Hemepath: Chronic Myeloproliferative Disease (Who)

Front 1

In CMPD, the proliferation is associated with ___ maturation that is ___, resulting in _____ numbers of granulocytes, RBCs, and/or platelets

Back 1

1. relatively normal maturation
2. effective hematopoiesis
3. increased

Front 2

Hepatomegaly and splenomegaly are ___ in CMPD's

Back 2

common

Front 3

All CMPD's have the potential for clonal evolution, with ___ or ___

Back 3

1. progression terminating in bone marrow failure due to myelofibrosis or INEFFECTIVE hematopoiesis

2. transformation into an acute blast phase

Front 4

Indications of worsening disease (progression) in CMPDs include:

Back 4

1. cytogenetic or molecular evidence of clonal evolution

2. increase in the percentage of blast in the blood and/or bone marrow

10-19% blasts in blood or bone marrow indicates disease acceleration

20%+ = blast phase

Front 5

The various CMPDs can all exhibit an overlapping constellation of findings including leukocytosis, thrombocytosis, excessive megakaryocytic proliferation, myelofibrosis and organomegaly.

If the initial findings do not allow a specific classification, the designation ____ may be preferable until the process becomes more clearly defined.

Back 5

CMPD, unclassifiable

Front 6

Only one CMPD has a defining cytogenetic abnormality, and that is ___

Back 6

CML with the BCR/ABL fusion gene t(9;22)(q34,q11)

Front 7

Bone marrow cellularity for the follow is usually __
CMPD___
MDS __
MDS/CMPD___
AML___

Back 7

CMPD: increased

MDS: increased (occaisonally decreased)

MDS/CMPD: increased

AML: increased (occaisonally decreased)

Front 8

Typical marrow blast % for each
CMPD___
MDS __
MDS/CMPD___
AML___

Back 8

CMPD <10%
MDS <20%
MDS/CMPD <20%
AML >/=20%

Front 9

CMPD are associated with ___ blood cell counts, while MDS is associated with ___

Back 9

1. CMPD: increased

2. MDS: cytopenias

Front 10

In CMPDs, the myelofibrosis is a ___ event, most likely due to ___

Back 10

Secondary event - the fibroblasts are not clonal

Megakaryocytes and other marrow cells abnormally producing several cytokines (PDGF, TGF-beta) that stimulate fibroblastic proliferation and synthesis of collagen and fibronectin

Front 11

CML is a CMPD that is consistently associated with the ___ and/or the ___

Back 11

Philadelphia (Ph) chromosome and/or BCR/ABL gene fusion

Front 12

In CML, the abnormal fusion gene is found in which myeloid lineage(s)?

Back 12

All myeloid lineages as well as some lymphoid cells!

Front 13

CML is biphasic or triphasic, in that the initial __ phase is followed by ___

Back 13

chronic phase (indolent)
(CML-CP)

one or both of the aggressive TRANSFORMED phases:
CML-AP (accelerated phase)
CML-BP (blast phase)

Front 14

The mose common type of CMPD is __

Back 14

CML

Front 15

In CML-CP, the leukemic cells are minimally invasive, meaning that they are confined to ____, in the transformed stages (CML-AP or CML-BP), the leukemic cells are found __

Back 15

1. hematopoietic tissue (BM, PB, spleen, liver)

2. extramedullary tissues (skin, lymph nodes, soft tissues, CNS)

Front 16

Most patients with CML are diagnosed in the ___ phase

___percent are ____at diagnosis

Back 16

chronic phase, with an insidious onset

20-40% are asymptomatic at diagnosis, and discovered due to a high WBC count on a routine exam

Front 17

Common findings seen in CML at the time of diagnosis

Back 17

fatigue
weight loss
anemia
night sweats
SPLENOMEGALY

Front 18

An atypical presentation of CML may include

Back 18

presentation in the blast phase (CML-BP) without the detection of a chronic phase
(basically AML w/ the Ph chromosome)

Front 19

The peripheral blood in CML-CP

Back 19

Median WBC count 170 (mostly neutrophils in different stages of maturation (increased myelocytes and bands)

Blasts usually <2%

Absolute basophilia (invariable!!)

