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Heme/Onco > Hematopoietic stem cells and discorders > Flashcards

Hematopoietic stem cells and discorders Flashcards

1
Q

Polycythemia Vera

Sx

A

Pruritus after a warm shower
Erythromelalgia
TIA
DVT/PE

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2
Q

Polycythemia Vera

Physical exam

A

Splenomegaly

Plethora

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3
Q

Polycythemia Vera

Labs

A 
Low EPO
Basophilia (smear)
Leukocytosis
Thrombocytosis
JAK2 positive
High Vitam in B12 levels
Hyperuricemia
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4
Q 
DDx with PV
Mediated by hypoxemia
COPD/OSA
Congenital heart disease
Intrapumonary shutting
Elevated altitude
RAS
A

Thrombosis
TIA
Erythromelalgia unlikely
Pruritus unlikely

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5
Q

Mediated by hypoxemia

Physicals

A

Plethora

No splenomegaly

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6
Q

Mediated by hypoxemia

Labs

A

High EPO
No basophilia
No leukocytosis
JAK2 negative

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7
Q

mediated by ectopic or excessive EPO

A

Renal cell carcinoma
Hepatocellular carcinoma
Uterine fibroids

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8
Q

Mediated by ectopic or excessive EPO

Sx:

A

Thrombosis possible
TIA unlikely
Erythromelagia unikely
Pruritus unlikely

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9
Q

Mediated by ectopic or excessive EPO

Physicals

A

Plethora

No HSM

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10
Q

Mediated by ectopic or excessive EPO

Labs

A 
High EPO
Microscopic hematuria
No basophilia
Leukocytosis possible
JAK 2 negative
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11
Q

Unusual causes similar to PV

A

High oxygen affinity hemoglobin

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12
Q

Unusual causes
High oxygen affinity hemoglobin
Sx:

A

Thrombosis
TIA
Erythromelagia unlikely
Pruritus unlikely

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13
Q

Unusual causes
High oxygen affinity hemoglobin
Physicals

A

Plethora

No HSM

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14
Q

Unusual causes
High oxygen affinity hemoglobin
Labs:

A

High EPO
No basophilia
No Leukocytosis
JAK2 negative

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15
Q

PV

What are the 3 phases

A
  1. Latent phase
  2. Proliferative phase
  3. Spent phase (mimics PMF)
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16
Q

PV
Treatment
When to start?

A

At the time of diagnosis

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17
Q

PV

Treatment

A

ASA + phlebotomy if age < 60 yo w/o prior VTE or arterial thrombosis
Hydroxyurea + phlebotomy if age>60 or w/ prior VTE or arterial thrombosis

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18
Q

PV

Treatment goal

A

Hct <45%

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19
Q

PV

Prognosis

A

10% evolve into 2nd AML

20% spent phase: bone marrow fibrosis

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20
Q

ET

Dx:

A

Plt>600 at least 1 month apart

Exclude 2nd causes (iron deficiency and inflammation/infection)

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21
Q

ET
Rx:
when to treat

A

Can be observed

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22
Q

ET

Sx:

A

Digital ischemia, erythromelalgia, TIA, visual disturbances, VTE or bleeding

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23
Q

ET

Low risk population

A

Age<60;
No prior VTE;
WBC<11

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24
Q

ET

Rx: for high risk patients

A

Hydroxyurea

Plateletpheresis if plt>1000

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25
Q

ET

JAK 2 mutation

A

Only 50% of patients

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26
Q 
Primary Myelofibrosis (PMF)
Causes
A

One of the MPN;
Clonal myeloid disorder–>abnormal proliferating megakaryocytes–>excess fibroblast growth factor–>marrow fibrosis and extramedullary hematopoiesis

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27
Q

PMF
Rx:
When to start

A

Can be observed;

Rx for symptomatic splenomegaly, worsening cytopenias, and constitutional symptoms.

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28
Q

PMF

Rx:

A

Splenectomy (perilous);
JAK2 inhibitor: roxolitinib;
HSCT: applied in younger patients, treatment is palliative

29
Q

Eosinophilia and hypereosinophilic syndrome

Dx

A

Eosinophilia>1.5x10 9

30
Q

Eosinophilia and hypereosinophilic syndrome

Causes

A

Molecular activation of platelet-derived growth factor receptor (PDGFR) alpha or beta or without a known stimulanting factor

31
Q

Eosinophilia and hypereosinophilic syndrome

Rx:

A

Glucocorticoids;

Imatinib better in steroid-refractory primary hypereosinophilic syndrome

32
Q

Myelodysplastic syndrome

Bone marrow character:

A

Hypercellular

33
Q

Myelodysplastic syndrome

Dx and prognosis

A

Bone marrow biopsy and aspiration with cytogenetic studies

34
Q

Myelodysplastic syndrome

Rx goals:

