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Haematology > Myeloproliferative disorders > Flashcards

Myeloproliferative disorders Flashcards

1
Q

MYELOPROLIFERATIVE DISORDERS

A

clonal proliferation of bone marrow stem cells which can manifest as increased number of cells

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2
Q

Myeloproliferative disorders arise from

A

Hematopoietic stem cell

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3
Q

Characteristics of myeloproliferative disorders

A

Clonal - group of cells derived from a common progenitor which in this case is abnormal

Characterized by proliferation of

megakaryocytes
erythroid
granulocyte which will have the same abnormality

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4
Q

Classification of myeloproliferative disorders

A

Lineage of predominant proliferation
level of marrow fibrosis
clinical and laboratory data

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5
Q

How does clonal evolution occur

A
  1. The acquired abnormality is with the stem cell

2. The abnormal myeloid lineage proliferates into abnormal megakaryocytes, granulocytes, erythroid/red cell precursors

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6
Q

If granulocyte precursors are dominant it forms

A

Chronic myeloid leukemia

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7
Q

If red cell precursors are dominant it forms

A

polycythemia vera

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8
Q

If megakaryocytes are dominant in proliferation it forms

A

Essential thrombocytoss

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9
Q

70% of chronic myeloid leukemia transforms into

A

acute myeloid leukemia

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10
Q

10% of polycythemia vera patients can transform into

A

acute myeloid leukemia

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11
Q

30% of PV cases transform into

A

myelofibrosis

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12
Q

10% of myelofibrosis transforms into

A

Acute myeloid leukemia

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13
Q

Myeloproliferative disorders are found in

A

Adults usually in their 70s

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14
Q

Pathogenesis of myeloproliferative disorders

A

A genetic abnormality that inhibits the body’s ability to switch of the excess production of cells- thus a dysregulated proliferation leading to high amounts of a particular cell line in the system.

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15
Q

Most MPD results in

A

Bone marrow fibrosis

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16
Q

Polycythemia vera

A

Clonal stem cell disorder characterized by increased red cell production. Characterized by a raised packed cell volume or a raised Hb level

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17
Q

Classification of Polycythemia vera

A

NB not not all PV results from MPD

  1. Absolute polycythemia ; increase in RBC mass due to
    * Primary causes ; PV
    * secondary causes ; hypoxia, increased EPO production
    * Idiopathic
  2. Apparent polycythemia; Plasma volume changes due to i.e dehydration makes red blood cell count increase
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18
Q

Mutation responsible for polycythemia vera disorder

A

JAK2V617F mutation

Exon 12

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19
Q

Disease phases of polycythemia vera

A

3 phases.

Proliferative phase
spent phase
rarely transformed into acute leukemia

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20
Q

In proliferative phase of PV

A

Red cell and Hb count very high

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21
Q

Spent phase of PV

A

Here bone marrow fibrosis occurs and less space is available for RBC production. There is a start in decline of numbers, though they are still high

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22
Q

Clinical features

A 
Age - 55-60 yrs may occur in young adults and rarely children
Thrombosis
DVT
Hypertension
headache 
poor vision
skin complications i.e pruritis
haemorrhhage due to platelet defect
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23
Q

Presentation of PV

A

Hepatosplenomegaly

Erythromelalgia ( red skin, burning sensation)

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24
Q

Lab findings in PV

A

high Hb and RBC

Associated increase in platelets, neutrophils

Normal neuclotide alkaline phosphatase

high plasma urate- high cell turnover

hypercellular bone marrow

Low serum erythropoietin

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25
Q

Treatment of PV

A

Venesection
Chemotherapy with hydroxyurea/gamma interferon
Treatment of complications

26
Q

Secondary polycythemia causes

A

Compensatory increase in EPO

  • high altitude
  • pulmonary diseases
  • heart diseases
  • Abnormal Hb
  • Heavy cigarette smoker

Abnormal increases in EPO
* Renal disease
Tumors

27
Q

Relative polycythemia causes

A 
stress
burns
smoking
dehydration
alcohol
28
Q

What is myelofibrosis

A

Occur due to progressive fibrosis of the marrow with increased connective tissue

29
Q

What genetic malformations cause myelofibrosis

A

JAK 2 55%
CALR 25%
MPL 10%

JCM

30
Q

Characteristics of myelofibrosis

A

myeloid metaplasia leading to extramedullary erythropoiesis in spleen /liver(outside bone marrow)

abnormal megakaryocytes when trephine biopsy is done

31
Q

Chronic idiopathic myelofibrosis characteristics

A 
insidious  onset
splenomegaly
bleeding issues
bone pain
gout
32
Q

Presentation of myelofibrosis

A 
Anemia
high WBC initially, leukopenia later
tear drop red cells
no bone marrow aspiration; dry tap due to fibrosis
increased NAP
33
Q

Causes of leucoerythroblastic blood film(combination of normoblasts and nucleated rbcs)

A 
Severe sepsis
sick neonate
severe hemolysis
idiopathic myelofibrosis
bone marrow infiltration
34
Q

