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TT DD ex 3 and BL ex 2 > Cancer stuffs - MPNs and MDS > Flashcards

Cancer stuffs - MPNs and MDS Flashcards

1
Q

MDS

A

Neoplastic clonal population from STEM CELLS → take over marrow → failure to make normal cells in 1/m lineages → ineffective hematopoiesis → AML risk

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2
Q

MPNs

A

Neoplastic clonal population from NORMAL cells → take over marrow (Hypercellular marrow) → make too many normal cells in 1/m lineages → splenomegaly and/or hepatomegaly →

can escalate to marrow fibrosis → BM failure → MDS or AML

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3
Q

Is MDS or MPNs due to persistant cytopenias in 1/m lineages?

A

MDS

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4
Q

Marrow findings of MDS

A
  1. Dysplasia:
    - dyserythropoiesis
    - dysgranulopoiesis
    - dysmegakaryopoiesis
  2. ringed sideroblasts
  3. bi-lobed PMNs : pseudo-pelger huet cells
  4. small megakaryocytes
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5
Q

Types of MDS

A
  1. Primary/idiopathic

2. Secondary/ Therapy related

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6
Q

Types of MPNs

A
  1. Chronic Myelogenous leukemias (CML)
  2. Polycythemia Vera
  3. Primary Myelofibrosis (PMF)
  4. Essential Thrombocytopenia (ET)
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7
Q

Onset of Primary vs Secondary MDS

A

primary: insidious
secondary: 2-8 yr latency

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8
Q

Cytogenic abnormality of primary/idiopathic MDS

A
  • Monosomy 5 or 7
  • del 7q or 5q
  • trisomy 8
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9
Q

MDS vs MPNs Pt population target

A

MDS: >50, median: 70

MPNs: 40-60yrs

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10
Q

Neoplastic causes of secondary (T-MDS)

A

whole/partial del ch 5 or 7

in conjunction w/ use of alkylating agents and ionizing radiation

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11
Q

Non-neoplastic causes of secondary (T-MDS)

A
  1. chemo drugs
  2. Vit Def.
  3. Viral infection
  4. toxin/heavy metals
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12
Q

Low grade MDS

A

Myeloblasts <5% of marrow cells

  1. RC-UD
  2. RC-MD
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13
Q

High grade MDS

A

Myeloblasts >5
and/or >2% of peripheral blood cells

(CML of MPNs have >20% blasts)

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14
Q

Types of low grade MDS and prognosis

A
  1. Refractory Cytopenia with Unilineage Dysplasia (RC-UD)
    - good prognosis
  2. Refractory Cytopenia with Multilineage Dysplasia (RC-MD)
    - worse prognosis
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15
Q

Types of high grade MDS and prognosis

A
  1. Refractory Anemia with Excess Blasts-1 (RAEB-1)
    - dismal prognosis
  2. Refractory Anemia with Excess Blasts-2 (RAEB-2)
    - very dismal prognosis
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16
Q

Chromosomal abnormalities of:

CML

A

BCR-ABL t(9;22) p(210)

17
Q

Chromosomal abnormalities of:

Polycythemia Vera

A

Jak2

- V617 point mutation

18
Q 
Chromosomal abnormalities of:
Primary myelofibrosis (PMF)
A

Jak2 in 50%

or CALR or MPL

19
Q 
Chromosomal abnormalities of:
Essential thromocytopenia (ET)
A

Jak2 in 50%

or CALR or MPL

20
Q

Prognosis of:

  • Polycythemia Vera
  • Primary Myelofibrosis (PMF)
  • Essential Thrombocytopenia (ET)
A 
Polycythemia Vera
- Good (10-20 yrs)
Primary Myelofibrosis (PMF)
- poor (~5 yrs)
 Essential Thrombocytopenia (ET)
- Good (10-15 yrs)
21
Q

Stages/phases of:

CML

A

Initial phase
(accelerated phase)
Blast phase

22
Q

Stages/phases of:

PV

A

Polycythemic stage

Spent phase

23
Q

Stages/phases of:

PMF

A

Prefibrotic stage

Fibrotic stage

24
Q

Observations in CML:

  • Initial phase
  • Blast phase
A 
Initial phase:
(Hypercellular bone marrow): 
- ↑neutrophils (neutrophilia)
- ↑ basophils
- ↑platelets
- Small megakaryocytes (w round, non lobulated nuclei)

Blast phase:
- > 20% blasts in marrow/blood

Can progress to AML

25
Q

Observations in PV:

  • Polycythemic stage
  • Spent phase
A

Polycythemic stage

  • ↑ cell count (hypercellularity)
  • trilineage hyperplasia (erythrocytosis, neutrophilia, thrombocytosis)

Spent phase

  • ↓ cell count
  • marrow fibrosis
  • Large macrophages
26
Q

Observations in PMF:

  • Prefibrotic stage
  • Fibrotic stage
A 
Prefibrotic stage
(hypercellular marrow)
- ↑neutrophils (neutrophilia)
(no  ↑ basophils)
- ↑platelets
- Large megakaryocytes (bizarre)

Fibrotic stage

  • reticulin fibrosis of marrow
  • dacrocytes
  • extramedullary hematopoiesis
  • leukoerythroblastosis
27
Q

histo findings in ET

A
  • normocellular
  • Thrombocytosis (↑platelets)
  • Large megakaryocytes (bizzare)
28
Q

Symptoms of CML

A
  • Splenomegaly
  • Fatigue
  • Anemia
  • Weight loss
  • Night Sweats
29
Q

Symptoms of PV

A
  • Hepato/splenomegaly
  • Headaches
  • dizziness
  • Visual problems
  • Paresthesia
  • Plethora (redness)
  • Itching
30
Q

Symptoms of ET

A
  • No splenomegaly
  • Usually asymptomatic (Usu diagnosed from - ↑platelets)
  • transient ischemic attack
31
Q

Which of the MPNs is at risk for thrombosis? Of which arteries? how do you treat it?

A

Polycythemia Vera

  • Portal
  • Mesenteric
  • Splenic

–> treat w/ serial phlebotomy

TT DD ex 3 and BL ex 2 (21 decks)

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