Cancer stuffs - MPNs and MDS Flashcards Preview

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Flashcards in Cancer stuffs - MPNs and MDS Deck (31):
1

MDS

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

2

MPNs

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

3

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

MDS

4

Marrow findings of MDS

1. Dysplasia:
- dyserythropoiesis
- dysgranulopoiesis
- dysmegakaryopoiesis

2. ringed sideroblasts
3. bi-lobed PMNs : pseudo-pelger huet cells
4. small megakaryocytes

5

Types of MDS

1. Primary/idiopathic
2. Secondary/ Therapy related

6

Types of MPNs

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

7

Onset of Primary vs Secondary MDS

primary: insidious

secondary: 2-8 yr latency

8

Cytogenic abnormality of primary/idiopathic MDS

- Monosomy 5 or 7
- del 7q or 5q
- trisomy 8

9

MDS vs MPNs Pt population target

MDS: >50, median: 70

MPNs: 40-60yrs

10

Neoplastic causes of secondary (T-MDS)

whole/partial del ch 5 or 7
in conjunction w/ use of alkylating agents and ionizing radiation

11

Non-neoplastic causes of secondary (T-MDS)

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

12

Low grade MDS

Myeloblasts <5% of marrow cells

1. RC-UD
2. RC-MD

13

High grade MDS

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

(CML of MPNs have >20% blasts)

14

Types of low grade MDS and prognosis

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

15

Types of high grade MDS and prognosis

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

16

Chromosomal abnormalities of:
CML

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

17

Chromosomal abnormalities of:
Polycythemia Vera

Jak2
- V617 point mutation

18

Chromosomal abnormalities of:
Primary myelofibrosis (PMF)

Jak2 in 50%

or CALR or MPL

19

Chromosomal abnormalities of:
Essential thromocytopenia (ET)

Jak2 in 50%

or CALR or MPL

20

Prognosis of:
- Polycythemia Vera
- Primary Myelofibrosis (PMF)
- Essential Thrombocytopenia (ET)

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

21

Stages/phases of:
CML

Initial phase
(accelerated phase)
Blast phase

22

Stages/phases of:
PV

Polycythemic stage
Spent phase

23

Stages/phases of:
PMF

Prefibrotic stage
Fibrotic stage

24

Observations in CML:
- Initial phase
- Blast phase

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

Observations in PV:
- Polycythemic stage
- Spent phase

Polycythemic stage
- ↑ cell count (hypercellularity)
- trilineage hyperplasia (erythrocytosis, neutrophilia, thrombocytosis)

Spent phase
- ↓ cell count
- marrow fibrosis
- Large macrophages

26

Observations in PMF:
- Prefibrotic stage
- Fibrotic stage

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

Fibrotic stage
- reticulin fibrosis of marrow
- dacrocytes
- extramedullary hematopoiesis
- leukoerythroblastosis

27

histo findings in ET

- normocellular
- Thrombocytosis (↑platelets)
- Large megakaryocytes (bizzare)

28

Symptoms of CML

- Splenomegaly
- Fatigue
- Anemia
- Weight loss
- Night Sweats

29

Symptoms of PV

- Hepato/splenomegaly
- Headaches
- dizziness
- Visual problems
- Paresthesia
- Plethora (redness)
- Itching

30

Symptoms of ET

- No splenomegaly
- Usually asymptomatic (Usu diagnosed from - ↑platelets)
- transient ischemic attack


31

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

Polycythemia Vera
- Portal
- Mesenteric
- Splenic

--> treat w/ serial phlebotomy