Haematology 6 - Haematology in systemic disease and intro to haematopathology Flashcards

1
Q

What disease is characterised by primary raised erythrocytes?

A

Polycythaemia vera

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2
Q

What disease is characterised by a secondary reduction in erythrocytes?

A

Auto-immune haemolytic anaemia

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3
Q

Which disease is caused by a genetic deficiency of factor IX?

A

Haemophilia B

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4
Q

Which disease is caused by a genetic excess of factor IX?

A

Factor IX Padua

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5
Q

Which disease is caused by a genetic deficiency of erythrocytes?

A

Sickle cell disease/ HbS (beta globin gene mutation)

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6
Q

How can haemophilia B be treated using gene therapy?

A

Factor IX Padua gene can be put into adenoviruses as a vector, to cause factor IX production

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7
Q

Which disease is caused by an acquired mutation in JAK2?

A

Polycythaemia vera

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8
Q

Which disease is caused by an acquired mutation in PIG A?

A

PNH paroxysmal nocturnal haemoglobinuria

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9
Q

What is a raised factor VIII likely to be secondary to?

A

An inflammatory process

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10
Q

How can haemophilia be acquired? (Rather than genetic)

A

Auto-immune disorder common in elderly - body produces autoantibodies directed against factor VIII

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11
Q

Recall 2 secondary causes of raised erythrocytes?

A

Altitude

EPO-secreting tumour

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12
Q

Recall 3 secondary causes of reduced erythrocytes

A

Bone marrow inflitration
Deficiency (B12/Fe)
Haemolytic anaemia

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13
Q

What is the most likely cause of iron deficiency anaemia?

A

Bleeding (until proven otherwise!)

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14
Q

How is iron deficiency anaemia diagnosed?

A

Ferritin and transferrin saturation: both would be low

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15
Q

Recall 3 types of cancer that may present first with an iron deficiency anaemia?

A

Gastric
Renal
Bladder

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16
Q

What are the morphological features of leuco-erythroblastic anaemia?

A

Erythroblasts (nucleated red blood cells and tear drop red blood cells)
Immature myeloid cells

17
Q

What are the causes of leucoerythroblastic anaemia?

A

Infection: miliary TB/severe fungal infection
Malignancy: myelofibrosis/ leukaemia/lymphoma/myeloma/ metastatic Ca
USUALLY A MALIGNANCY INVOLVING BM

18
Q

What are the laboratory features of haemolytic anaemia?

A

Anaemia (though may be compensated)

Reticulocytosis

Unconjugated hyperbilirubinaemia

LDH raised

Reduced haptoglobins

19
Q

Which test detects immune haemolytic anaemia?

A

DAT/Coombs

20
Q

Recall 2 malignancies that can cause immune haemolytic anaemia?

A

Lymphoma

CLL

21
Q

What type of anaemia is caused by mycoplasma infection?

A

Immune haemolytic anaemia

22
Q

What are the 2 main causes of non-immune/ Dat neg haemolytic anaemia?

A
  1. Malaria

2. MAHA - microangiopathic haemolytic anaemia

23
Q

Recall 2 causes of MAHA

A
  1. Underlying adenocarcinoma

2. Haemolytic uraemic syndrome

24
Q

How can CLL and AML be distinguished on blood film?

A

CLL has mature cells, AML has immature cells

25
Q

What is the main cause of neutrophilia?

A

Pyogenic infection

26
Q

How do you distinguish a reactive neutrophilia vs a malignant one?

A

Reactive neutrophilia has a limit (they won’t be sky high)

No immature cells in reactive neutrophilia

Malignant hasimmature cells + either basophils (indicative of CML) or myeloblasts (indicative of AML)

27
Q

What is the most common cause of a reactive lymphopaenia?

A

HIV

28
Q

In which 2 conditions might smear cells appear on the blood film?

A

CLL

Non-Hodgkins lymphoma

29
Q

Name 2 primary causes of haemolytic anaemia

A
Any of: 
Hereditary spherocytosis
G6PD deficiency
Sickle cell
Thalassaemia
30
Q

How can DIC cause MAHA?

A

Procoagulant factors released

In places where blood moves slowly (eg small vasculature) there is platelet deposition and fibrinogen is converted to fibrin

Red cells pushed through fibrin web and fragmented

31
Q

How is B cell clonality determined?

A

Look for light chain restriction - the ratio of kappa and lambda (60:40 would be reactive, 99:1 would be malignant)

32
Q

Recall 2 tests that can determine immunophenotype in blood malignancies, and one use of knowing this info?

A

Flow cytometry
Immunohistology
Use: working out if T or B lineage, working out if myeloid or lymphoid

33
Q

Recall 2 tests that can determine cytogenetics in blood malignancies and one use of knowing this info?

A

Translocations
FISH studies
Use: Philadelphia chromosome identification

34
Q

Recall 2 tests that can determine molecular genetics in blood malignancies and one use of knowing this info?

A

PCR
Pyro sequencing
Use: Detect JAK2 mutation, or BCR ABL cDNA