiesis

MDTI (42 decks)

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