Myeloproliferative Disorders Flashcards

1
Q

Basophilia means

A

CML

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2
Q

What does basophilia mean

A

CML

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3
Q

Basophilia means what

A

CML

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4
Q

Why is there basophilia in CML

A

CML results in proliferation of all myeloid lineage cells
including basophils
this is the only real cause we know of that causes basophilia

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5
Q

Myeloproliferative disorders may lead to what complications

A

hyperuricemia and gout

marrow fibrosis

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6
Q

CML translocation

A

t(9;22) Philadelphia chromosome

*also seen in bad form of B-ALL

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7
Q

In which cell in the myeloid lineage is the mutation driving CML

A

in hematopoietic stem cell

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8
Q

CML may progress to

A

AML or ALL
*may progress to either because driving mutation is in the hematopoietic stem cell and not a more mature myeloid progenitor cell

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9
Q

t(9;22)
pathology
disease
treatment

A

BCR-ABL fusion: constant activation of tyrosine kinase
CML and some B-ALL
imatinib is Tx, inhibits TK activity

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10
Q

CML stages

A

chronic: steady
accelerated: on its way to acute
transformation: acute leukemia, either AML or ALL

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11
Q

CML moving into accelerated or acute

may be marked by

A

hepatosplenomegaly

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12
Q

Leukocyte alkaline phosphatase (LAP) stain

usefulness in CML

A

CML are LAP -

LAP commonly used in leukemoid reaction (rxn to infection) to help destroy infection

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13
Q

CML labs:
LAP stain
WBC w/ diff
cytogenetics

A

LAP negative
basophilia
t(9;22): Ph+, BCR-ABL TK mutation

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14
Q

Polycythemia vera

driving mutation

A

JAK2 kinase mutation

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15
Q

Polycythemia vera

presentation

A
*hyperviscocity Sx
blurry vision and headache
venous thrombosis, ie Budd-Chiari
flushed face
itching, especially after bathing
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16
Q

Why is there itching after bathing in polycythemia vera

A

release of histamine from mast cells

17
Q

Tx of polycythemia vera

A

phelbotomy

hydroxyurea (2nd line)

18
Q

Polycythemia
EPO
SaO2

A

low EPO

normal SaO2

19
Q

Reactive polycythemia to low O2
EPO
SaO2

A

high EPO

low SaO2

20
Q

Ectopic EPO production
cancer
EPO
SaO2

A

renal cell carcinoma
high EPO
normal SaO2

21
Q

Driving mutation in essential thrombocythemia

A

JAK2 kinase mutation

22
Q

Sx of essential thrombocythemia

A

bleeding and/or thombosis

platelets either hyperfunction of not at all really

23
Q

Which myeloproliferative disorder does not have an increased risk for gout

A

essential thrombocythemia
because platelets are derived from megakaryocytes
don’t have their own nuclei

24
Q

Myelofibrosis caused by proliferation of

A

megakaryocytes

PDGF (platelet-derived growth factor) release