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Hematology > Myeloproliferative Disorders > Flashcards

Myeloproliferative Disorders Flashcards

1
Q

Myeloproliferative disorders (MPDs)

A

includes leukemias & disorders
MPDs are not clearly malignant but are characterized by neoplastic proliferation of hematopoietic precursors
affects the myeloid line
acquired, chronic disorders that may transform into acute leukemias

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2
Q

CML Variants - Juvenile CML

A

rare
10-14 yrs old
similar to adult CML

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3
Q

CML Variants - Chronic Neutrophilic leukemia

A 
sustained increase in neutrophils
VERY rare
median age - 67 yrs
most patients are asymptomatic
Ph chromosome NEGATIVE!!!
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4
Q

CML Variants - Chronic Neutrophilic Leukemia lab findings

A

neutrophilia >80%
increased LAP
poor prognosis

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5
Q

CML Variants - Chronic Eosinophilic leukemia (CEL)

A

Ph chromosome negative
middle aged men
release of eosinophilic granules cause damage to digits & organs
30-70% eos

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6
Q

CML Variants - Chronic basophilic leukemia

A

rarest variant of CML

40-80% basophils

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7
Q

Polycythemia Vera (PV) General Characteristics

A

AKA polycythemia rubra vera, primary polycythemia
unregulated proliferation of the erythroid elements in the BM
increase RBCs in PB

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8
Q

Epidemiology of PV

A

relatively rare
20-80 yrs
frequently in men than women
more common in whites from jewish descent

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9
Q

Eitology of PV

A

acquired mutation

EPO hypersensitivity

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10
Q

Symptoms of PV

A

related to the thickened blood
red coloration, especially of the face
vascular occlusion or thrombosis (clots)
myocardial infarction - cardiovascular diseases

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11
Q

Lab findings of PV

A 
erythrocytosis
elevated hgb
elevated hct
no feathered edge
thrombocytosis - giant platelets
LAP increased
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12
Q

Treatment of PV

A

no cure
therapeutic phlebotomy
myelosuppressive drugs

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13
Q

Complications of PV

A

may lead to AML

cardiovascular disease

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14
Q

Prognosis of PV

A

median survival w/ treatment: 11-15 yrs
w/o treatment - 18 months
major cause of death is strokes / heart attack

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15
Q

Secondary Polycythemia

A

increased EPO due to tissue hypoxia or due to renal tumors

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16
Q

Relative Polycythemia

A

mild polycythemia due to dehydration resulting in hemoconcentration
will have normal WBC & PLT counts

17
Q

Essential Thrombocythemia (ET)

A

large # of abnormal platelets that cause clotting & bleeding / hemorrhaging.

18
Q

Hallmark of ET

A

thrombocytosis greater than 600 x 10 ^9

all other cells in the blood are normal

19
Q

Eitology of ET

A

acquired mutation

cause unknown

20
Q

Epidemiology of ET

A

rare disorder
older age group (65)
affects males & females equally

21
Q

Symptoms of ET

A

often asymptomatic
thrombosis
hemorrhage from nose, gums, GI tract

22
Q

Lab findings of ET in PB

A

thrombocytosis > 600
megakaryocytes
giant, bizarre platelets

23
Q

Lab findings of ET in BM

A

hypercellular

large increase in abnormal megakaryocytes (increased size, clustering, increased lobules)

24
Q

treatment of ET

A

anticoagulants & drugs to inhibit platelet function

25
Q

Prognosis of ET

A

10+ years

common cause of death are clots & bleeding

26
Q

Chronic Idiopathic Myelofibrosis (CIMF)

A

AKA agnogenic myeloid metaplasia (AMM), myelofibrosis w/ myeloid metaplasia (MMM), and primary fibrosis (WHO 2008)
scarring of the bone marrow

27
Q

CIMF pathophysiology

A

scarring of bone marrow causing extramedullary hematopoiesis
Fibroblasts are collagen producing cells that eventually disrupt normal architecture of the marrow & replaces hematopoietic tissue

28
Q

Hallmark of CIMF

A

bone marrow is replaced by collagen - DRY TAP aspirate

29
Q

Epidemiology of CIMF

A

uncommon disease
very chronic - gradual onset
older patients > 50 yrs

30
Q

Symptoms of CIMF

A

may be asymptomatic
pain in extremities
splenomegaly & hepatomegaly

31
Q

Lab findings of CIMF

A 
normo, normo anemia
striking aniso & poik
TEAR DROP CELLS
basophilic stippling
left shift expected
platelet count is variable!!!
32
Q

lab findings in BM of CIMF

A

DRY TAP- no aspirate
hypercellular
presence of fibrosis

Hematology (14 decks)

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