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Year 2 Heme lymph > myeloid, WBC III, Putthoff, lec > Flashcards

myeloid, WBC III, Putthoff, lec Flashcards

1
Q

myeloid neoplasms come from what

A

hematopoietic progenitor cells

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2
Q

how do you Dx a myeloid neoplasm

A

bone marrow, at least 20% blasts

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3
Q

3 categories of myeloid neoplasms

A

acute myeloid leukemia
myelodysplastic syndromes
myeloproliferative disorders

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4
Q

what type of cancer is from immature blasts in bone marrow that suppress normal hematopoiesis

A

acute myeloid leukemia

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5
Q

defective maturation of myeloid progenitors leading to ineffective hematopoiesis and cytopenia

A

myelodysplastic syndrome

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6
Q

what is characteristic of myeloproliferative disorders

A

increase production in 1+ types of blood cells

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7
Q

what is CD34 on

A

multipotent stem cell

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8
Q

what is CD64 indicative of

A

mature myeloid cell

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9
Q

what is CD33

A

immature myeloid cell

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10
Q

what genes are commonly affected in AML

A

RUNX1 and CBFB genes that are needed for hemtopoiesis

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11
Q

what are the WHO categories of AML

A

I AML with genetic changes
II AML with MDS-like features
III AML therapy related
IV AML not otherwise specified

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12
Q

what are the AML not otherwise specificed classifications

A 
M0 minimally differentiated
M1 AML w/o maturation
M2 AML w/ myelocytic maturation
M3 Acute promyelocytic leukemia
M4 AML w/ myelomonocytic maturation
M5 AML w/ monocytic maturation
M6 AML w/ erythroid maturation
M7 AML w/ megakaryocyte maturation
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13
Q

what are the common genetic mutations of AML

A

t 8;21
t 15;17
t 11q23;V
inv (16)

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14
Q

majority of AML is what stage and what is assoc genetic mutation

A

M2 AML with myelocytic maturation

t8;21

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15
Q

which AML is associated with DIC? stage? age of occurrence?

A

Acute promyelocytic leukemia stage M3

age 35-40

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16
Q

What AML is peroxidase + and non specific esterase +

A

AML with myelomonocytic maturation class M4

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17
Q

nonspecific esterase + in AML means what

A

from monoblast

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18
Q

if auer rods are seen in AML then can tell arised from what lineage

A

myeloblast

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19
Q

which AML has platelet specific Ag to vWF or GpIIb/IIIa

A

the megakaryocyte M7

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20
Q

clincal features o fAML

A

anemia, neutropenia, thrombocytopenia, fatigue, infecitonm bleeding, mucosal bleeding and opportunistic infections

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21
Q

what is leukemia cutis and when is it seen

A

skin infiltration of tumor on cutaneous skin

seen in monocytic AML

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22
Q

what should you do is suspect AML

A

karyotyping because 90% associ with abberations

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23
Q

Myelodysplastic syndrome patients are at high risk in develping what

A

AML

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24
Q

what are types of myelodysplastic syndromes

A

primary idiotpathic and secondary genotoxic

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25
Q

what are Sx of idiopathic myelodysplastic syndrome

A

weakness, infections, hemorrhages

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26
Q

how do you Dx myelodysplastic syndromes

A

bloos mear

bone marrow and cytologic studies

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27
Q

what type of myelodysplastic syndrome is more aggressive and what is onset timing and survival

A

genotoxic
2-8 yrs post exposure
4-8 mo survival

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28
Q

age onset myelodysplastic syndrome

A

around 70

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29
Q

what will histo look like in myelodysplastic syndromes

A

multilobated nuclei of RBC progenitors
ringed sideroblasts, iron laden macrophages
pseudo pelger huet cells (neutrophils with 2 lobes)
megakaryocytes with multiple nuclei

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30
Q

what will you see in peripheral blood in myelodysplastic syndromes

A

pancytopenia

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31
Q

what is Tx for young patient with myelodysplastic syndrome? older?

A

young: hematopoietic stem cell transplant
older: suppportive, thalidomide like drugs for 5q variants and DNa methylation inhibitors

32
Q

what are mutations associated with myelodysplastic syndromes

A

monosomies 5 and 7
deletions: 5q 7q 20q
trisomy 8– MYC

33
Q

what mutations occur in myeloproliferative disorders

A

mutated constitutively activates tyrosine kinase

34
Q

What is mutation in CML

A

chimeric BCR-ABL, 9;22

35
Q

how to Dx CML

A

fluorescence in situ hybridization or PCR based

leukocytosis >100,000, low LAP

36
Q

CML preferentially drives what cell lines

A

granulocytic, megakaryocytic progenitors

37
Q

what is distribution of CML

A

50 y.o M>F

38
Q

clnical features CML

A

insidious onset: fatigue, weakness, weight loos, anorexia, abdmonial fullness or pain
massive splenomegaly

