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Hematology > Polycythemia Vera and Other Myeloproliferative Diseases > Flashcards

Polycythemia Vera and Other Myeloproliferative Diseases Flashcards

1
Q

role of chemotherapy in reducing RBC mass in PV

A

no role

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1
Q

Coagulation studies in ET

A

PT and aPTT are normal

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2
Q

So2 in PV

A

Normal

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2
Q

synonyms for chronic PMF

A

idiopathic myelofibrosis

agnogenic myeloid metaplasia

myelofibrosis with myeloid metaplasia

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3
Q

Most common Chronic myeloproliferative disorder

A

PV

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4
Q

Causes of microcytic erythrocytosis

A

ß thalassemia trait

hypoxic erythrocytosis

PV

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5
Q

Treatment of erythromelalgia

A

salicylates

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5
Q

Anagrelide

A

Phosphodiesterase inhibitor

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6
Q

Feature of red cell mass elevation in PV

A

SYSTOLIC hypertension

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6
Q

Non malignant causes of myelofibrosis

A

HIV infection

Hyperparathyroidism

Renal osteodystrophy

Systemic lupus erythematosus

Tuberculosis

Vitamin D deficiency

Thorium dioxide exposure

Gray platelet syndrome

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7
Q

WHO Classification of Chronic Myeloproliferative Disorders

A

Chronic myelogenous leukemia, bcr-abl–positive

Chronic neutrophilic leukemia

Chronic eosinophilic leukemia, not otherwise specified

Polycythemia vera

Primary myelofibrosis

Essential thrombocytosis

Mastocytosis

Myeloproliferative neoplasms, unclassifiable

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7
Q

Anticoagulants in PV

A

Only if thrombosis occurs

monitoring of PT,aPTT is difficult with erythrocytosis

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7
Q

least common chronic MPD

A

PMF

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8
Q

Causes of relative erythrocytosis

A

Dehydration

diuretics

alcohol

androgen

tobacco

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9
Q

Tumors causing absolute erythrocytosis?

A

Hypernephroma

hepatoma

cerebellar hemangioblastoma

uterine myoma

adrenal tumors

meningioma

pheochromocytoma

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10
Q

Treatment of hyperuricemia in PV

A

No Rx for asymptomatic hyperuricemia< 10mg%

admn allopurinol when chemotherapy is started

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11
Q

Features of exuberant extramedullary hematopoeisis in pmf

A

ascites

portal/pulmonary/intracranial hypertension

intestinal or ureteral obstruction

cardiac tamponade

spinal cord compression

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11
Q

Why Hydroxyurea is effective in preventing TIA

A

It is a NO donor

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12
Q

Diagnosis and management of acquired vWF deficiency in ET

A

Ristocetin cofactor assay

Avoid aspirin

Treat with epsilon aminocaproic acid

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13
Q

Translocation in CNL

A

t(15;19)

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13
Q

anagrelide in PV

A

Reduces platelet count

prevents thrombosis

no marrow toxicity

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14
Q

Common mutation in PV,PMF,ET

A

JAK2 mutation,V617F

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16
Q

Only symptom that distinguishes PV from other causes of erythrocytosis

A

aquagenic pruritis

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17
Q

Target Hb level in PV

A

Males:

females:

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18
Q

Cause of pruritis in PV?

A

basophil activaton by JAK2 mutation

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18
Q

Phenotypic correlation of mpl mutation in PMF

A

more anemia

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19
Q

Blood picture characteristic of PV

A

erythrocytosis

thrombocytosis

leukocytosis

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20
Q

sex prediliction for ET

A

females

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21
Q

Drugs effective for prevention of TIA in ET

A

Hydroxyurea and aspirin are more effective than anagrelide and aspirin but not more effective for the prevention of other types of arterial thrombosis and are actually less effective for venous thrombosis

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22
Q

Factor essential for transformation of ET to PV

A

JAK2 mutation

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22
Q

bone x rays in PMF

A

osteosclerosis

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23
Q

Bleeding disorder caused by PV

A

acquired von wilibrand disease

adsorption and proteolysis of high-molecular-weight von Willebrand factor (vWF) multimers by the expanded platelet mass

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24
Q

Absent bone marrow iron in the presence of marrow hypercellularity

A

polycythemia vera

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25
Q

Hypoxic causes of absolute erythrocytosis

A

High altitude

R to L shunts

Hepatopulmonary syndrome

High affinity Hb

pulmonary disease

sleep apnea syndrome

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25
Q

mutations in PMF

A

JAK2

MPL(thrombopoeitin receptor)

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26
Q

role of salicylates in prevention of thrombosis in PV?

