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Hematology > Polycythemia Vera and Other Myeloproliferative Diseases > Flashcards

Polycythemia Vera and Other Myeloproliferative Diseases Flashcards

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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
Cause of pruritis in PV?
basophil activaton by JAK2 mutation
18
Phenotypic correlation of mpl mutation in PMF
more anemia
19
Blood picture characteristic of PV
erythrocytosis thrombocytosis leukocytosis
20
sex prediliction for ET
females
21
Drugs effective for prevention of TIA in ET
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
22
Factor essential for transformation of ET to PV
JAK2 mutation
22
bone x rays in PMF
osteosclerosis
23
Bleeding disorder caused by PV
acquired von wilibrand disease adsorption and proteolysis of high-molecular-weight von Willebrand factor (vWF) multimers by the expanded platelet mass
24
Absent bone marrow iron in the presence of marrow hypercellularity
polycythemia vera
25
Hypoxic causes of absolute erythrocytosis
High altitude R to L shunts Hepatopulmonary syndrome High affinity Hb pulmonary disease sleep apnea syndrome
25
mutations in PMF
JAK2 MPL(thrombopoeitin receptor)
26
role of salicylates in prevention of thrombosis in PV?
No role
26
Demography of PMF
male,60 yrs and above
27
risk factors for poor prognosis in PMF
Age \>65 years Constitutional symptoms Hemoglobin WBC \>25 x 109/L Blood blasts \>10%
29
In PV,Intraabdominal venous thrombosis is common in
women
30
Why RBC mass and plasma volume determinations are essential in PV?
Plasma volume expansion in PV may mask elevated red cell mass
31
Myelofibrosis in PV
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
32
migrane that occurs in PV
ocular migrane
32
Malignant disorders causing myelofibrosis
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
unique feature of megakaryocytes
endomitotic reduplication
32
risk of GI bleeding is increased if aspirin is combined with which drug in ET?
anagrelide
34
Hypoxic erythrocytosis with normal So2
High oxygen affinity Hb disorder
35
Role of BMA and biopsy in PV
Provide no specific diagnostic information since these may be normal or indistinguishable from ET or PMF
37
Renal diseases causing absolute erythrocytosis
Renal artery stenosis Focal sclerosing or membranous glomerulonephritis Postrenal transplantation Renal cysts Bartter's syndrome
38
Stress erythrocytosis
gaisbock syndrome
39
Type of thrombosis in PV Venous or arterial
Both
40
Only test to distinguish between PV and ET is
RBC mass and plasma volume determination
42
cytogenetic abnormalities commonly associated with PV
trisomy 8,trisomy 9 20q-
43
Frequency of phlebotomy in polycythemia vera
Till iron deficiency is induced after that once in 3 months
44
LDH in pmf
elevated
45
JAK2 is cognate tyrosine kinase for
erythropoietin and thrombopoietin receptor
46
Bone marrow studies in ET
BMA may be dry Bone marrow biopsy: megakaryocyte hyperplasia and hypertrophy, as well as an overall increase in marrow cellularity
47
Age group affected in PV
all age groups
48
Lab artifacts due to ET
Hyperkalemia Arterial O2 measurements
50
Difference between true erythrocytosis and plasma volume contraction
Hb\> 20g/dl or HCT\> 60
50
MPD commonly associated with night sweats, fatigue, and weight loss
PMF
50
Spleen in ET
Mild Massive splenomegaly is indicative of another MPD, in particular PV, PMF, or CML
51
Chronic MPNs that are a diagnosis of exclusion
PMF ET
52
myelophthisis
marrow fibrosis secondary to tumor or granuloma
54
PV pts more prone to thrombosis are
Those with massive splenomegaly erythrocytosis masked by increased plasma volume
55
Features favouring MDS over ET
anemia ringed sideroblasts
56
autoimmune abnormalities in PMF
ANA RF positive coombs test
57
extramedullary hematopoisis tear drop RBC
