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Heme/Lymph > Tuesday Groupings/Definitions/Presentations > Flashcards

Tuesday Groupings/Definitions/Presentations Flashcards

1
Q

What are the 4 myeloproliferative neoplasms we know?

A

CML
PV
PMF
ET

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2
Q

What are the categories of myeloid neoplasms?

A

MPN
MDS
AML

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3
Q

What is MPN (myeloproliferative neoplasm)?

A

clonal hematopoietic stem cell disorders

proliferation of one of more of the myeloid lineages

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4
Q

What is similar and different about MPN vs MDS?

A

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

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5
Q

What is MDS (myelodysplastic syndrome)?

A

clonal hematopoietic stem cell disorder

cytopenias, dysplasia, ineffective hematopoiesis

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6
Q

What does MPN have the potential to develop to?

A

BM fibrosis or acute leukemia

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7
Q

What does MDS hav the potential to develop to?

A

AML (30% of cases)

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8
Q

What percentage of blasts are found in MDS?

A

<20%

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9
Q

What kills with MDS?

A

infection or bleeding

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10
Q

Median survival of MDS?

A

9-29mo

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11
Q

What is acute leukemia?

A

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

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12
Q

Acute vs Chronic leukemias

A

acute-immature cells, untreated live weeks to months

chronic-mature cells, untreated live months to years

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13
Q

AML vs ALL

A

AML-adults

ALL-kiddos

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14
Q

What is AML (acute myeloid leukemia)?

A

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

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15
Q

AML types and prognoses

A

AML w/recurrent cytogenic abnormalities-favorable

AML w/myelodysplasia associated chagnes-poor

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16
Q

Feature of histiocytic conditions?

A

very high ferratin

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17
Q

What are the 3 phases of CML?

A

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

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18
Q

What is the leukematoid reaction? Types and causes?

A 
exaggerated resonse to infection
WBC >50,000
PMN-appendicitis
lymphocytic-whooping cough
eosinophilic-cutaneous larva migrans
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19
Q

What is the leukoerythroblastic reacton?

A

immature bone cells in the PB

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20
Q

What causes leukoerythroblastic rxns?

A

bone marrow fibrosis
metastatic cancer
severe bone marrow stress

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21
Q

Neutrophilia counts and causes?

A

> 7000
infection
sterile infl w/necrosis
drugs

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22
Q

What drugs cause neutrophilia?

A

G-CSF
steroids
catecholamines
lithium

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23
Q

Neutropenia counts and causes?

A 
<1500
aplastic anemia
immune destruction
septic shock
chemo
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24
Q

Eosinophilia counts and causes?

A 
>700
Type 1 HSN
invasive helminths
hypocortisolism
neoplasms
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25
Q

Basophilia counts and causes?

A

> 200
chronic kidney disease
CML

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26
Q

Clinical pres for MPN

A

5th-7th decade

splenomegaly and/orhepatomegaly

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27
Q

CML counts? penias/cytoses?

A 
>100,000
Basophilia!!!!
neutrophilia
thrombocytosis
granulocytic hyperplasia
28
Q

PV presentation?

A

splenomegaly
thrombotic events due to hyperviscosity
signs of increased histamine

29
Q

PV lab findings?

A

increased RBC mass
decreased EPO
normal SaO2

30
Q

BM with PV?

A

hypercellular with erythroid hyperplasia

31
Q

PV survival?

A

> 10yrs

32
Q

Progression of PV?

A

“spent phase” similar to PMF

may develop MDS or AML

33
Q

What kills pts w/PV?

A

thrombosis or hemorrhage

34
Q

What is PMF?

A

MPN w/bone marrow fibrosis and extramedullary hematopoiesis

35
Q

Clinical presentation of PMF?

A

splenomegaly w/portal hypertension
anemia
leukoerythroblastic reaction

36
Q

Lab findings of PMF?

A

teardrop cells

increased platelet count

37
Q

BM biopsy finding of PMF?

A

fibrosis

clusters of atypical megakaryocytes

38
Q

PMF stages & survival

A

pre-fibrotic >10 yr

fibrotic 3-7 yr

39
Q

What may PMF pts develop/advance to?

A

AML

40
Q

What is ET (essential thrombocythemia)? Survival?

A

proliferation of megakaryocytes
MPN
12-15yr

41
Q

Lab results of ET?

A

elevated platelet count in PB (>450,000)

atypical platelets

42
Q

BM biopsy findings of ET?

A

hypercellular

abnormal megakaryocytes

43
Q

Presentation of ET?

A

bleeding or thrombosis

44
Q

Lab findings of MDS?

A 
cytopenias
dysplastic features
ring sideroblasts
multinucleated RBC precursors
hypolobated megakaryocytes
45
Q

What are ring sideroblasts?

A

iron accumulation in functionally impaired mitochondria

46
Q

Causes of ring sideroblasts?

A

EtOH

Drugs/Toxins

47
Q

Does ALL or AML have auer rods?

A

AML

48
Q

AML outcome?

A

poor

49
Q

AML clinical findings

A

severe leukopenia to marked leukocytosis
coagulopathy
anemia and thrombocytopenia

50
Q

AML labs

A

> 20% myeloid blasts

hypercellular BM

51
Q

AML w/t(8,21) features

A

granulocytic maturation
extramedullary involvement
favorable prognosis

52
Q

AML w/inv(16) features

A

myelomonocytic
eosinophils w/dysplastic features
extramedullary involvement
favorable prognosis

53
Q

AML w/t(15,17) features

A 
atypical promyelocytes
hypergranular
reniform nuclei
sometimes prominent auer rods
leukopenia
DIC
favorable prognosis
54
Q

AML w/t(15,17) alias?

A

Acute promyelocytic leukemia (APL)

55
Q

AML w/11q23 (MLL) rearrangements features?

A

infantile AML
monocytic
hyperleukocytosis
extramedularry issue infiltration

56
Q

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

A

intermediate

57
Q

AML w/11q23 is MLL what is prognosis?

A

intermediate to poor prognosis

58
Q

What does NSE stain indicate?

A

monocytic lineage

59
Q

AML w/myelodysplasia-related changes features?

A

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

60
Q

Therapy-related AML and MDS causes?

A

chemo/radiation
alkylating agents
topoisomerase II inhibitors

61
Q

Alkylating agent related AML/MDS features?

A

5yrs post tx
multilineage dysplasia
very poor prognosis

62
Q

Topoisomerase II inhibitor-related AML features?

A

2.5-3 yrs post tx
monocytic or myelomonocytic
poor prognosis

63
Q

AML NOS features?

A

FAB classification-degree of maturation, lineage of differentiation

64
Q

What disease is FAB3?

A

APL

65
Q

What is myeloid sarcoma?

A

extramedullary tumor of immature myeloid cells

66
Q

Key histologic feature of langerhans cell histiocytoses?

A

birbeck granules, tennis racket appearance

67
Q

What is hemophagocytic lymphohistiocytosis?

A

hyperinflammatory condition

primary or familial

Heme/Lymph (18 decks)

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