A

No role

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26
Q

Demography of PMF

A

male,60 yrs and above

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27
Q

risk factors for poor prognosis in PMF

A

Age >65 years
Constitutional symptoms
Hemoglobin
WBC >25 x 109/L
Blood blasts >10%

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29
Q

In PV,Intraabdominal venous thrombosis is common in

A

women

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30
Q

Why RBC mass and plasma volume determinations are essential in PV?

A

Plasma volume expansion in PV may mask elevated red cell mass

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31
Q

Myelofibrosis in PV

A

Myelofibrosis appears to be part of the natural history of the disease but is a reactive, reversible process that does not itself impede hematopoiesis and by itself has no prognostic significance

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32
Q

migrane that occurs in PV

A

ocular migrane

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32
Q

Malignant disorders causing myelofibrosis

A

Acute leukemia (lymphocytic, myelogenous, megakaryocytic)

Chronic myelogenous leukemia

Hairy cell leukemia

Hodgkin’s disease

Idiopathic myelofibrosis

Lymphoma

Multiple myeloma

Myelodysplasia

Metastatic carcinoma

Polycythemia vera

Systemic mastocytosis

32
Q

unique feature of megakaryocytes

A

endomitotic reduplication

32
Q

risk of GI bleeding is increased if aspirin is combined with which drug in ET?

A

anagrelide

34
Q

Hypoxic erythrocytosis with normal So2

A

High oxygen affinity Hb disorder

35
Q

Role of BMA and biopsy in PV

A

Provide no specific diagnostic information since these may be normal or indistinguishable from ET or PMF

37
Q

Renal diseases causing absolute erythrocytosis

A

Renal artery stenosis

Focal sclerosing or membranous glomerulonephritis

Postrenal transplantation

Renal cysts

Bartter’s syndrome

38
Q

Stress erythrocytosis

A

gaisbock syndrome

39
Q

Type of thrombosis in PV

Venous or arterial

A

Both

40
Q

Only test to distinguish between PV and ET is

A

RBC mass and plasma volume determination

42
Q

cytogenetic abnormalities commonly associated with PV

A

trisomy 8,trisomy 9

20q-

43
Q

Frequency of phlebotomy in polycythemia vera

A

Till iron deficiency is induced

after that once in 3 months

44
Q

LDH in pmf

A

elevated

45
Q

JAK2 is cognate tyrosine kinase for

A

erythropoietin and thrombopoietin receptor

46
Q

Bone marrow studies in ET

A

BMA may be dry

Bone marrow biopsy: megakaryocyte hyperplasia and hypertrophy, as well as an overall increase in marrow cellularity

47
Q

Age group affected in PV

A

all age groups

48
Q

Lab artifacts due to ET

A

Hyperkalemia

Arterial O2 measurements

50
Q

Difference between true erythrocytosis and plasma volume contraction

A

Hb> 20g/dl or HCT> 60

50
Q

MPD commonly associated with night sweats, fatigue, and weight loss

A

PMF

50
Q

Spleen in ET

A

Mild

Massive splenomegaly is indicative of another MPD, in particular PV, PMF, or CML

51
Q

Chronic MPNs that are a diagnosis of exclusion

A

PMF

ET

52
Q

myelophthisis

A

marrow fibrosis secondary to tumor or granuloma

54
Q

PV pts more prone to thrombosis are

A

Those with massive splenomegaly

erythrocytosis masked by increased plasma volume

55
Q

Features favouring MDS over ET

A

anemia

ringed sideroblasts

56
Q

autoimmune abnormalities in PMF

A

ANA

RF

positive coombs test

57
Q
A

extramedullary hematopoisis

tear drop RBC

59
Q

Indications for treatment of thrombocytosis in PV

A

bleeding diathesis

ocular migrane

erythromelalgia

60
Q

Which correlates with thrombosis in PV?

erythrocytosis or thrombocytosis

A

erythrocytosis

61
Q

Causes of anemia in PV

A

GI bleed due to PUD

Leukemic transformation

massive splenomegaly(plasma volume expansion)

63
Q

Cause of microcytic hypochromic anemia in PV

A

occult GI bleed due to PUD

64
Q

Pulmonary complication in PV

A

Pulmonary hypertension due to fibrosis and extramedullary hematopoeisis

65
Q

Absolute erythrocytosis with elevated plasma erythropoeitin

A

Hypoxic or autonomous erythropoietion production

67
Q

location of JAK2 gene

A

chromosome 9p

67
Q

PV should be suspected in any patient who develops

A

hepatic vein thrombosis

68
Q

PMF pts with JAK2 mutations

A

50%

70
Q

familial causes(with normal Hb function) of absolute erythrocytosis

A

Erythropoietin receptor mutation

VHL mutations (Chuvash polycythemia)