NO! significant dysgranulopoiesis

Usually mild anemia
Normal or increased platelets (rarely thrombocytopenia)

Front 20

Bone marrow in CML-CP

Back 20

hypercellular due increased neutrophils and precursors

Front 21

In the bone marrow in CML-CP, the paratrabecular cuff of myeloid precursors ___

Back 21

(in some cases) may be thickened to 5-10 cell layers

normal: 2-3 cell layers

Front 22

In the bone marrow in CML-CP, the blasts usually account for __% of marrow cells, but more than ___ indicates____

Back 22

usually <5%, but more than 10-19% indicates transformation to CML-AP

Front 23

In CML-CP bone marrow, the megakaryocytes are characteristically __

Back 23

Small in size (micromegakaryocytes) and hypolobated

Often increased in number (40-50% cases), othertimes normal or decreased

Front 24

Up to 40% of CML-CP patients present with ___ reticulin fibrosis in the marrow, this usually correlates with __

Back 24

Increased reticulin fibrosis

Increased reticulin fibrosis correlates with:
1. Increased numbers of megakaryocytes
2. Larger spleen size
3. More severe anemia

Front 25

Pseudo-___ cells and sea-blue ___ are seen in about 30% of CML-CP marrows

Back 25

pseudo-Gaucher cells
sea-blue histiocytes

These are commonly seen in marrow aspirates. They ARE derived from the neoplastic clone, BUT are secondary to increased cell turnover

Front 26

In CML-CP, megakaryocytes are characteristically __ and often ___ in number

Back 26

smaller than normal

increased

Front 27

Accelerated phase CML is characterized by one or more of the following:(5)

Back 27

1. Blasts 10-19% in BP and/or
BM

2. Peripheral basophilia
>/=20%

3a. Persistent
thromboCYTOPENIA (<100)
UNRELATED to therapy
3b. Persistent thromboCYTOSIS
UNRESPONSIVE to therapy

4. Worsening splenomegaly
with increasing WBC count,
unresponsive to therapy

5. Cytogenetic evidence of
clonal evolution

Front 28

In the setting of diagnosed CML-CP, the subsequent finding of marked granylocytic dysplasia or prominent proliferation of small, dysplastic megakaryocytes indicates disease __

Back 28

Progression (CML-AP)

Independent significance of these findings is unknown.

Front 29

In the setting of CML-CP, the finding of lymphoblasts in the blood or marrow is cause for concern, because ___

Back 29

May indicate a lymphoblastic transformation

Front 30

CML-BP resembles __

Back 30

Acute leukemia! but with the Ph chromosome and/or BCR/ABL gene fusion

Front 31

CML-BP is diagnosed if one or more of the following is present:

Back 31

1. Blasts >/= 20% PB or BM
nucleated cells

2. Extramedullary blast
proliferation

3. Large foci or clusters of
blasts in the bone marrow
BIOPSY (even if the remainder of the marrow shows chronic phase)

Front 32

In CML-BP, in __ % of cases, the blast lineage is ___, while in ___%, the lineage is____

Back 32

70% myeloblast
20-30% lymphoblast

rarely both

Front 33

Extramedullary blast proliferations, in the setting of CML, indicate __. They are found in ____

Back 33

Progression to CML-BP

Skin, lymph nodes, bone, spleen, CNS, anywhere

Front 34

How does the degree of myelofibrosis, in the setting of CML, effect the classification

Back 34

It does not.

