Lecture 6 - Myeloid probs

Question 1

acute vs chronic leukemias:
acute leukemias are made of mostly _____.
chronic leukemias contain both ____ and ____ cells.
most common condition in children?
most common condition in adults?

Answer 1

immature blasts;
immature, mature;
ALL;
AML

Question 2

symptoms of erythromelalagia: burning sensation and redness of ____ and ____. seen in what disorder?

Answer 2

hands, feet;

essential thrombocytosis

Question 3

essential thrombocytosis is characterized by increased _____. symptoms include ____ or _____

Answer 3

platelets;

thrombosis, bleeding

Question 4

essential thrombocytosis:

mutations in ____, ___, or ______ are characteristic

Answer 4

JAK, c-MPL, Cal-R

Question 5

myeloproliferative disorders are characterized by increased numbers of ____ blood cells

Answer 5

mature

Question 6

CML is characterized by the _____ chromosome, a _____ translocation that causes a constitutively active ___ ____

Answer 6

philadelphia;
9, 22;
tyrosine kinase

Question 7

CML:
characterized by massive ____ and _____ stem cell proliferation, leading to elevated WBC with all _____ precursors in the peripheral blood.

Answer 7

splenomegaly, myeloid;

granulocyte

Question 8

CML:
notably, what granulocyte shows increased numbers (rarely seen in other disorders)?
does CML cause leukostasis?

Answer 8

basophils;

no

Question 9

CML:

stages include a _____ phase, a ____ phase, a “____ ____”, and eventual progression to _____

Answer 9

chronic, acceleration, blast crisis;

AML OR ALL

Question 10

treatment of CML?

what does it do?

Answer 10

imatinib;

tyrosine kinase inhibitor

Question 11

polycythemia vera almost always is characterized by a V617F ____ mutation. RBC levels are ____. EPO levels are _____

Answer 11

JAK2 (causes increased sensitivity to cytokines); elevated, decreased

Question 12

polycythemia vera:
classic symptom (although rarely seen):
usual death from _____

Answer 12

pruritis after bathing;
thrombosis

also DVT, ruddy complexion

Question 13

_____ is characterized by bone marrow obliteration with fibrosis. symptoms include massive _____ and extreme _____

Answer 13

myelofibrosis;
splenomegaly, fatigue

teardrop cells

Question 14

the pseudo-pelger-huet anomaly is seen in what condition? what is it?

Answer 14

myelodysplastic syndromes;

bilobed nuclei

Question 15

myelodysplastic syndromes:
bone marrow is characterized as _____.
____penia is common

Answer 15

HYPERcellcular (with dysplastic cells);

pancyto

Question 16

myelodysplastic syndromes:

clonal disorder where abnormal clone has ____ maturation. this ______ normal hematopoeisis. may progress to ____

Answer 16

ineffective;
suppresses;
AML

Question 17

myelodysplastic syndrome:
what chemical exposure can cause it?
cytogenetics: _____ has a good prognosis. ____ has a poor prognosis

Answer 17

benzene;
5q abnormality;
monosomy 7.

Question 18

Auer rods are characteristic of what disease? ____ positive inclusions are also seen

Answer 18

AML;

MPO

Question 19

gingival hyperplasia is characteristic of what kind of AML?

what about DIC?

Answer 19

monocytic form;

APL

Question 20

APL:
characterized by involvement the ______ receptor alpha. this stops maturation of the cell at the ____ stage. what is the treatment? in relapsed patients, use _____

Answer 20

retinioic acid;
promyelocyte;
all-trans retinoic acid;
arsenic trioxide

Question 21

aplastic anemia:
what antibiotic is classically associated with it?
what 3 viruses are?

Answer 21

chloramphenicol;

HIV, hepatitis, parvovirus B19

Question 22

aplastic anemia:

____openia. bone marrow is ____. caused by ___ cell injury

Answer 22

pancyto; HYPOcellular; stem

Question 23

aplastic anemia is classically associated with what other type of anemia?

Answer 23

paroxysmal nocturnal hemoglobinuria

Question 24

what genetic disorder can cause aplastic anemia?

due to a ____ ____ defect causing bm failure

Answer 24

fanconi anemia;

DNA repair

Question 25

aplastic anemia:

______ and ______ are typically not seen!

Answer 25

lymphadenopathy, organomegaly