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Hematology > Myeloproliferative Disorders > Flashcards

Myeloproliferative Disorders Flashcards

1
Q

Part of the body affected in myekoproliferaive disorders

A

Hematopoietic stem cell

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2
Q

Types of cells that proliferate in myeloproliferative disorders

A

Granulocytes
Ethrytocytes
Megakaryocytes

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3
Q

Classification based on

A

Lineage of predominant proliferation
Level of marrow fibrosis
Clinical data
Lab data

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4
Q

Clinal evolution in myeloproliferative disorder

A

Acquired abnormality of bone marrow stem cells

-> granulocytes precusors, red cell precursors , megakaryocytes

granulocytes precusors -> chronic myeloid leukemia -> acute myeloid leukemia

Red cell precursors -> polycythemia Vera ->acute myeloid leukemia or myeloid fibrosis

Megakaryocytes -> essential thrombocythemia or polycythemia Vera - > myelofibrosis

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5
Q

Is myeloproliferative disorder common in children or adults ?

A

Adults

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6
Q

Peak incidence of myeloproliferative disorder

A

7th

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7
Q

Pathogenesis

A

Dysregulated proliferation

Genetic abnormality

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8
Q

Is there bone marrow fibrosis in all MPD

A

Yes

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9
Q

What is polycythemia

A

Raised packed cell volume HCT

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10
Q

Polycythemia level in male

A

More than 49%

Hb more than 16.5 g/dl

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11
Q

Polycythemia level in female

A

More than 48%

Hb more than 16g/dl

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12
Q

Classification of MPD

A

Absolute

Apparent

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13
Q

Types of absolute MPD

A

Primary proliferative polycythemia (polycythemia Vera)
Secondary polycythemia
Idiopathic erythrocytosis

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14
Q

Type of apparent MPD

A

Plasma volume changes

Red cell mass changes

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15
Q

What is polycythemia Vera

A

Clonal stem cell disorder with increased red cell production

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16
Q

Mutation linked to polycythemia Vera

A

JAk2V617F

Exon 12(only 3%)

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17
Q

Disease phase of polycythemia Vera

A

Proliferative phase
Spent post polycythemia phase
Rarely become acute leukemia

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18
Q

Main age of polycythemia vera

A

55-60yo

Sometimes young adults

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19
Q

Clinical prevention of polycythemia Vera

A 
Thrombosis 
DVT
Hypertension 
Headache 
Poor vision
Dizziness
Skin complications like pruritus , erythomelalgia
Hemorrhage due to platelet defect 
Hepatosplenomegaly 
Increased skin temp 
Burning sensation 
Redness
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20
Q

Lab findings of polycythemia Vera

A 
Hb
PCV
Red cell mass increased
Increased neutrophils and platelets 
Normal NAP
Plasma urate high
Hyper cellular bone marrow 
Low serum erythropoietin
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21
Q

Treatment of polycythemia vera

A

Decrease hematocrit by venesection , chemotherapy (hydroxyurea)
treat complication

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22
Q

What is secondary polycythemia

A

Increase in red cell mass due to other known -conditions in the body

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23
Q

When is there a compensatory y increase in erythropoietin

A

When there is hypoxia like in
In high altitude
pulmonary disease
Heart condition like Cyanotic heart disease
abnormal hemoglobin like high affinity hemoglobin
heavy cigarette smoker

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24
Q

When can you say that there is inappropriate erythropoietin production

A

In renal disease like carcinoma or hydronephrosis

in tumors lake fibromyoma and liver carcinoma

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25
Q

What is relative polycythemia

A

When there is an apparent polycythemia or pseudo polycythemia which is due to plasma volume contraction

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26
Q

What are some causes of relative polycythemia

A

Stress
cigarette smoking
alcohol intake dehydration
plasma loss like in burn injury

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27
Q

What is myelofibrosis or chronic idiopathic myelofibrosis

A

It is the progressive fibrosis of the marrow and increase in connective tissue element