Front 35

In the spleen, involvement by CML is a __ pulp process

Back 35

red pulp

Front 36

The neutrophils in CML-CP, have markedly __ neutrophilic alkalline phosphatase (LAP)

Back 36

decreased

Front 37

Neutrophils in CML-CP may demonstrate __ expression of CD15 and HLA-DR

Back 37

weak expression of normal neutrophil antigens (dimCD15, dimHLA-DR)

Front 38

In CML-BP, the myeloblast may or may not express ___,but will express ___ antigens

Back 38

myeloperoxidase (strong, weak, or absent)
Will express:
Myeloid/Monocytic (CD13, CD14, CD15, CD33, etc.)
Megakaryocytic (CD61)
Erythroid (glycophorin, HbA)

Not uncommonly, lympoid markers too!!

Front 39

Most cases of CML, with LYMPHOBLASTIC blast phase, are ____ immunophenotype.

Oftentimes, they also __

Back 39

precursor B (CD10, CD19, CD34, TdT, sIg-negative)

Often coexpress one or more myeloid markers

Front 40

At the time of diagnosis __% of CML have t(9;22)

Back 40

90-95%

The remaining cases:

1. Have either variant translocations involving a THIRD chromosome IN ADDITION to 9 and 22

OR

2. Have cryptic translocations of 9q34 and 22q11 that are not identified by routine cytogenetics

Front 41

t(9;22)(q34,q11)

Back 41

Ph chromosome [der(22q)]
BCR/ABL fusion gene

BCR gene on chromosome 22
ABl gene on chromosome 9

Front 42

Cryptic t(9;22)(q34,q11) can be detected by __

Back 42

FISH, RT-PCR, or Southern blot techniques

Front 43

The site of breakpoint on the BCR gene may influence the ___ in CML

Back 43

phenotype

Front 44

In CML, the breakpoint in the BCR gene is almost always in the ____ breakpoint cluster region, leading to the formation of an abnormal fusion protein ____, with increased __ activity

Back 44

Major breakpoint cluster region

p210 with increased tyrosine kinase activity

Front 45

In CML, other fusion proteins, due to alternate breakpoints in the BCR gene, include: ___ and ___

Back 45

p230: (RARE) patients may demonstrate CML with promient neutrophilic maturation

p190: most often associated with Ph positive ALL, HOWEVER, small amounts of p190 are detected in 90% of CML!!!

Front 46

CML with p190 BCR/ABL fusion protein

Back 46

Small amounts of p190 product can be detected in 90% of CML with the typical p210

BUT! p190 can also be associated with RARE CML cases with increased numbers of MONOCYTES, resembling CMML

Front 47

At the time of diagnosis of CML, chromosomal abnormalities in addition to Ph chromosome signify?

Back 47

Unknown significance!!

But, evidence of clonal evolution AFTER the diagnositic specimen usually signifies transformation

Front 48

Median survival times for treated CML range from __

Back 48

5-7 years

Front 49

Chronic neutrophilic leukemia is a ___ disease

Back 49

rare!! (only about 100 cases reported)

Front 50

CNL is characterized by:

Back 50

1. Sustained peripheral neutrophilia
2. BM hypercellularity due to neutrophilic granulocytes
3. hepatosplenomegaly
4. NO Ph chromosome or BCR/ABL fusion gene

Front 51

CNL is a diagnosis of ____!

Back 51

Exlusion

Rule out reactive neutrophilia and other MPDs

Front 52

20% of reported CNL cases are associated with ___

Back 52

an underlying neoplasm, most commonly multiple myeloma

Most of these of these "CNL" cases lack clonal chromosomal abnormalities and are most likely secondary proliferations due to abnormal cytokine production by myeloma cells

Front 53

The peripheral blood in CNL demonstrates:

Back 53

1. neutrophilia (>25), with mostly mature segmented forms, maybe increased bands, but few immature forms (<5%)

Front 54

In CNL, myeloblast are __ seen in the peripheral blood

Back 54

never

Front 55

In CNL, the neutrophils often appear ___, but can appear ___

Back 55

toxic, but can appear normal

NO! dysplasia

Front 56

The M:E ratio in the bone marrow in CNL may be up to

Back 56

20:1 or more with increased mature neutrophils and myelocytes, NO increased blasts or promyelocytes

Front 57

The variant of Ph+, BCR/ABL+ CML with a peripheral blood neurtophilia similar to that seen in CNL, is associated with a variant ___

Back 57

BCR/ABL fusion protein (p230)

Front 58

A clonal proliferation of eosinophilic precursors resulting in persistently increased peripheral eosinophils, bone marrow eosinophils, and peripheral tissue eosinophilia?

