Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Heme/Onco > Hematopoietic stem cells and discorders > Flashcards

Hematopoietic stem cells and discorders Flashcards

(68 cards)

Start Studying
1
Q

Polycythemia Vera

Sx

A

Pruritus after a warm shower
Erythromelalgia
TIA
DVT/PE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Polycythemia Vera

Physical exam

A

Splenomegaly

Plethora

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Polycythemia Vera

Labs

A 
Low EPO
Basophilia (smear)
Leukocytosis
Thrombocytosis
JAK2 positive
High Vitam in B12 levels
Hyperuricemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Mediated by hypoxemia

Physicals

A

Plethora

No splenomegaly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Mediated by hypoxemia

Labs

A

High EPO
No basophilia
No leukocytosis
JAK2 negative

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

mediated by ectopic or excessive EPO

A

Renal cell carcinoma
Hepatocellular carcinoma
Uterine fibroids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Mediated by ectopic or excessive EPO

Sx:

A

Thrombosis possible
TIA unlikely
Erythromelagia unikely
Pruritus unlikely

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Mediated by ectopic or excessive EPO

Physicals

A

Plethora

No HSM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Mediated by ectopic or excessive EPO

Labs

A 
High EPO
Microscopic hematuria
No basophilia
Leukocytosis possible
JAK 2 negative
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Unusual causes similar to PV

A

High oxygen affinity hemoglobin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Unusual causes
High oxygen affinity hemoglobin
Sx:

A

Thrombosis
TIA
Erythromelagia unlikely
Pruritus unlikely

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Unusual causes
High oxygen affinity hemoglobin
Physicals

A

Plethora

No HSM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Unusual causes
High oxygen affinity hemoglobin
Labs:

A

High EPO
No basophilia
No Leukocytosis
JAK2 negative

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

PV

What are the 3 phases

A
  1. Latent phase
  2. Proliferative phase
  3. Spent phase (mimics PMF)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

PV
Treatment
When to start?

A

At the time of diagnosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

PV

Treatment goal

A

Hct <45%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

PV

Prognosis

A

10% evolve into 2nd AML

20% spent phase: bone marrow fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

ET

Dx:

A

Plt>600 at least 1 month apart

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

ET
Rx:
when to treat

A

Can be observed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

ET

Sx:

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

ET

Low risk population

A

Age<60;
No prior VTE;
WBC<11

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

ET

Rx: for high risk patients

A

Hydroxyurea

Plateletpheresis if plt>1000

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
ET | JAK 2 mutation
Only 50% of patients
26
``` Primary Myelofibrosis (PMF) Causes ```
One of the MPN; Clonal myeloid disorder-->abnormal proliferating megakaryocytes-->excess fibroblast growth factor-->marrow fibrosis and extramedullary hematopoiesis
27
PMF Rx: When to start
Can be observed; | Rx for symptomatic splenomegaly, worsening cytopenias, and constitutional symptoms.
28
PMF | Rx:
Splenectomy (perilous); JAK2 inhibitor: roxolitinib; HSCT: applied in younger patients, treatment is palliative
29
Eosinophilia and hypereosinophilic syndrome | Dx
Eosinophilia>1.5x10 9
30
Eosinophilia and hypereosinophilic syndrome | Causes
Molecular activation of platelet-derived growth factor receptor (PDGFR) alpha or beta or without a known stimulanting factor
31
Eosinophilia and hypereosinophilic syndrome | Rx:
Glucocorticoids; | Imatinib better in steroid-refractory primary hypereosinophilic syndrome
32
Myelodysplastic syndrome | Bone marrow character:
Hypercellular
33
Myelodysplastic syndrome | Dx and prognosis
Bone marrow biopsy and aspiration with cytogenetic studies
34
Myelodysplastic syndrome | Rx goals:
1. Relieve transfusion dependence | 2. Prevent transformation to AML
35
Myelodysplastic syndrome Rx Low-risk patient
Does not require treatment at all or infrequent transfusions
36
Myelodysplastic syndrome | Risk stratification
``` IPSS-R score: Bone marrow blasts (%); Cytogenetics; Hgb; Plt count; ANC. ```
37
``` Myeloproliferative Neoplasms (MPN) What does it include ```
``` CML PV ET Primary myelofibrosis Eosinophilia and hypereosinophilic syndromes ```
38
CML | Causes
Philadelphia chromosome; Translocation of chromosome 9 and 22 t(9;22) BCR-ABL gene
39
CML Rx When to treat
Treatment required at diagnosis
40
CML Rx What to treat
TKI: Imatinib, nilotinib, or dasatinib and ponatinib
41
CML Rx SE
TKI: prolong QT
42
CML Rx SE: dasatinib:
Pericardial and pleural effusion, pulmonary artery HTN
43
CML Rx SE: ponatinib
severe VTE
44
AML | Dx
20% or more myeloblasts in either peripheral blood or the marrow
45
AML | Prognosis
Genetic profile of the leukemic cells.
46
AML Rx decisions Confirm AML
Exclude leukemoid reaction, atypical monocytosis, and chronic leukemias: peripheral blood smear review, flow cytometry
47
AML Rx decisions AML vs ALL
Auer rod on blood smear suggests AML, confirmed with flow cytometry; different treatment paths
48
AML Rx decisions Exclude APL
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
AML Rx decisions AML not APL
Begin induction therapy with cytosine arabinoside and an anthracycline
50
AML Rx decisions ALL
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
AML Rx decisions Later dexisions- Allogeneic HSC consolidation
Based on fitness of patient and risk of leukemia, predominantly determined by cytogenetic and molecular risk profile
52
AML Rx decisions Later dexisions- Chemotherapy consolidation
Lower risk leukemia, older or less fit patient
53
AML Genetic risk profile cytogenetic category Favorable
t (8;21) inv (16) t (15;17)
54
AML Genetic risk profile cytogenetic category Intermediate
Neither favorable nor high
55
AML Genetic risk profile cytogenetic category High risk
``` complex (>=5 abnormalities) -5 -7 del (5q) 3q abnormal ```
56
AML Treatment Non-APL
7 day course of cytarabine + 3 day course of anthracycline
57
AML Treatment Non-APL- if patient is age>70 or ill
less toxic hypomethylating agent | Azacitidine or decitabine
58
ALL | How to stage
CNS analysis with intrachecal chmo
59
ALL | Dx
25% lymphoblasts in the blood or bone marrow
60
ALL | Lymphoid blasts features
TdT positve; | MPO negative
61
ALL | Risk factors
``` MLLgene rearrangement Hypodiploidy Philadephia chromosome (treatment with TKI) ```
62
ALL Treatment Young adults
Intensive pediatric regimens: asparaginase | HIgh-risk: allogeneic HSCT
63
Hematopoietic growth factors | G-CSF
Stimulate neutrophil: 1. autoimmune neutropenia 2. hasten neutrophil recovery after cytotoxic chemotherapy 3. HSC mobilization
64
Hematopoietic growth factors | EPO
``` 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
HSCT | Treatment-most helpful
AA High-risk MDS AML
66
HSCT | Risks
Opportunistic infection; | GVH disease: atact gut, liver and skin
67
HSCT | Treatment for GVHD
Steroids
68
HSCT | Primary cause of mortality after HSCT for malignant disease
Relapse of the original disease

Heme/Onco (19 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions