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ENGR 1 > MDS_and_MPNS > Flashcards

MDS_and_MPNS 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 primary: insidious

secondary: 2-8 yr latency

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 20% blasts)

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13
Q

High grade MDS

A

Myeloblasts >5 but >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 Jak2

- V617 point mutation

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)

Spent phase 
- ↓ cell count 
- marrow fibrosis 
- trilineage hyperplasia (erythrocytosis, neutrophilia, thrombocytosis) 
- 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
    
- Large megakaryocytes (bizzare)
    
- ↑platelets
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

ENGR 1 (29 decks)

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