Back 58

Chronic eosinophilic leukemia (CEL)

Front 59

What percentage of Chronic eosinophilic leukemia (CEL)cases are associated with Ph chromosome?

Back 59

NONE!!
Neither t(9;22) nor BCR/ABL fusion gene

Front 60

The blast% in CEL is by definition:

Back 60

<20% in the blood and bone marrow

Front 61

To make a diagnosis of CEL, there should be evidence of ___ or ____

Back 61

- clonality among the eosinophils
or
- increased blasts in blood or BM

Front 62

Persistent eosinophilia without identifiable cause, without evidence of clonality, without an abnormal T cell population with an aberrant phenotype, and without increased blasts?

Back 62

Idiopathic hypereosinophilic syndrome (HES)

Front 63

When considering a diagnosis of CEL/HES, you should rule out secondary causes of eosinophilia such as:

Back 63

Reactive eosinophilia:
-allegry
-parasites
-infectious disease
-pulmonary diseases (hypesensitivity pneumonitis, Loeffler's syndrome)
-collagen vascular disease

Exclude underlying neoplasia with secondary, reactive eosinophilia:
-T cell lymphomas
-HL
-ALL
-Mastocytosis

Front 64

Neoplastic disorders that can be associated with eosinophilia as PART of the clonal process include:

Back 64

1. CML (Ph+, BCR/ABL+)
2. AML, inlcuding AML with inv(16), t(16;16)
3. other CMPDs (PV, ET, CIMF)
4. MDS

Front 65

Hypereosinophilic syndrome (HES) is more common in __

Back 65

men 9:1

Front 66

CEL/HES is a multisystem disease affecting the ___ and ___ always, plus __

Back 66

blood and bone marrow always

tissue infiltration

Front 67

Most serious complication of CEL/HES is __

Back 67

endomyocardial fibrosis with resultant restrictive cardiomegaly

secondary to prolonged eosinophilia

Front 68

In the peripheral blood of CEL/HES the most strikining finding is __

Back 68

eosinophilia with mainly mature eos, only small numbers of eosinophilic myelocyts and promyelocytes

eos may show a range of morphologic abnormalities

Front 69

In considering between CEL and HES, if blasts are >2%, ___ should be considered

Back 69

CEL

However, blasts are typically infrequent to absent in most cases of CEL/HES

Front 70

ALL cases associated with eosinophilia are often associated with ___

Back 70

t(5;14)(q31;q32)

Front 71

PV is characterized by increased RBC production that is ___

Back 71

independent of the normal regulatory mechanisms

Front 72

Two phases of PV are recognized:

Back 72

1. polycythemic phase - initial proliferative phase associated with increased RBC mass

2. post-polycythemic phase or spent phase - cytopenias (including anemia) due to ineffective hematopoiesis, bone marrow fibrosis, and associated extramedullary hematopoiesis and hyperplenism

Front 73

WHO criteria for the diagnosis of PV: A1 and A2 must be present

Back 73

A1: elevated RBC mass (HCT)>25% above the mean normal predicated value OR HB>18.5 g/dl in men and >16.5 g/dl in women

A2: No cause of secondary erythrocytosis (familial erythrocytosis; no elevation of EPO)

p.32

Front 74

Mean age at diagnosis of PV

Back 74

60

rare < 20

Front 75

In PV, splenomegaly and hepatomegaly are due to __

Back 75

extramedullary hematopoiesis in the later stages

Front 76

Major symptoms of PV are due to ___ or ___

Back 76

1. HTN
2. vascular abnormalities due to increased RBC mass

Front 77

25% of patients with PV present with __

Back 77

venous or arterial thrombosis (DVT, AMI, stroke)

