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Myeloproliferative Disorders Flashcards

(70 cards)

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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
What is relative polycythemia
When there is an apparent polycythemia or pseudo polycythemia which is due to plasma volume contraction
26
What are some causes of relative polycythemia
Stress cigarette smoking alcohol intake dehydration plasma loss like in burn injury
27
What is myelofibrosis or chronic idiopathic myelofibrosis
It is the progressive fibrosis of the marrow and increase in connective tissue element
28
Types of gene mutation in myelofibrosis
JAK2 55% CALR 25% MPL 10%
29
Is myeloid metaplasia Common in myelofibrosis
Yes
30
What is myeloid metaplasia and where does erythropoietin occur
Bone marrow replaced with fibrous tissue It’s extramedullary erythropoiesis mostly in the spleen and liver
31
What type of patient is Myelofibrosis mostly occur
Older patients
32
Clinical manifestation of myelofibrosis
``` Massive splenomegaly Hypermetabolic symptoms like loss of weight, fever ,and night sweats Bleeding problems bone pain gout ```
33
Can myelofibrosis become acute leukemia
Yes in 10 to 20% of
34
Lab findings of myelofibrosis
``` 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
What is dry tap
In bone marrow aspiration not being able to get marrow
36
What are the causes of a leucoeryhtroblastic blood film
``` Idiopathic myelofibrosis bone marrow infiltration severe sepsis severe hemolysis sick neonates ```
37
What is the treatment of myelofibrosis
Blood transfusions ,regular folic acid therapy for anemia Rudolitinib as oral JAK2 inhibitor to reduce spleen size hydroxycarbamide
38
What is essential thrombocythemia
Clonal Myeloproliferative disease of megakaryocytic lineage. With thrombocytosis for more than six months ,increased megakaryocytes ,thrombotic or hemorrhagic episodes
39
What are some ways to have positive criteria for essential thrombocythemia
Platelet count of more than 450×10 ^9 per liter Bone marrow biopsy large and increased megakaryocytes
40
Mutation in essential thrombocythemia
``` JAK2 mutation (50-60%) CALR 75% if neg to jak 2 ```
41
Diagnostic criteria for essential thrombocythemia
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
Which criteria’s are required for diagnosis of essential thrombocythemia
A1-A3 Or A1+ A3-A5
43
What are some causes of reactive thrombocytosis
``` Bleeding Trauma Post operation chronic iron deficiency malignancy chronic infection connective tissue disorder’s post splenectomy ```
44
Clinical features of essential thrombocythemia
Hemorrhage microvascular occlusion splenic or hepatic vein thrombosis hepatosplenomegaly
45
Treatments of essential thrombocythemia
Anticoagulants chemotherapy with hydroxycarbamide, JAK2 inhibitors Aspirin
46
Percentage of population that develop myelofibrosis in essential thrombocythemia
25%
47
Are acute leukemia transformation and deaths due to cardiovascular complication common disease course in essential thrombocythemia
Yes
48
What is leukemias
Neoplasm of white blood cells and it’s precursor with clonal proliferation and accumulation of cells in marrow
49
Two types of leukemias
Acute leukemias | chronic leukemia’s
50
What is chronic myeloid leukemia
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
Etiology of chronic myeloid leukemia
Not clear but BCR ABL fusion GENE
52
People at risk of chronic myeloid leukemia
Survivors of an atomic disasters of neck Nagasaki and Hiroshima Post radiation therapy
53
What is the acquired cytogenetic anomaly occurring in all leukemic cells in chronic myeloid leukemia
Philadelphia chromosome
54
What’s translocation occurs in the Philadelphia chromosome
Translocation reciprocal between Chromosomes 22 and chromosome nine
55
Why is the fusion of the Protein BCR abl leading to leukemia
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
Can you call features of chronic myeloid leukemia
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
Blood findings in chronic myeloid leukemia
``` Raised white blood cell counts Blast cell less than 10% Granulocytes at all stage High eosinophils, basophils Hb low Raised platelet ```
58
Bone marrow lab features
``` 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
Test for chronic myeloid leukemia
``` 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
What characterize the chronic phase of myeloid leukemia
Less than 10% blast cell in the blood or the marrow | basophils less than 20% in the blood
61
What happens in the accelerated phase which last for 3.5 to 5 years
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
What happens in the blastic phase of myeloid leukemia
Ressemble acute leukemia More than 20% blessed in blood or bone marrow 2/3 myeloid blastic phase 1/3 in lymphoid blastic phase
63
Which has higher survival rates lymphoid or myeloid leukemia
Name for it
64
General management of chronic myeloid leukemia
Discussion with family about prognosis ,disease ,diagnosis ,choice of treatment (cytotoxic drug or bone marrow transplant)
65
What is the principle of the treatment in chronic myeloid leukemia
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
Action of imatinib mesilate
inhibits enhanced protein tyrosine kinase activity of BCR ABL oncoprotein
67
What is busulphan
Alkylating agent used in old patient which are not candidate for transplant
68
Side effect of busulphan
Prolonged myelosuppression skin pigmentation infertility pulmonary fibrosis
69
Hydroxyurea action
Inhibits ribonucleotide reductase
70
Which treatment has the longest survival rate for chronic myeloid leukemia
Transplant with more than five years survival | Interferon and chemotherapy for six years survival

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