59
Indications for treatment of thrombocytosis in PV
bleeding diathesis ocular migrane erythromelalgia
60
Which correlates with thrombosis in PV? erythrocytosis or thrombocytosis
erythrocytosis
61
Causes of anemia in PV
GI bleed due to PUD Leukemic transformation massive splenomegaly(plasma volume expansion)
63
Cause of microcytic hypochromic anemia in PV
occult GI bleed due to PUD
64
Pulmonary complication in PV
Pulmonary hypertension due to fibrosis and extramedullary hematopoeisis
65
Absolute erythrocytosis with elevated plasma erythropoeitin
Hypoxic or autonomous erythropoietion production
67
location of JAK2 gene
chromosome 9p
67
PV should be suspected in any patient who develops
hepatic vein thrombosis
68
PMF pts with JAK2 mutations
50%
70
familial causes(with normal Hb function) of absolute erythrocytosis
Erythropoietin receptor mutation VHL mutations (Chuvash polycythemia) 2,3-BPG mutation
72
LAP in PMF
low, normal, or high
73
Number of circulating CD34+ cells in PMF
\>15,000/µL
74
LAP score in PV
elevated
75
cytogenetics in PMF
9p- 13q- 20q- trisomy 8,9 partial trisomy 1q
75
Blood smear in PMF
teardrop/nucleated RBCs myelocytes,promyelocytes,myeloblasts anemia(occurs as a rule) leukocyte and platelet count may be normal or increased or depressed
76
development of leukemia in PV is related to
chemotherapy development of extramedullary hematopoeisis,hepatosplenomegaly,transfusion dependant anemia
77
Blood studies in ET
Anemia is uncommon Mild neutrophilic leukocytosis may be seen
78
Preferred bcr-abl assay in thrombocytosis patients with negative cytogenetic study for philadelphia chromosome
FISH bcr-abl reverse transcriptase polymerase chain reaction is associated with false-positive results
80
drugs used in PV
Interferon alpha(reduces splenomegaly) Pegylated IFN Hydroxyurea anagrelide
82
Difference btw JAK2 mutation positive and negative Myeloproliferative disorders
JAK2 mutation positive Disease course in decades transformation to acute leukemia is rare
83
hepatomegaly in PMF
Mild hepatomegaly may accompany the splenomegaly but is unusual in the absence of splenic enlargement
84
ET complication that requires reduction in platelet count
migrane
86
Negative predictive value of plasma erythropoeitin in erythrocytosis
low A normal erythropoietin level does not exclude a secondary cause for erythrocytosis or PV
87
Rx of pruritis in PV
anti histaminics anti depressants like doxepin hydroxy urea Interferon alpha PUVA
88
Comparison of erythropoietin and thrombopoietin
89
Is JAK V617F mutation diagnostic of PV?
NO Not every patient with PV expresses this mutation, while patients without PV do
90
Neurologic symptoms in PV
Due to Hyperviscosity headache vertigo tinnitus visual disturbance TIA
92
marrow fibrosis
94
Rx of polycythemia vera
phlebotomy
96
Drugs causing absolute erythrocytosis?
Androgens Recombinant erythropoietin
98
Diff btw ß thalassemia trait and other causes of microcytic erythrocytosis
normal RDW Increased RDW in other causes due to iron deficiency
99
Clinical features of PV
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
Cytogenetics in CEL
deletion or balanced translocation involving PDGFR alpha
101
Increase in marrow reticulin in ET
consider alternative diagnosis
102
Factors necessary for megakaryopoeisis
IL-3 SCF SDF-1(stromal cell derived factor)
103
Thrombosis of which vessels commonly occurs in PV
cerebral cardiac mesenteric
104
Causes of thrombocytosis
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
Glucocorticoids in PMF
improvement of anemia when used alone or with thalidomide control constitutional symptoms autoimmune complications
106
Splenectomy in PMF
increase risk blastic transformation
107
Lymphadenopathy in PMF
isolated lymphadenopathy should suggest another diagnosis

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