Treatment of myelofibrosis

A

Treatment aimed at reducing effects of anemia and splenomegaly

Blood transfusion
Folic acid
Ruxolitinib a JAK2 inhibitor
Hydroxycarbamide

35
Q

Essential thrombocythemia is

A

Clonal myeloproliferative disease of megakaryocytic lineage

36
Q

Essential thrombocytosis is characterized by

A

Sustained thrombocytosis for more than 6 months
Increased megakaryocytes
Thrombotic and hemorrhagic episodes can induce high platelet count

37
Q

Mutation in essential thrombocytosis

A

JAK2
CALR
MPL

38
Q

Diagnosis criteria for essential thrombocytosis

A
  1. sustained platelet count above 450*10 raised to power 9
  2. Prescence of JAK2 or CALR
  3. No other myeloid malignancy
  4. No reactive cause of thrombocytosis
  5. Bone marrow trephine biopsy showing increased megakaryocytes
  6. Normal iron stores
39
Q

Causes of reactive thrombocytosis

A 
Bleeding
trauma
post operation
chronic iron deficiency
malignancy
chronic infection
connective tissue disorders
post splenectomy
40
Q

Clinical features of essential thrombocythemia

A

Haemorrhage
microvascular occlusion
splenic /hepatic vein thrombosis
hepatosplenomegaly

41
Q

TReatment for essential thrombocythemia

A 
Anticoagulant
Chemotherapy
Hydroxycarbamide
JAK 2 inhibitor
Aspirin
42
Q

Transformations that can occur from essential thrombocythemia

A

Acute myeloid leukemia
25% develop myelofibrosis
Death due to cardiovascular complication

43
Q

myeloid leukemia is

A

neoplasm of white blood cells and its precursor leading to clonal proliferation and accumulation of cells in the marrow.

Acute/chronic

44
Q

What is chronic myeloid leukemia

A

Characterized by increased proliferation of granulocytic cell line without the loss of their capacity to differentiate

You’ll see granulocyte at all stages of development

There is increased myeloid, erythroid,platelet cells in peripheral blood

45
Q

Median age of CML

A

53yrs

46
Q

Age group affected

A

All age groups

47
Q

Cause of CML.

A

Fusion of BCR gene on chromosome 22 fusing with ABL gene on chromosome 9

This forms a BCR-ABL protein that transforms hematopoietic cells so their growth is independent of cytokines–> No apoptosis

48
Q

This gene is present in 90-95% of CML patients

A

Philadelphia gene., an acquired cytogenic anomaly

49
Q

Clinical features of CML

A
  • About 40% asymptomatic
  • Splenomegaly
  • hypermetabolism
  • Anemia
  • Abnormal platelet function
  • hyperleukocytosis- priapism, visual and hearing -impairment, gout
50
Q

Phases of CML

A

Chronic phase
Acelerated phage
Transformation to acute leukemia

51
Q

Perpheral blood findings in CML

A 
Raised WBC count
primitive blast cells
granulocytes at all stages of development
raised platelet count
low Hb concs
high basophils and eosinophils
52
Q

Lab features

A 
hypercellular bone marrow
Myeloid- erythroid ratio is 10;1 as compared to 30;1
Predominant cells are myelocytes
Myeloblast are less than 10 percent
increased dysplastic megakaryocytes
Reticulin fibrosis
53
Q

Investigations for CML

A 
full blood count
neutrophil alkaline phosphatase
urea, electrolytes
serum lactate dehydrogenase
bone marrow aspirate/trephine biopsy
Vitamin B12 and B12 binding capacity test
54
Q

Chronic phase of CML

A

Blast cell percentage is less than 10%

Basophil percentage less than 20%

55
Q

Accelerated phase of CML

A

Blast cells are between 10-19% in blood

Basophil count more than 20%

Disease becoming more aggressive

56
Q

Blastic or acute myeloid leukemia phase

A

Blasts are more than 20%

2/3 transform to myeloid blastic phase and 1/3 to lymphoid blastic phase

57
Q

Treatment of CML

A
  • Relieve symptoms of hyperleukocytosis, splenomegaly, thrombocytosis
  • Control and prolong chronic phase using alpha interferon, hydroxyurea, chemotherapy, imatinib mesylate

-Eradicate malignant clone - curative
via transplantation or imatinin/glivec ; a tyrosine kinase inhibitor

58
Q

Imatinib/Glivec MOA

A

It acts to inhibit the enhanced tyrosine kinase activity of BCR-ABL oncoprotein thus reversing the pathology

59
Q

Drugs for chemotherapy

A
  • Busulphan
  • Hydroxyurea- inhibits ribonucleotide reductase; disease progression isn’t altered because of persistence of Philadelphia chromosome
60
Q

If philadelphia chromosome is seen to be present ideal drug to be used to prevent progression from chronic phase is

A

Imatinib. tyrosine kinase inhibitor

Haematology (29 decks)

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