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28
Q

Types of gene mutation in myelofibrosis

A

JAK2 55%
CALR 25%
MPL 10%

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29
Q

Is myeloid metaplasia Common in myelofibrosis

A

Yes

30
Q

What is myeloid metaplasia and where does erythropoietin occur

A

Bone marrow replaced with fibrous tissue

It’s extramedullary erythropoiesis
mostly in the spleen and liver

31
Q

What type of patient is Myelofibrosis mostly occur

A

Older patients

32
Q

Clinical manifestation of myelofibrosis

A 
Massive splenomegaly
Hypermetabolic symptoms like loss of weight, fever ,and night sweats
Bleeding problems 
bone pain 
gout
33
Q

Can myelofibrosis become acute leukemia

A

Yes in 10 to 20% of

34
Q

Lab findings of myelofibrosis

A 
Anemia 
high white blood cells 
Late leukopenia and thrombocytopenia 
leucoerythroblastic blood filmed 
teardrop red cells 
bone marrow aspiration with DRY TAP due to fibrosis 
TREPHINEbiopsy with fibrotic hyper cellular marrow 
increase in NAP score
35
Q

What is dry tap

A

In bone marrow aspiration not being able to get marrow

36
Q

What are the causes of a leucoeryhtroblastic blood film

A 
Idiopathic myelofibrosis 
bone marrow infiltration 
severe sepsis 
severe hemolysis 
sick neonates
37
Q

What is the treatment of myelofibrosis

A

Blood transfusions ,regular folic acid therapy for anemia

Rudolitinib as oral JAK2 inhibitor to reduce spleen size

hydroxycarbamide

38
Q

What is essential thrombocythemia

A

Clonal Myeloproliferative disease of megakaryocytic lineage. With thrombocytosis for more than six months ,increased megakaryocytes ,thrombotic or hemorrhagic episodes

39
Q

What are some ways to have positive criteria for essential thrombocythemia

A

Platelet count of more than 450×10 ^9 per liter
Bone marrow biopsy
large and increased megakaryocytes

40
Q

Mutation in essential thrombocythemia

A 
JAK2 mutation (50-60%)
CALR 75% if neg to jak 2
41
Q

Diagnostic criteria for essential thrombocythemia

A

A1 sustain platelet count above 450×109 per liter
A2 acquired pathogenetic mutation
A3. No other myeloid malignancy primary myelofibrosis Clinic my Lloyd leukemia myelodysplastic syndrome

A4. No reactive cause for thrombocytosis and normal iron stores
A5 Bone marrow trephine histology shows increaesed megakaryocytes with large hyper lobulated forms , reticulin not generally increased

42
Q

Which criteria’s are required for diagnosis of essential thrombocythemia

A

A1-A3
Or
A1+ A3-A5

43
Q

What are some causes of reactive thrombocytosis

A 
Bleeding
 Trauma
 Post operation 
chronic iron deficiency
 malignancy 
chronic infection
 connective tissue disorder’s 
post splenectomy
44
Q

Clinical features of essential thrombocythemia

A

Hemorrhage
microvascular occlusion
splenic or hepatic vein thrombosis
hepatosplenomegaly

45
Q

Treatments of essential thrombocythemia

A

Anticoagulants
chemotherapy with hydroxycarbamide, JAK2 inhibitors
Aspirin

46
Q

Percentage of population that develop myelofibrosis in essential thrombocythemia

A

25%

47
Q

Are acute leukemia transformation and deaths due to cardiovascular complication common disease course in essential thrombocythemia

A

Yes

48
Q

What is leukemias

A

Neoplasm of white blood cells and it’s precursor with clonal proliferation and accumulation of cells in marrow

49
Q

Two types of leukemias

A

Acute leukemias

chronic leukemia’s

50
Q

What is chronic myeloid leukemia

A

Clonal malignant myeloproliferative disorder with increased proliferation of granulocytic cell line without loss of the capacity to differentiates

Increase in myeloid cells ,erythroidu cells and platelets in peripheral blood

marked myeloid hyperplasia in the bone marrow

51
Q

Etiology of chronic myeloid leukemia

A

Not clear but BCR ABL fusion GENE

52
Q

People at risk of chronic myeloid leukemia

A

Survivors of an atomic disasters of neck Nagasaki and Hiroshima
Post radiation therapy

