Tuesday Groupings/Definitions/Presentations

Question 1

What are the 4 myeloproliferative neoplasms we know?

Answer 1

CML
PV
PMF
ET

Question 2

What are the categories of myeloid neoplasms?

Answer 2

MPN
MDS
AML

Question 3

What is MPN (myeloproliferative neoplasm)?

Answer 3

clonal hematopoietic stem cell disorders

proliferation of one of more of the myeloid lineages

Question 4

What is similar and different about MPN vs MDS?

Answer 4

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

Question 5

What is MDS (myelodysplastic syndrome)?

Answer 5

clonal hematopoietic stem cell disorder

cytopenias, dysplasia, ineffective hematopoiesis

Question 6

What does MPN have the potential to develop to?

Answer 6

BM fibrosis or acute leukemia

Question 7

What does MDS hav the potential to develop to?

Answer 7

AML (30% of cases)

Question 8

What percentage of blasts are found in MDS?

Answer 8

<20%

Question 9

What kills with MDS?

Answer 9

infection or bleeding

Question 10

Median survival of MDS?

Answer 10

9-29mo

Question 11

What is acute leukemia?

Answer 11

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

Question 12

Acute vs Chronic leukemias

Answer 12

acute-immature cells, untreated live weeks to months

chronic-mature cells, untreated live months to years

Question 13

AML vs ALL

Answer 13

AML-adults

ALL-kiddos

Question 14

What is AML (acute myeloid leukemia)?

Answer 14

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

Question 15

AML types and prognoses

Answer 15

AML w/recurrent cytogenic abnormalities-favorable

AML w/myelodysplasia associated chagnes-poor

Question 16

Feature of histiocytic conditions?

Answer 16

very high ferratin

Question 17

What are the 3 phases of CML?

Answer 17

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

Question 18

What is the leukematoid reaction? Types and causes?

Answer 18

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

Question 19

What is the leukoerythroblastic reacton?

Answer 19

immature bone cells in the PB

Question 20

What causes leukoerythroblastic rxns?

Answer 20

bone marrow fibrosis
metastatic cancer
severe bone marrow stress

Question 21

Neutrophilia counts and causes?

Answer 21

> 7000
infection
sterile infl w/necrosis
drugs

Question 22

What drugs cause neutrophilia?

Answer 22

G-CSF
steroids
catecholamines
lithium

Question 23

Neutropenia counts and causes?

Answer 23

<1500
aplastic anemia
immune destruction
septic shock
chemo

Question 24

Eosinophilia counts and causes?

Answer 24

>700
Type 1 HSN
invasive helminths
hypocortisolism
neoplasms

Question 25

Basophilia counts and causes?

Answer 25

>200 chronic kidney disease CML

Question 26

Clinical pres for MPN

Answer 26

5th-7th decade | splenomegaly and/orhepatomegaly

Question 27

CML counts? penias/cytoses?

Answer 27

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

Question 28

PV presentation?

Answer 28

splenomegaly thrombotic events due to hyperviscosity signs of increased histamine

Question 29

PV lab findings?

Answer 29

increased RBC mass decreased EPO normal SaO2

Question 30

BM with PV?

Answer 30

hypercellular with erythroid hyperplasia

Question 31

PV survival?

Answer 31

>10yrs

Question 32

Progression of PV?

Answer 32

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

Question 33

What kills pts w/PV?

Answer 33

thrombosis or hemorrhage

Question 34

What is PMF?

Answer 34

MPN w/bone marrow fibrosis and extramedullary hematopoiesis

Question 35

Clinical presentation of PMF?

Answer 35

splenomegaly w/portal hypertension anemia leukoerythroblastic reaction

Question 36

Lab findings of PMF?

Answer 36

teardrop cells | increased platelet count

Question 37

BM biopsy finding of PMF?

Answer 37

fibrosis | clusters of atypical megakaryocytes

Question 38

PMF stages & survival

Answer 38

pre-fibrotic >10 yr | fibrotic 3-7 yr

Question 39

What may PMF pts develop/advance to?

Answer 39

AML

Question 40

What is ET (essential thrombocythemia)? Survival?

Answer 40

proliferation of megakaryocytes MPN 12-15yr

Question 41

Lab results of ET?

Answer 41

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

Question 42

BM biopsy findings of ET?

Answer 42

hypercellular | abnormal megakaryocytes

Question 43

Presentation of ET?

Answer 43

bleeding or thrombosis

Question 44

Lab findings of MDS?

Answer 44

``` cytopenias dysplastic features ring sideroblasts multinucleated RBC precursors hypolobated megakaryocytes ```

Question 45

What are ring sideroblasts?

Answer 45

iron accumulation in functionally impaired mitochondria

Question 46

Causes of ring sideroblasts?

Answer 46

EtOH | Drugs/Toxins

Question 47

Does ALL or AML have auer rods?

Answer 47

AML

Question 48

AML outcome?

Answer 48

poor

Question 49

AML clinical findings

Answer 49

severe leukopenia to marked leukocytosis coagulopathy anemia and thrombocytopenia

Question 50

AML labs

Answer 50

>20% myeloid blasts | hypercellular BM

Question 51

AML w/t(8,21) features

Answer 51

granulocytic maturation extramedullary involvement favorable prognosis

Question 52

AML w/inv(16) features

Answer 52

myelomonocytic eosinophils w/dysplastic features extramedullary involvement favorable prognosis

Question 53

AML w/t(15,17) features

Answer 53

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

Question 54

AML w/t(15,17) alias?

Answer 54

Acute promyelocytic leukemia (APL)

Question 55

AML w/11q23 (MLL) rearrangements features?

Answer 55

infantile AML monocytic hyperleukocytosis extramedularry issue infiltration

Question 56

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

Answer 56

intermediate

Question 57

AML w/11q23 is MLL what is prognosis?

Answer 57

intermediate to poor prognosis

Question 58

What does NSE stain indicate?

Answer 58

monocytic lineage

Question 59

AML w/myelodysplasia-related changes features?

Answer 59

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

Question 60

Therapy-related AML and MDS causes?

Answer 60

chemo/radiation alkylating agents topoisomerase II inhibitors

Question 61

Alkylating agent related AML/MDS features?

Answer 61

5yrs post tx multilineage dysplasia very poor prognosis

Question 62

Topoisomerase II inhibitor-related AML features?

Answer 62

2.5-3 yrs post tx monocytic or myelomonocytic poor prognosis

Question 63

AML NOS features?

Answer 63

FAB classification-degree of maturation, lineage of differentiation

Question 64

What disease is FAB3?

Answer 64

APL

Question 65

What is myeloid sarcoma?

Answer 65

extramedullary tumor of immature myeloid cells

Question 66

Key histologic feature of langerhans cell histiocytoses?

Answer 66

birbeck granules, tennis racket appearance

Question 67

What is hemophagocytic lymphohistiocytosis?

Answer 67

hyperinflammatory condition | primary or familial