Front 78

Mesenteric, splenic, and portal venous thrombosis should lead to a diagnostic consideration of __

Back 78

PV

Front 79

In PV (polycythemic stage) the BM exhibits __

Back 79

1. normoblastic erythroid proliferation
2. typically hyercellular for age
3. panmyelosis (increased erythroid, megakaryocytic, and granulocytic proliferation)
4. increase megakaryocytes even if normocelluar overall

Front 80

In PV (polycythemic stage) the PB exhibits __

Back 80

1. excess normochromic, normocytic RBCs (increased HCT, Hb)
2. oftentimes - neutrophilia and basophilia
3. thrombocytosis (50%)

Front 81

In PV, stainable iron is __ in the marrow aspirate

Back 81

lacking!!

Front 82

In PV (post-polycythemic phase), the red cell mass __ and then ___, while the spleen ___

Back 82

normalizes and then decreases, while the spleen gets larger (EMH)

Front 83

In PV, the most common pattern of disease progression is ____ and ___

Back 83

post-polycythemic myelofibrosis and myeloid metaplasia (PPMM)

Front 84

PPMM is characterized by:

Back 84

1. leukoerythroblastic blood
smear due to EMH
2. myelofibrosis (hallmark), reticulin and collagen fibrosis

Front 85

In PPMM bone marrow, clusters of ___ are prominent

Back 85

hyperchromatic, dysmorphic megakaryocytes

Front 86

In PV (spent phase), the finding of >10% blasts in the blood or marrow or significant myelodysplasia are both ____,but may signfiy:

Back 86

unusal!!

transformation to an MDS or acute leukemia

Front 87

In PV there are ___ unique immunophenotypic findings or specific genetic abnormalites

Back 87

No

Front 88

Chronic idiopathic myelofibrosis (CIMF) is aka ___

Back 88

agnogenic myeloid metaplasia or myelofibrosis with myeloid metaplasia

Front 89

CIMF is a clonal stem cell disorder characterized by:

Back 89

1. dysplastic megakaryocytic hyerplasia
2. relative increase in immature granulocytes
3. ineffective erythropoiesis
4. reactive reticulin or collagen myelofibrosis
5. EMH (myeloid metaplasia)

(use of MDS buzz words: dysplastic, ineffective)

Front 90

CIMF can have overlapping features with __ or __, therefore, it is a diagnosis made after __

Back 90

other CMPDs or MDSs

exluding of clonal and non-clonal process that can be associated with myelofibrosis

Front 91

CIMF has two phases

Back 91

Prefibrotic stage
Fibrotic stage

Front 92

Pre-fibrotic CIMF is characterized by a ___ marrow with ___ reticulin or collagen fibrosis

Back 92

hypecelluar!!

minimal

Front 93

Fibrotic stage CIMF is characterized by __ marrow reticulin or collagen fibrosis and a peripheral blood ___

Back 93

marked

leukoerythroblastosis due to EMH with splenomegaly and hepatomegaly

Front 94

CIMF is a __ disorder that occurs most commonly in the ___ decade of life

Back 94

rare

7th decade (70's)

Front 95

A significant percentage of patients are ___ at the time of diagnosis of CIMF

Back 95

asymptomatic (up to 30%)

detection of splenomegaly on routine exam or abnormal CBC

Front 96

___ % of CIMF paitents are diagnosed in the prefibrotic (cellular) stage

Back 96

20-30%

Front 97

Prefibrotic CIMF, findings include

Back 97

-mild to moderate anemia
-some evidence of EMH (leukoerythroblastosis), usually minimal
-HYPERCELLULAR bone marrow
-megakaryocytes are markedly abnormal: key to recognizing the prefibrotic stage of CIMF

