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Flashcards in Tuesday Groupings/Definitions/Presentations Deck (67):
1

What are the 4 myeloproliferative neoplasms we know?

CML
PV
PMF
ET

2

What are the categories of myeloid neoplasms?

MPN
MDS
AML

3

What is MPN (myeloproliferative neoplasm)?

clonal hematopoietic stem cell disorders
proliferation of one of more of the myeloid lineages

4

What is similar and different about MPN vs MDS?

similar: hypercellular BM
different: MPN-cytoses, increased cell proliferation, aka effective hematopoiesis, MDS-cytopeniass, increased cell death, aka ineffective hematopoeisis

5

What is MDS (myelodysplastic syndrome)?

clonal hematopoietic stem cell disorder
cytopenias, dysplasia, ineffective hematopoiesis

6

What does MPN have the potential to develop to?

BM fibrosis or acute leukemia

7

What does MDS hav the potential to develop to?

AML (30% of cases)

8

What percentage of blasts are found in MDS?

<20%

9

What kills with MDS?

infection or bleeding

10

Median survival of MDS?

9-29mo

11

What is acute leukemia?

neoplastic proliferation of immature cells (blasts) recapitulating progenitor cells of the hematopoietic system

12

Acute vs Chronic leukemias

acute-immature cells, untreated live weeks to months
chronic-mature cells, untreated live months to years

13

AML vs ALL

AML-adults
ALL-kiddos

14

What is AML (acute myeloid leukemia)?

heterogenous, poor outcome
systemic neoplasms of myeloid progenitor cells
>20% blasts in the blood or marrow

15

AML types and prognoses

AML w/recurrent cytogenic abnormalities-favorable
AML w/myelodysplasia associated chagnes-poor

16

Feature of histiocytic conditions?

very high ferratin

17

What are the 3 phases of CML?

Chronic (3-5yr)
Acute (1-2yr)
Blast phase (3-6mo)

18

What is the leukematoid reaction? Types and causes?

exaggerated resonse to infection
WBC >50,000
PMN-appendicitis
lymphocytic-whooping cough
eosinophilic-cutaneous larva migrans

19

What is the leukoerythroblastic reacton?

immature bone cells in the PB

20

What causes leukoerythroblastic rxns?

bone marrow fibrosis
metastatic cancer
severe bone marrow stress

21

Neutrophilia counts and causes?

>7000
infection
sterile infl w/necrosis
drugs

22

What drugs cause neutrophilia?

G-CSF
steroids
catecholamines
lithium

23

Neutropenia counts and causes?

<1500
aplastic anemia
immune destruction
septic shock
chemo

24

Eosinophilia counts and causes?

>700
Type 1 HSN
invasive helminths
hypocortisolism
neoplasms

25

Basophilia counts and causes?

>200
chronic kidney disease
CML

26

Clinical pres for MPN

5th-7th decade
splenomegaly and/orhepatomegaly

27

CML counts? penias/cytoses?

>100,000
Basophilia!!!!
neutrophilia
thrombocytosis
granulocytic hyperplasia

28

PV presentation?

splenomegaly
thrombotic events due to hyperviscosity
signs of increased histamine

29

PV lab findings?

increased RBC mass
decreased EPO
normal SaO2

30

BM with PV?

hypercellular with erythroid hyperplasia

31

PV survival?

>10yrs

32

Progression of PV?

"spent phase" similar to PMF
may develop MDS or AML

33

What kills pts w/PV?

thrombosis or hemorrhage

34

What is PMF?

MPN w/bone marrow fibrosis and extramedullary hematopoiesis

35

Clinical presentation of PMF?

splenomegaly w/portal hypertension
anemia
leukoerythroblastic reaction

36

Lab findings of PMF?

teardrop cells
increased platelet count

37

BM biopsy finding of PMF?

fibrosis
clusters of atypical megakaryocytes

38

PMF stages & survival

pre-fibrotic >10 yr
fibrotic 3-7 yr

39

What may PMF pts develop/advance to?

AML

40

What is ET (essential thrombocythemia)? Survival?

proliferation of megakaryocytes
MPN
12-15yr

41

Lab results of ET?

elevated platelet count in PB (>450,000)
atypical platelets

42

BM biopsy findings of ET?

hypercellular
abnormal megakaryocytes

43

Presentation of ET?

bleeding or thrombosis

44

Lab findings of MDS?

cytopenias
dysplastic features
ring sideroblasts
multinucleated RBC precursors
hypolobated megakaryocytes

45

What are ring sideroblasts?

iron accumulation in functionally impaired mitochondria

46

Causes of ring sideroblasts?

EtOH
Drugs/Toxins

47

Does ALL or AML have auer rods?

AML

48

AML outcome?

poor

49

AML clinical findings

severe leukopenia to marked leukocytosis
coagulopathy
anemia and thrombocytopenia

50

AML labs

>20% myeloid blasts
hypercellular BM

51

AML w/t(8,21) features

granulocytic maturation
extramedullary involvement
favorable prognosis

52

AML w/inv(16) features

myelomonocytic
eosinophils w/dysplastic features
extramedullary involvement
favorable prognosis

53

AML w/t(15,17) features

atypical promyelocytes
hypergranular
reniform nuclei
sometimes prominent auer rods
leukopenia
DIC
favorable prognosis

54

AML w/t(15,17) alias?

Acute promyelocytic leukemia (APL)

55

AML w/11q23 (MLL) rearrangements features?

infantile AML
monocytic
hyperleukocytosis
extramedularry issue infiltration

56

AML w/11q23 (MLL) is t(9,11) what is prognosis?

intermediate

57

AML w/11q23 is MLL what is prognosis?

intermediate to poor prognosis

58

What does NSE stain indicate?

monocytic lineage

59

AML w/myelodysplasia-related changes features?

dysplasia
occurs w/or w/o MDS
older individuals
poor prognosis

60

Therapy-related AML and MDS causes?

chemo/radiation
alkylating agents
topoisomerase II inhibitors

61

Alkylating agent related AML/MDS features?

5yrs post tx
multilineage dysplasia
very poor prognosis

62

Topoisomerase II inhibitor-related AML features?

2.5-3 yrs post tx
monocytic or myelomonocytic
poor prognosis

63

AML NOS features?

FAB classification-degree of maturation, lineage of differentiation

64

What disease is FAB3?

APL

65

What is myeloid sarcoma?

extramedullary tumor of immature myeloid cells

66

Key histologic feature of langerhans cell histiocytoses?

birbeck granules, tennis racket appearance

67

What is hemophagocytic lymphohistiocytosis?

hyperinflammatory condition
primary or familial