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Clinical Pathology > CP7-10 chronic myeloproliferative disorders > Flashcards

CP7-10 chronic myeloproliferative disorders Flashcards

1
Q

What is chronic myeloid leukaemia?

A

A long-lasting cancer arising from white blood cells deriving from myeloid cells e.g. basophils, neutrophils and eosinophils

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2
Q

What is the epidemiology of chronic myeloid leukaemia?

A

Less than 1% of cancer cases
Peaking in 85-89 year olds
Between 800 cases per year in the UK
Rates are decreasing

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3
Q

What chromosome is associated with chronic myeloid lymphoma?

A

Philadelphia chromosome which results due to translocation of ABL1 gene from chromosome 9 to adjacent to the BCR gene of 22 forming the BCR-ABL gene

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4
Q

What does BCR-ABL gene do?

A

induces mitogenesis (i.e. increases rate of division) of fibroblast and hematopoietic cells and protects cells from apoptosis

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5
Q

What drugs are used to treat chronic myeloid leukaemia?

A

Imatinib (and other -tinib drugs)

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6
Q

What are significant results in a FBC that suggest a patient has CML?

A

With WCC ranging from just above normal to >500
Anaemia is common
Platelets are raised

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7
Q

What WBC are increased in the blood in CML?

A

Neutrophils
Myelocytes
Eosinophils
Basophils
Small blast cells - slightly but not as much as in acute myeloid leukaemia

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8
Q

How may a patient with CML present?

A

Potentially asymptomatic
Headache
Blurred vision
Night sweats
Fatigue
Bone pain (less common)
Abdominal pain or swelling due to enlarging spleen (spleen become palpable)
SoB
Bruising or bleeding
Weight loss
Priapism (persistent and painful erection)
Hyperviscosity syndrome

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9
Q

What is polycythaemia?

A

Increased Hb and haematocrit in the blood.

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10
Q

What is the BCSH guidelines for diagnosing polycythaemia?

A

> 0.48 haematocrit count in females and >0.52 in males sustained for more than 2 months

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11
Q

What categories are polycythaemia divided into?

A

Congenital
Acquired

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12
Q

What are some secondary causes of polycythaemia?

A

Appropriate reaction to low oxygen:
- Lung or cyanotic heart disease
- High altitude
- Sleep apnoea
- Renal hypoxia due to artery stenosis or cysts
Lifestyle:
- smoking
- alcohol
- sports performance enhancing drugs
Testosterone treatment

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13
Q

What are some congenital causes of polycythaemia?

A

High affinity haemoglobin
EPO receptor mutation
Defect in oxygen sensing pathway

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14
Q

What are some causes of acquired polycythaemia?

A

Primary polycythaemia
Secondary polycythaemia
Idiopathic erythrocytosis

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15
Q

What are come cancers that can cause secondary polycythaemia due to ectopic erythropoietin secretion?

A

Renal tumours
Uterine Tumours
Hepatocellular carcinoma
Cerebella haemangioma
Parathyroid tumours

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16
Q

What mineral deficiency can disguise polycythaemia?

A

Iron deficiency

17
Q

What blood findings are found in polycythaemia?

A

Sustained raised haematocrit
Low MCV
50% will have raised white cell count or raised platelets
Basophilia and or eosinophilia

18
Q

How are patients with potential polycythaemia assessed?

A

Through history - social and family specifically
Examination - can the spleen be palpated and oxygen sats
Chest X-ray
Blood tests
Potential further tests:
- US abdomen
- Head and neck CT
- Gene sequencing

19
Q

What genetic mutation is associated with polycythaemia?

A

Jak 2 mutation

20
Q

How can Jak 2 mutation cause primary polycythaemia?

A

gives myeloid progenitor cells a survival advantage and causes clonal overproliferation

21
Q

How do patients with polycythaemia present?

A

Headache
Blurred vision
Fatigue
Itching - aquagenic pruritus
+/- splenomegaly
Thrombosis
Gout
Not as common but erythromelalgia

22
Q

How is polycythaemia treated?

A

Low dose aspirin
Venesection
Low dose chemotherapy e.g. with hydroxycarbamide

23
Q

What are potential complications of polycythaemia?

A

Transformation into acute leukaemia in 2% after 10 years
Increase risk of transformation into myelofibrosis

24
Q

What is essential thrombocythaemia?

A

A myeloproliferative neoplasm where there is a raised platelet count +/- raised WCC

25
Q

What causes reactive thrombocytosis?

A

Bleeding
Iron deficiency
Post-operative
Infection
Inflammation
Malignant disease

26
Q

How do patients with essential thrombocytopenia present?

A

Often asymptomatic and picked up on routine testing
Pruritus
Erythromelalgia
Thrombosis in 20-30% of patients

27
Q

What disease causes a high platelet count associated with paradoxical higher bleeding risk?

A

Acquired vWD

28
Q

When gene mutations are associated with essential thrombocytopenia?

A

JAK2
CALR
MPL

29
Q

When is a bone marrow biopsy taken?

A

If unexplained cause of thrombocytopenia/ nothing picked up genetically

30
Q

How is bone marrow morphology characterised in patients with essential thrombocytopenia?

A

Larger megakaryocytes forming loose clusters

31
Q

What is myelofibrosis?

A

Fibrosis due to an interaction between coronal megakaryocytes producing microbes such as PDGF increasing fibroblast proliferation

32
Q

What is the risk of myelofibrosis transforming into acute myeloid leukaemia?

A

10-20% after 10 years

33
Q

What are the pathology in patients with myelofibrosis?

A

In leucoerythroblastic blood film:
- Immature white and red cells
- initial increase in platelets and normal Hb
- develops into bone marrow failure with pancytopenia
Bone marrow biopsy:
- initially hypercellular +/- pre-fibrotic phase
- unlit at Ely hypocellular eith increase fibrosis on reticulin staining

34
Q

What is extramedullary haemopoiesis?

A

Production of blood cells in organs other than the bone marrow e.g. spleen or liver

35
Q

What commonly causes extramedullary haemopoiesis?

A

Myelofibrosis

36
Q

How do patients with myelofibrosis present?

A

Generally unwell
Anaemia
Bruising/bleeding
Increased infections
Fever, weight loss, drenching night sweats, bone pain
early hunger
Pain due to splenomegaly
Capsular distension in spleen or splenic infarct

37
Q

How is myelofibrosis treated?

A

Erythropoietin or transfusion for anaemia
Ruxolitinab
Splenectomy
Stem cell transplant in younger and high risk patients

38
Q

How does the drug ruxolitinib work?

A

Inhibits Jak2

Clinical Pathology (65 decks)

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