Front 98

The key to recognizing prefibrotic stage CIMF in the marrow is ___

Back 98

the abnormal megakaryocytes

-clusters of variable sizes adjacent to sinuses and trabeculae
-most are enlarged megaks, but some small too
-abnormal chromatin and nuclei
-freqent NAKED megak nuclei

OVERALL, the megaks in CIMF are MORE aytpical than those seen in other CMPDs

Front 99

Most patients with CIMF are diagnosed in the ___ stage

Back 99

fibrotic stage (70-80%) with hepatosplenomegaly due to EMH, and anemia with a leukoerythroblastic PB smear

Front 100

The finding of dysgranulopoiesis or >10% blasts, in the setting of CIMF, suggest _

Back 100

transformation to a more aggressive phase (accelerated or blast phase)

Front 101

In fibrotic stage CIMF the bone marrow is often ____

Back 101

normocellular or hypocelluar, with PATCHES of hematopoiesis separated by regions of loose connective tissue or fat

Note: BM can be hypercellular

Front 102

In fibrotic stage CIMF, the marrow sinuses are characteristically __

Back 102

increased in number and dilated with intrasinusoidal hematopoiesis

Front 103

In fibrotic stage CIMF, the most notable marrow cell type is most often __

Back 103

the megakaryocytes, which are aytpical and occur in clusters and sheets or within dilated sinuses

Front 104

Osteoslcerosis can be seen in CIMF marrow, resulting in

Back 104

broad, irregular trabeculae that can occupy up to 50% of the marrow space

Front 105

In patients with a previous diagnosis of CIMF, the subsequent finding of 10-19% blasts and >/=20% blasts indicate

Back 105

10-19% blasts (PB or BM) - accelerated phase

>/=205 blasts - transformation

Front 106

Occasional patients present initially with accelerated phase CIMF

Back 106

DDX:
- MDS w/ fibrosis
- MDS/MPD unclassifiable
- panmyelosis with fibrosis

The clue to the diagnosis of CIMF is the characteristic morphologic findings, plus these DDX lack the characteristic hepatosplenomegaly

Front 107

CIMF may present in the acute phase (>/= 20% blasts), in this case the diagnosis should be __

Back 107

AML, with only mention of the possibility that this may have derived from CIMF

Front 108

There are myeloid, lymphoid and non-hematologic disorders that are associated with myelofibrosis

Back 108

Myeloid:
-CIMF
-AML
-Acute myelofibrosis/acute megakaryoblastic leukemia
-CML
-MDS/MPD
-PV
-CMML
-CEL/HES
-Systemic mastocytosis

Lymphoid;
-HCL
-HL
-NHL
-MM
-ALL
Non-hematologic:
-metastatic tumor
-connective tissue disease
-HIV
-renal osteodystrophy
-tuberculosis
-hyperparathyroidism
-vitamin D deficiency (rickets)
-grey platelet syndrome

Front 109

Essential thrombocythemia (ET) is a clonal proliferation primarily of the ___ lineage

Back 109

megakaryocytic

Front 110

ET is characterized by ____ thrombocytosis in teh blood and ___ in the marrow

Back 110

sustained thrombcytosis in the PB

increased numbers of large, mature megakaryocytes in the BM

Front 111

The difference between the megakaryocytic proliferations in ET vs. CIMF

Back 111

ET: increased numbers of large to giant, mature megak's with abundant cytoplasm, deeply lobulated and hyperlobulated nuclei; megak's may aggregate in loose clusters or be dispersed

CIMF: proliferation of dysplastic, bizzare, atypical megak's forming clusters and/or sheets, as well as intrasinusoidal

Front 112

Clinically ET is characterized by

Back 112

presentation in middle age with an indolent course, with long asymptomatic periods, with occasional episodes of thrombosis and/or hemorrhage

Front 113

In ET, the spleen is a site for __, but there is relatively little ___

Back 113

platelet sequestration

little EMH

Front 114

At presentation, __% of patients are asymptomatic

Back 114

about 50% are asymptomatic, and discovered fortuitously

20-50% present some vascular occlusive event (stroke, TIA, AMI, digital ischemia) or mucosal hemorrhage (GI tract, pulmonary)