39
Q

what shoul dyou suspect in CML patient with severe LUQ pain

A

splenic infarct

40
Q

histo of CML looks how

A

hypercellularity

Macrophages are wrinkled with blue/green cytoplasm “sea blue” histiocytes

41
Q

what is prognosis CML

A

3 yr w/o Tx

42
Q

What occurs within first year of CML

A

blast crisis and accelerated phase

43
Q

what is Tx CML

A

BCR-ABL inhibitors

young- stem cell transplant 75% curable

44
Q

If LAP measured and is high what do you suspect

A

reactive (infection

45
Q

how come myeloproliferative disorders lead to cytopenias

A

the bone marrow is exhausted and gives up

46
Q

What occurs in Polycythemia vera

A

JAK2 point mutation that is activated
dec need for erythropoeitin
increase RBC, granulocytes and platelets

47
Q

what is the point mutation in PCV

A

JAK2 valine- phenlyalanine 600

48
Q

what age group affected PCV and Sx

A

late middle age

insidious: plethora, cyanosis, intense pruritis, HA, HTN, GI ulceration, splenomegaly, hyperuricemia

49
Q

What will CBC show for PCV

A

increase Hct increase RBC increase platelets

basophilia

50
Q

what accounts for pruritis in PCV

A

basophilia

51
Q

what is Tx for PCV

A

phlebotomy inc surivval 10 yrs

52
Q

what is first sign of PCV in many patients

A

DVt or MI or stroke

53
Q

what is spent stage of PCV

A

marrow fibrosis and splenomegaly because bone marrow exhausted

54
Q

What is Essential thrombocytosis

A

thrombocytosis without PCV

55
Q

mutaiton in ET

A

JAK2 or MPL increasing platelet count and megakaryocytes

56
Q

how Dx ET

A

bone marrow biopsy and Dx of exclusion

57
Q

what must you rule out for ET

A

reactive procress (inflammation or infeciton)

58
Q

what are Sx and age onset ET

A

adults>60
insidious
erthromelalgia

59
Q

what is erythromelalgia

A

throbbing and burning of hands and feet from occlusion of small arterioles by platelet aggregates

60
Q

What is Tx for ET

A

chemo

survival 12-15 yr

61
Q

What markers are on Langerhans cell histiocytosis

A

S100+ CD1a+ HLA-DR+

62
Q

histo of Langerhans cell histiocytosis

A

vesicular nuclei with linear grooves or folds & vacuolated cytoplasm
birbeck granules in cytoplasm (langerin in granules)

63
Q

what is mutation in Langerhans cell histiocytosis

A

valine to glutamate at 600 BRAF

also have TP53 RAS and MET

64
Q

what are the categories of Langerhans cell histiocytosis

A

multifocal/multisystemic Langerhans cell histiocytosis
unifocal and multifocal unisystem Langerhans cell histiocytosis
pulmonary Langerhans cell histiocytosis

65
Q

what are Sx of mutlifocal multisystemic Langerhans cell histiocytosis

A

cutaneous lesions like seborrheic eruption

HSM, lymphadenopathy, pulm lesions, bones lesions, anemia, throbocytopenia and recurrent infecitons

66
Q

age Dx multifocal multisystem Langerhans cell histiocytosis

A

before 2 y.o

sometimes adult

67
Q

what is Tx for multifocal mulisystemic Langerhans cell histiocytosis

A

50% survive 5 yrs

68
Q

what do you see in blood fo unifocal and multifocal unisystem Langerhans cell histiocytosis

A

langerhans + eos+ plasma cells + lymphocytes + neutrophils

69
Q

how does unifocal Langerhans cell histiocytosis present

A

skel system in older children like ribs, skull and femur

70
Q

how does multifocal Langerhans cell histiocytosis present

A

multiple erosive bony masses in young children, 50% have DI because involvement of posterior pituitary stlak

71
Q

What is Hans Schuller christian triad and assoc with?

A

calvarial defects + DI + exopthalmus

assoc with multifocal unisystem Langerhans cell histiocytosis

72
Q

Tx for unisystem Langerhans cell histiocytosis

A

unifocal is resection or irradiation

multifocal is chemo

73
Q

what is pulmonary Langerhans cell histiocytosis

A

b/l interstitial lung disease in middle and upper zones

smoking

74
Q

What are normal markers on langerhans cells

A

CCR6

75
Q

neoplastic markers on Langerhans cells

A

CCR6 AND CCR7!

Year 2 Heme lymph (28 decks)

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