2,3-BPG mutation

72
Q

LAP in PMF

A

low, normal, or high

73
Q

Number of circulating CD34+ cells in PMF

A

>15,000/µL

74
Q

LAP score in PV

A

elevated

75
Q

cytogenetics in PMF

A

9p-

13q-

20q-

trisomy 8,9

partial trisomy 1q

75
Q

Blood smear in PMF

A

teardrop/nucleated RBCs

myelocytes,promyelocytes,myeloblasts

anemia(occurs as a rule)

leukocyte and platelet count may be normal or increased or depressed

76
Q

development of leukemia in PV is related to

A

chemotherapy

development of extramedullary hematopoeisis,hepatosplenomegaly,transfusion dependant anemia

77
Q

Blood studies in ET

A

Anemia is uncommon

Mild neutrophilic leukocytosis may be seen

78
Q

Preferred bcr-abl assay in thrombocytosis patients with negative cytogenetic study for philadelphia chromosome

A

FISH

bcr-abl reverse transcriptase polymerase chain reaction is associated with false-positive results

80
Q

drugs used in PV

A

Interferon alpha(reduces splenomegaly)

Pegylated IFN

Hydroxyurea

anagrelide

82
Q

Difference btw JAK2 mutation positive and negative Myeloproliferative disorders

A

JAK2 mutation positive

Disease course in decades

transformation to acute leukemia is rare

83
Q

hepatomegaly in PMF

A

Mild hepatomegaly may accompany the splenomegaly but is unusual in the absence of splenic enlargement

84
Q

ET complication that requires reduction in platelet count

A

migrane

86
Q

Negative predictive value of plasma erythropoeitin in erythrocytosis

A

low

A normal erythropoietin level does not exclude a secondary cause for erythrocytosis or PV

87
Q

Rx of pruritis in PV

A

anti histaminics

anti depressants like doxepin

hydroxy urea

Interferon alpha

PUVA

88
Q

Comparison of erythropoietin and thrombopoietin

A
89
Q

Is JAK V617F mutation diagnostic of PV?

A

NO

Not every patient with PV expresses this mutation, while patients without PV do

90
Q

Neurologic symptoms in PV

A

Due to Hyperviscosity

headache

vertigo

tinnitus

visual disturbance

TIA

92
Q
A

marrow fibrosis

94
Q

Rx of polycythemia vera

A

phlebotomy

96
Q

Drugs causing absolute erythrocytosis?

A

Androgens

Recombinant erythropoietin

98
Q

Diff btw ß thalassemia trait and other causes of microcytic erythrocytosis

A

normal RDW

Increased RDW in other causes due to iron deficiency

99
Q

Clinical features of PV

A

Splenomegaly

Hyperviscosity syndrome

thrombosis

Systolic hypertension

Hyperuricemia: secondary gout,uric acid stones

Thrombocytosis complications:

Digital ischemia

epistaxis

easy bruising

PUD

GI hemorrhage

Erythromelalgia(Erythema,burning and pain in extremities)

100
Q

Cytogenetics in CEL

A

deletion or balanced translocation involving PDGFR alpha

101
Q

Increase in marrow reticulin in ET

A

consider alternative diagnosis

102
Q

Factors necessary for megakaryopoeisis

A

IL-3

SCF

SDF-1(stromal cell derived factor)

103
Q

Thrombosis of which vessels commonly occurs in PV

A

cerebral

cardiac

mesenteric

104
Q

Causes of thrombocytosis

A

collagen vascular disease, inflammatory bowel disease

malignancy

infection

Myeloproliferative disorders: polycythemia vera, primary myelofibrosis, essential thrombocytosis, chronic myelogenous leukemia

Myelodysplastic disorders: 5q-syndrome, idiopathic refractory sideroblastic anemia

Postsplenectomy or hyposplenism

hemorrhage

iron deficiency anemia

hemolysis

surgery

rebound:Correction of vitamin B12 or folate deficiency, post-ethanol abuse

Thrombopoietin overproduction, constitutive Mpl activation

105
Q

Glucocorticoids in PMF

A

improvement of anemia when used alone or with thalidomide

control constitutional symptoms

autoimmune complications

106
Q

Splenectomy in PMF

A

increase risk blastic transformation

107
Q

Lymphadenopathy in PMF

A

isolated lymphadenopathy should suggest another diagnosis

Hematology (9 decks)

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