Front 115

Diagnositic criteria for ET
include __ positive criteria and __ criteria of exclusion

Back 115

(2) positive criteria:
1. sustained platelet count
>/=600
2. marrow shoing proliferation of megak's with many large, mature megak's

(5) criteria of exlusion
1. No evidence of PV
-normal HCT, Hb, stainable
iron, nml serum ferritin
and MCV (or atleast
correctable with iron if
needed)
2. No evidence of CML
- no Ph chromosome or
BCR/ABL fusion gene
3. No evidence of CIMF
- no collagen fibrosis
- no significant reticulin
fibrosis
4. No evidence of MDS
5. No evidence of secondary
cause of thrombocytosis

Front 116

Secondary causes of sustained thrombocytosis include:

Back 116

1. infection or inflammation
2. underlying neoplasm
3. prior splenectomy

Front 117

Most striking PB smear finding in ET is

Back 117

thrombocytosis
- platelet aniscytosis (tiny to large)
- no significant numbers of bizarre forms

- WBC count normal w/ normal differential
- RBCs normal (unless hemorrhage leading to iron def)
- no leukoerythroblastosis becuase EMH is NOT significant part of ET

Front 118

In ET the marrow is typically ___ cellular, with the most striking finding being __

Back 118

normocellular or slightly hypercellular

marked megak proliferation with large to giant forms

no increased blasts or significant dysplasia

Front 119

The presence of significant reticulin or ANY collagen fibrosis speaks strongly in ___ of a diagnosis of ET

Back 119

against!!

Front 120

A common finding in megak's in ET is ___(although not specific for ET)

Back 120

emperipolesis of marrow cells

Front 121

There is ___ specific cytogenetic or molecular genetic abnormality known for ET, but ___

Back 121

No!

But cytogenetic and molecular studies should be done to rule out CML as the cause of thrombocytosis

besides, an abnormal karyotype is unusual in ET

Front 122

Is splenectomy the treatment of choice of ET?

Back 122

NO!!!

It could lead to a dramatic increase in the platelet count.

Front 123

Is transformation of ET to an AML or MDS common?

Back 123

NO! only 5% or less

Front 124

Given the proliferation of megak's in the marrow of ET patients, what percentage develop marked myelofibrosis

Back 124

Not many, it is uncommon.

Plus, at the time of diagnosis, myelofibrosis speaks stronly against a diagnosis of ET

Front 125

CMPD, unclassifiable should be followed up every ___ until a more specific diagnosis can be made

Back 125

4-6 months will usually provide sufficient information

Front 126

Before making the diagnosis of CMPD, U ___

Back 126

rule out secondary causes of myelofibrosis that can mimic a MPD

Front 127

In late stage disease with marked myelofibrosis and/or osteomyelosclerosis (terminal or burnt-out stage) the distinction between ___ and ___ may be impossible, without previous history

Back 127

fibrotic stage CIMF and post-polycythemic stage PV

Front 128

A clue that one is dealing with CML with marked myelofibrosis and not an unclassifiable CMPD would be __

Back 128

finding micromegak's and Ph chromosome and/or BCR/ABL gene fusion

Front 129

As with other CMPD's, CMPD, unclassifiable can progress to accelerated stage with ___ and/or ____

Back 129

10-19% blasts in the PB and/or marrow

and/or

acute leukemia (>/= 20% blasts) and the suggestion that this may be a blast transformation of a previous but unclassifiable CMPD

Front 130

Prognosis for ET, CIMF,PV, and CML

Back 130

ET: indolent, presents in midlife so may not significantly effect longevity, median survival 1-15 yrs

CIMF: median survival 3-5 years

PV: without therapy only a few months to live, but with therapy 10 years is not uncommon, alsot presents in midlife

CML: median survival is 5-7 years with therapy, also commonly presents in midlife