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Disease and Defense > MPN/MDS > Flashcards

MPN/MDS Flashcards

1
Q

MDS

A

myelodysplastic syndrome

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2
Q

Characterize MDS

A

ineffective hematopoiesis - cytopenias

increased risk to transform to AML (replaces marrow)

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3
Q

Secondary MDS (cause, time frame, genetics)

A

t-AML

2-8 years after alkylating agents/ionizing radiation - complex karyotype with whole/partial chr5

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4
Q

When to suspect MDS

A

2 or more lineage cytopenia + older,

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5
Q

MDS Diagnosis (3)

A

morphological evidence of dysplasia
Increased myeloblast, less than 20% of blood/marrow cell
Presence of clonal cytogenetics abnormality

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6
Q

Morphologic evidence of Dysplasia

A

10%+ of cell in one linage –> dysplastic = dyshematopoiesis

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7
Q

Cytogenetic evidence of MDS

A
  • complex karyotype with whole or partial deletions of chromosomes 5 and/or 7
  • isolated deletion 5q
  • trisomy 8
  • others
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8
Q

Low grade MDS (2)

A

myeloblasts not increased in frequency < 5% marrow/2% perperipheral

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9
Q

High grade MDS (2)

A

Myeloblasts increased, less than 20% (5-19% marrow, 3-19% blood cell)

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10
Q

2 types of low grade MDS

A

Refractory cytopenia with unilineage dysplasia (RCUD)
Refractory cytopenia with multilineage dysplasia (RCMD)
# lineages

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11
Q

2 types of high grade

A

refractory anemia with excess blast 1 (RAEB1)
Refractory anemia with excess blasts 2 (RAEB2)
#% myeloblastes

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12
Q

List 4 myeloproliferative neoplasms

A 
chronic myelogenous leukemia (CML)
Polycythemia vera (PV)
Primary myelofirosis (PMF)
Essential Thrombocythemia (ET)
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13
Q

Prognosis of RCUD and RCMD

A

RCUD: good - median survival > 5 years, risk –> AML 2% after 5 years
RCMD - poor - median survival 2.5 years, 10% AML in 2 yrs

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14
Q

Prognosis of RAEB1 and RAEB2

A

RAEB2 - med survival 16 mo; AML - 25%

RAEB2 - Med survival 9 mo, 33% to AML

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15
Q

MPN vs MDS

A

MPN - increase production of normal cell

MDS - ineffective hematopoiesis - weird cells

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16
Q

MPN pt’s age

A

50-70 y/o

17
Q

CML genetics

A

BCR-ABL1 t(9;22)(q34;q11.2)

18
Q

CML chronic phase

A 
slight blast elevate, neutrophilia, increase baso/plt
Hypercellular marrow (granulocytic hyperplasia)
19
Q

CML Blast phase

A

“acute” >20% blasts in marrow/blood

70/30% myeloblasts/lymphoblasts

20
Q

CML diagnosis tools

A

cytogenetics - karyotypes
Fish BCR-ABL fusion probes
RT-PCR of BCR-ABL mRNA transcriptis

21
Q

CML Treatments

A

PTKI - protein tyrosine inhibitors
Gleevac/Imatinib
Dasatinib (2nd gen)

22
Q

PV = ?

A

polycythemia vera, increase RBC mass

23
Q

PV phases and description

A

Polycythemic stage - increase peripheral blood cell counts

Spent phase: Post PV myelofibrosis - marrow fibrosis, decrease peripheral blood count

24
Q

PV complications

A

Thrombosis

mesenteric veins, portal veins, splenic veins

25
Q

PV treatment

A

serial phlebotomy, asprin therapy, chemo

26
Q

PMF = ?

A

Primary myelofibrosis - granulocytic/megakaryocytic hyperplasia (NOT RBC)

27
Q

PV genetics

A

JAK2 V617F

28
Q

PMF genetics

A

JAK2 MPL and CALR

29
Q

PMF 2 stages and major differences

A

Prefibrotic stage - hypercellular, neutrophilia, increased plt, large cluster megakaryocytes
Fibrotic stage - Intramedullary extramedullary hematopoiesis, leukoerythroblasts, enlarged spleen

30
Q

PMF main cause of death through…

A

Bone marrow failure due to (secondary) fibrosis

31
Q

ET = ?

A

Thrombocytosis, essential thrombocythemia

NO granulocytic hyperplasia

32
Q

ET genetics

A

JAK2 MPL and CALR

33
Q

PMF survival

A

5 year - because late findings

34
Q

ET survival and common death

A

10-15+ years, clot

35
Q

ET symptoms

A

Transient ischemic attacks, digital ischmeia, arterial/venous thrombosis (less common than PV)
NO splenomegaly

36
Q

Primary MDS age/incident

A

70, 3-5/100k

Disease and Defense (10 decks)

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