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Pathology Summaries > HAEM: Haemolytic anaemias and systemic disease > Flashcards

HAEM: Haemolytic anaemias and systemic disease Flashcards

1
Q

What does the VHL gene mutation cause?

A

Chuvash polycythaemia - excess erythrocytes

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2
Q

How do corticosteroids affect leukocytes?

A

Raised`

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3
Q

4 types of anaemia?

A

Iron deficiency
AI haemolytic
Microangiopathic
Leukoerythroblastic

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4
Q

2 cancers which cause occult blood loss.

A

GI

Urinary

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5
Q

What does leukoerythroblastic mean? Give an example of their morphology.

A

RBC and WBC of variable degree of maturation

e.g. Tear drop aniso/poikilocytes (this is usually a sign of myelofibrosis), myelocytes, nucleated RBCs

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6
Q

3 causes of leukoerythroblastic blood film?

A

Malignancy - haem or non-haem
Severe infection
Myelofibrosis (tear drop poikilocytes)

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7
Q

What bilirubin is raised in haemolytic anaemia?

A

Unconjugated

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8
Q

What are haptoglobin levels in HA?

A

Reduced

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9
Q

What Ig in cold vs warm HA?

A 
Warm = IgG -> extravascular 
Cold = IgM -> intravascular
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10
Q

Which HA presents with Raynaud’s?

A

Cold

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11
Q

Which HA occurs in CLL, SLE, methyldopa use?

A

Warm

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12
Q

Which HA occurs in EBV and mycoplasma infections?

A

Cold

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13
Q

Which HA in lymphoma?

A

Cold or warm

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14
Q

Which tx for cold HA?

A

Supportive + chlorambucil chemo

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15
Q

Which tx for warm HA?

A

Steroids, splenectomy, immunosuppress

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16
Q

Which abs in PNH?

A

Donath-Landsteiner IgG ,anti-RBCs - on rewarming cause complement-mediated HA

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17
Q

What is seen on blood film in immune HA?

A

Spherocytes and agglutination

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18
Q

What does the DAT vs IAT look for?

A

DAT - looks at surface of RBCs for complement/Abs

IAT - looks at serum

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19
Q

3 non-immune HA.

A

Malaria
MAHA
PNH (Ham’s test +ve)

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20
Q

Which malignancy can cause MAHA?

A

Adenocarcinoma –> granules into circulation –> pro-coagulant –> low-grade DIC + RBC fragmentation

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21
Q

What mutation causes TTP?

A

ADAMTS13 –> lack of vWF cleaving protease so it builds up to shred RBCs

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22
Q

Over what % myeloblasts is abnormal in BM?

A

> 5%

23
Q

3 infections without neutrophilia?

A

Viral
Brucella
Typhoid

24
Q

What malignancy: neutrophilia + basophilia + myelocytes + splenomegaly = ?

A

CML

25
Q

What malignancy: neutropenia + myeloblasts = ?

A

AML

26
Q

What does the FIP1L1-PDGFRa fusion gene cause?

A

Chronic eosinophilic leukaemia

27
Q

Which viral infections cause monocytosis?

A

CMV

VZV

28
Q

Which bacterial infections cause monocytosis?

A

EBV
CMV
Toxoplasmosis

29
Q

What technique is used to determine clonality of B cells?

A

light chain restriction

30
Q

What is normal B cell clonality?

A

Polyclonal k:l 60:40

Abnormal would be monoclonal e.g. k:l 99:1

31
Q

Which technique used to decide if myeloid or lymphoid?

A

Immunophenotyping

32
Q

What are the main 3 types of haem mutations?

A

Type 1 - pro-proliferation
Type 2 - anti-differentiation
Anti-apoptosis - pro-survival

33
Q

Name an anti-apoptosis mutation.

A

BCL-2

34
Q

Name a pro-proliferation mutation.

A

BCR-ABL, JAK2

35
Q

Name an anti-differentiation mutation.

A

PML-PARA

36
Q

Which neoplasm is TdT +ve, CD19 +ve , surface Ig -ve?

A

B cell ALL

37
Q

What does TdT +ve mean?

A

Immature cells - B

38
Q

What does TdT -ve, Ig +ve and CD138 +ve indicate?

A

MM

39
Q

Intravascular causes of haemolysis

A 
Malaria (MOST COMMON)
G6PdD
ABO
Cold AIHA
Drugs 
MAHA
PNH
40
Q

What is the mutation in Gilbert’s?

A

UGT1A1

41
Q

Which anaemia has alpha and beta spectrin and protein 4.1?

A

Elliptocytosis

42
Q

Which anaemia has band 3, protein 4.2, beta spectrin and ankyrin?

A

Spherocytosis

43
Q

What is the inheritance of spherocytosis and elliptocytosis?

A

AD for both

44
Q

What tests can be done in spherocytosis?

A

Osmotic fragility

Less Eosin-5-maleimide binding on flow cytometry

45
Q

What does the homozygous state for elliptocytosis cause?

A

Hereditary pyropoikilocytosis - fragmentation, budding, vesciculation of membrane and variation in size of RBCs

46
Q

What is the inheritance of G6PD?

A

X linked recessive

47
Q

Role of G6PD normally?

A

Generates NADPH to protect cell against oxidative stress

48
Q

What cell types are seen in G6PD?

A

Hemi ghosts (Hb on one side)
Bite cells
Nucleated RBC

49
Q

What is seen on G6PD slide when stained with methylviolet?

A

Heinz bodies = deformed haemoglobin peripherally

50
Q

Which pathway is defective in PK deficiency?

A

Glycolytic

51
Q

What cells are seen in PKD?

A

Echinocytes - short projections on RBCs

Spherocytes

52
Q

Which HA is basophilic stippling seen in?

A

Pyrimidine 5’-nucleotidase deficiency (uncommon) - nucleotide metabolism pathway defect

53
Q

When can splenectomy be done in HA?

A

> 3yo but <10yo for maximal benefit before puberty

Pathology Summaries (23 decks)

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