A
  1. Relieve transfusion dependence

2. Prevent transformation to AML

35
Q

Myelodysplastic syndrome
Rx
Low-risk patient

A

Does not require treatment at all or infrequent transfusions

36
Q

Myelodysplastic syndrome

Risk stratification

A 
IPSS-R score:
Bone marrow blasts (%);
Cytogenetics;
Hgb;
Plt count;
ANC.
37
Q 
Myeloproliferative Neoplasms (MPN)
What does it include
A 
CML
PV
ET
Primary myelofibrosis
Eosinophilia and hypereosinophilic syndromes
38
Q

CML

Causes

A

Philadelphia chromosome;
Translocation of chromosome 9 and 22 t(9;22)
BCR-ABL gene

39
Q

CML
Rx
When to treat

A

Treatment required at diagnosis

40
Q

CML
Rx
What to treat

A

TKI: Imatinib, nilotinib, or dasatinib and ponatinib

41
Q

CML
Rx
SE

A

TKI: prolong QT

42
Q

CML
Rx
SE: dasatinib:

A

Pericardial and pleural effusion, pulmonary artery HTN

43
Q

CML
Rx
SE: ponatinib

A

severe VTE

44
Q

AML

Dx

A

20% or more myeloblasts in either peripheral blood or the marrow

45
Q

AML

Prognosis

A

Genetic profile of the leukemic cells.

46
Q

AML
Rx decisions
Confirm AML

A

Exclude leukemoid reaction, atypical monocytosis, and chronic leukemias: peripheral blood smear review, flow cytometry

47
Q

AML
Rx decisions
AML vs ALL

A

Auer rod on blood smear suggests AML, confirmed with flow cytometry; different treatment paths

48
Q

AML
Rx decisions
Exclude APL

A

1 Clinically suspected with DIC, classically with promyelocytes and prominent Auer rods, microgranular variant evaluated by flow cytometry (within 24 hours is ideal, but not possible at all institutions),
2 FISH for t(15; 17).
3 Administer ATRA if suspected; do not await confirmation.

49
Q

AML
Rx decisions
AML not APL

A

Begin induction therapy with cytosine arabinoside and an anthracycline

50
Q

AML
Rx decisions
ALL

A

Philadelphia chromosome positivity or negativity (determination within 24 hours is ideal, but not possible at all institutions) decides TKI therapy during induction; adolescents/young adults benefit from more intensive pediatric regimens

51
Q

AML
Rx decisions
Later dexisions- Allogeneic HSC consolidation

A

Based on fitness of patient and risk of leukemia, predominantly determined by cytogenetic and molecular risk profile

52
Q

AML
Rx decisions
Later dexisions- Chemotherapy consolidation

A

Lower risk leukemia, older or less fit patient

53
Q

AML
Genetic risk profile cytogenetic category
Favorable

A

t (8;21)
inv (16)
t (15;17)

54
Q

AML
Genetic risk profile cytogenetic category
Intermediate

A

Neither favorable nor high

55
Q

AML
Genetic risk profile cytogenetic category
High risk

A 
complex (>=5 abnormalities)
-5
-7 
del (5q)
3q abnormal
56
Q

AML
Treatment
Non-APL

A

7 day course of cytarabine + 3 day course of anthracycline

57
Q

AML
Treatment
Non-APL- if patient is age>70 or ill

A

less toxic hypomethylating agent

Azacitidine or decitabine

58
Q

ALL

How to stage

A

CNS analysis with intrachecal chmo

59
Q

ALL

Dx

A

25% lymphoblasts in the blood or bone marrow

60
Q

ALL

Lymphoid blasts features

A

TdT positve;

MPO negative

61
Q

ALL

Risk factors

A 
MLLgene rearrangement
Hypodiploidy
Philadephia chromosome (treatment with TKI)
62
Q

ALL
Treatment
Young adults

A

Intensive pediatric regimens: asparaginase

HIgh-risk: allogeneic HSCT

63
Q

Hematopoietic growth factors

G-CSF

A

Stimulate neutrophil:

  1. autoimmune neutropenia
  2. hasten neutrophil recovery after cytotoxic chemotherapy
  3. HSC mobilization
64
Q

Hematopoietic growth factors

EPO

A 
Anemia of CKD in HD or predialysis
1. Iron, B12 and folic acid are repleted
2. TIBC>25%, serum ferritin >100ng/mL;
3. Goal 11g/dL
Hasten recovery in chemotherapy-associated anemia
65
Q

HSCT

Treatment-most helpful

A

AA
High-risk MDS
AML

66
Q

HSCT

Risks

A

Opportunistic infection;

GVH disease: atact gut, liver and skin

67
Q

HSCT

Treatment for GVHD

A

Steroids

68
Q

HSCT

Primary cause of mortality after HSCT for malignant disease

A

Relapse of the original disease

Heme/Onco (19 decks)

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