53
Q

What is the acquired cytogenetic anomaly occurring in all leukemic cells in chronic myeloid leukemia

A

Philadelphia chromosome

54
Q

What’s translocation occurs in the Philadelphia chromosome

A

Translocation reciprocal between Chromosomes 22 and chromosome nine

55
Q

Why is the fusion of the Protein BCR abl leading to leukemia

A

Has tyrosine kinase activity which transforms Hematopoietic cells To become independent of growth and survival cytokines And protect the cells from programmed cell death

56
Q

Can you call features of chronic myeloid leukemia

A

40% asymptomatic
Chronic phase with massive splenomegaly ! hypermetabolism symptoms like weight loss ,anorexia ,lassitude ,night sweats

Anemia with pallor, dyspnoea ,tachycardia

Abnormal platelet function with bruising ,epistaxis ,menorrhagia

Hyper leukocytosis With thrombosis ,increased purine breakdown with gout, visual disturbances, priapism

57
Q

Blood findings in chronic myeloid leukemia

A 
Raised white blood cell counts
Blast cell less than 10% 
Granulocytes at all stage 
High eosinophils, basophils
Hb low 
Raised platelet
58
Q

Bone marrow lab features

A 
Hypocellular 
reduced fat spaces 
myeload to erythroid ratio 10:1 to 30:1
Myélocytes predominant 
Blast cells less than 10% 
megakaryocytes increased and dysplastic
 increased ridiculous fibrosis in 30 to 40%
59
Q

Test for chronic myeloid leukemia

A 
Full blood counts 
blood film 
neutrophils alkaline phosphatase
 urea 
electrolytes
 serum lactate dehydrogenase 
bone marrow aspirate 
bone marrow trephine biopsy (extent of fibrosis 
BCR ABL chimeric gene  by fluorescent in situ
 hybridization or PCR 
vitamin B 12 and B12 binding capacity
60
Q

What characterize the chronic phase of myeloid leukemia

A

Less than 10% blast cell in the blood or the marrow

basophils less than 20% in the blood

61
Q

What happens in the accelerated phase which last for 3.5 to 5 years

A

Increased Splenomegaly because of chemotherapy
increased chemotherapy requirement
Blast cells about 10 to 19% in the blood
basophils more than 20% in the blood
persistent thrombocytopenia or thrombocytosis
cytogenetic Clonal evolution

62
Q

What happens in the blastic phase of myeloid leukemia

A

Ressemble acute leukemia
More than 20% blessed in blood or bone marrow
2/3 myeloid blastic phase 1/3 in lymphoid blastic phase

63
Q

Which has higher survival rates lymphoid or myeloid leukemia

A

Name for it

64
Q

General management of chronic myeloid leukemia

A

Discussion with family about prognosis ,disease ,diagnosis ,choice of treatment (cytotoxic drug or bone marrow transplant)

65
Q

What is the principle of the treatment in chronic myeloid leukemia

A

Relieve symptoms of hyper leukocytosis splenomegaly and thrombocytosis through hydration and chemotherapy

Control and prolong the chronic phase by , imatinib mesylate , hydroxyurea ,Alfa interferon+ chemotherapy

Eradicate malignant clone to cure with allogeneic transplant or imatinib

66
Q

Action of imatinib mesilate

A

inhibits enhanced protein tyrosine kinase activity of BCR ABL oncoprotein

67
Q

What is busulphan

A

Alkylating agent used in old patient which are not candidate for transplant

68
Q

Side effect of busulphan

A

Prolonged myelosuppression
skin pigmentation
infertility
pulmonary fibrosis

69
Q

Hydroxyurea action

A

Inhibits ribonucleotide reductase

70
Q

Which treatment has the longest survival rate for chronic myeloid leukemia

A

Transplant with more than five years survival

Interferon and chemotherapy for six years survival

Hematology (24 decks)

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