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Myeloproliferative disorders Flashcards

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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
Treatment of PV
Venesection Chemotherapy with hydroxyurea/gamma interferon Treatment of complications
26
Secondary polycythemia causes
Compensatory increase in EPO * high altitude * pulmonary diseases * heart diseases * Abnormal Hb * Heavy cigarette smoker Abnormal increases in EPO * Renal disease Tumors
27
Relative polycythemia causes
``` stress burns smoking dehydration alcohol ```
28
What is myelofibrosis
Occur due to progressive fibrosis of the marrow with increased connective tissue
29
What genetic malformations cause myelofibrosis
JAK 2 55% CALR 25% MPL 10% JCM
30
Characteristics of myelofibrosis
myeloid metaplasia leading to extramedullary erythropoiesis in spleen /liver(outside bone marrow) abnormal megakaryocytes when trephine biopsy is done
31
Chronic idiopathic myelofibrosis characteristics
``` insidious onset splenomegaly bleeding issues bone pain gout ```
32
Presentation of myelofibrosis
``` Anemia high WBC initially, leukopenia later tear drop red cells no bone marrow aspiration; dry tap due to fibrosis increased NAP ```
33
Causes of leucoerythroblastic blood film(combination of normoblasts and nucleated rbcs)
``` Severe sepsis sick neonate severe hemolysis idiopathic myelofibrosis bone marrow infiltration ```
34
Treatment of myelofibrosis
Treatment aimed at reducing effects of anemia and splenomegaly Blood transfusion Folic acid Ruxolitinib a JAK2 inhibitor Hydroxycarbamide
35
Essential thrombocythemia is
Clonal myeloproliferative disease of megakaryocytic lineage
36
Essential thrombocytosis is characterized by
Sustained thrombocytosis for more than 6 months Increased megakaryocytes Thrombotic and hemorrhagic episodes can induce high platelet count
37
Mutation in essential thrombocytosis
JAK2 CALR MPL
38
Diagnosis criteria for essential thrombocytosis
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
Causes of reactive thrombocytosis
``` Bleeding trauma post operation chronic iron deficiency malignancy chronic infection connective tissue disorders post splenectomy ```
40
Clinical features of essential thrombocythemia
Haemorrhage microvascular occlusion splenic /hepatic vein thrombosis hepatosplenomegaly
41
TReatment for essential thrombocythemia
``` Anticoagulant Chemotherapy Hydroxycarbamide JAK 2 inhibitor Aspirin ```
42
Transformations that can occur from essential thrombocythemia
Acute myeloid leukemia 25% develop myelofibrosis Death due to cardiovascular complication
43
myeloid leukemia is
neoplasm of white blood cells and its precursor leading to clonal proliferation and accumulation of cells in the marrow. Acute/chronic
44
What is chronic myeloid leukemia
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
Median age of CML
53yrs
46
Age group affected
All age groups
47
Cause of CML.
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
This gene is present in 90-95% of CML patients
Philadelphia gene., an acquired cytogenic anomaly
49
Clinical features of CML
- About 40% asymptomatic - Splenomegaly - hypermetabolism - Anemia - Abnormal platelet function - hyperleukocytosis- priapism, visual and hearing -impairment, gout
50
Phases of CML
Chronic phase Acelerated phage Transformation to acute leukemia
51
Perpheral blood findings in CML
``` Raised WBC count primitive blast cells granulocytes at all stages of development raised platelet count low Hb concs high basophils and eosinophils ```
52
Lab features
``` 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
Investigations for CML
``` full blood count neutrophil alkaline phosphatase urea, electrolytes serum lactate dehydrogenase bone marrow aspirate/trephine biopsy Vitamin B12 and B12 binding capacity test ```
54
Chronic phase of CML
Blast cell percentage is less than 10% Basophil percentage less than 20%
55
Accelerated phase of CML
Blast cells are between 10-19% in blood Basophil count more than 20% Disease becoming more aggressive
56
Blastic or acute myeloid leukemia phase
Blasts are more than 20% 2/3 transform to myeloid blastic phase and 1/3 to lymphoid blastic phase
57
Treatment of CML
- 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
Imatinib/Glivec MOA
It acts to inhibit the enhanced tyrosine kinase activity of BCR-ABL oncoprotein thus reversing the pathology
59
Drugs for chemotherapy
- Busulphan - Hydroxyurea- inhibits ribonucleotide reductase; disease progression isn't altered because of persistence of Philadelphia chromosome
60
If philadelphia chromosome is seen to be present ideal drug to be used to prevent progression from chronic phase is
Imatinib. tyrosine kinase inhibitor

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