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MRCP > Haem/Onc > Flashcards

Haem/Onc Flashcards

1
Q

What is affected when someone has alpha thalassemia

A

the alpha globin alleles on Ch16 (2x2)

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2
Q

What would happen if all 4 alpha globin alleles on Ch16 were affected

A

death in utero

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3
Q

What would happen if 3 alpha globin alleles on Ch16 were affected?

A

HbH disease, hypochromic microcytic anaemia with splenomegaly

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4
Q

What would happen if alpha globin alleles on Ch16 were affected?

A

Hypochromic and microcytic, normal Hb

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5
Q

What is HbA

A

alpha2 + beta2 = normal Haemoglobin

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6
Q

What is beta thalassemia trait?

A

autosomal recessive
mild hypochromic microcytic anaemia. Microcytosis disproportional to anemia

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7
Q

What is beta thalassemia major

A

Ch11, abscence of beta chains
FTT and hepatomegaly
microcytic anaemia
raised HbA2 and HbF
no HbA

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8
Q

How is beta thalassemia major managed

A

Repeated transfusions + desferrioxamine to prevent iron overload and organ failure

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9
Q

What genetic abnormality leads to Burkitts lymphoma

A

c-myc gene translocation t(8:14)

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10
Q

What is each form of Burkitts associated with?

A

Sporadiac: abdominal tumours, HIV
Endemic: maxilla, mandible, EBV

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11
Q

What would a starry sky on microscopy indicate

A

Burkitts lymphoma

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12
Q

Management of Burkitts

A

Chemo with rasburicase prior as high rise of tumour lysis syndrome

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13
Q

Blood results to indicate tumour lysis syndrome

A

high: phosphate, potassium, uric acid
low: calcium

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14
Q

Prevention of tumour lysis syndrome

A

IV Fluids
low risk: allopurinol
high risk: rasburicase (uric acid ->allantoin

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15
Q

G6PD deficiency inheritance

A

X linked

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16
Q

What is G6PD deficiency

A

increased RBC susceptibility to oxidative stress

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17
Q

How does G6PD present

A

neonatal jaundice
intravascular haemolysis
gallstones

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18
Q

What would Heinz bodies on blood film indicate

A

G6PD def

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19
Q

How do you diagnose G6PD

A

G6PD enzyme assay 3 months after acute episode

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20
Q

Triggers for G6PD

A

primaquine, cipro sulph-
infection
broad beans

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21
Q

What is polycythemia?

A

Increase in red cell volume
Primary: polycytheameia vera
Secondary: COPD, OSA
Relative: dehydration, stress

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22
Q

What causes polycythaemia Vera?

A

Associated with JAK2 mutation
myeloproliferative disorder, proliferation of marrow stem cells -> increased red cell volume

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23
Q

Symptoms of PCV

A

pruritis after hot bath
splenomegaly
HTN
arterial/venous thrombosis (hyperviscosity)
haemorrhage

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24
Q

Investigation of PCV

A

FBC
JAK 2
Low ESR

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25
Q

Management PCV

A

aspirin: reduced VTE risk
venesection
cheo: hydroxyurea

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26
Q

Prognosis PCV

A

5-15% develop myelofibrosis or acute leukaemia

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27
Q

Cancers likely to cause SVCO

A

SCLC
lymphoma

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28
Q

Presentation of SVCO

A

SOB
swelling
headache
blurred vision
pulseless jugular venous distension

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29
Q

Management of SVCO

A

endovasc stenting
radial chemo (SLCL, lymphoma)
steroids

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30
Q

Von Willebrands disease inheritance

A

Type 1: autosomal rec
Type 3: autosomal dom

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31
Q

Presentation of vWD

A

epistaxis, menorrhagia

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32
Q

Types of vWD

A

1: partial reduction in vWF (80%)
2: abnormal form of vWF
3: no vWF

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33
Q

Investigations vWD

A

increased bleeding time
increased APTT
decreased factor VIII
defective platelet aggregation with ristocetin

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34
Q

defective platelet aggregation with ristocetin

A

Von Willebrands

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35
Q

Management of vWD

A

mild bleed: tranexamic acid
desmopression: induces release of vWF from endothelial cells
factor VIII

36
Q

Examples of acquired thrombophilia

A

anti-phospholipid syndrome
COCP

37
Q

most common inherited thrombophilia

A

Factor V Leiden (activated protein C resistance)

38
Q

Types of haemophilia

A

A: factor VIII def
B: factor IX def

38
Q

Genetic inheritance of haemophilia

A

X linked

38
Q

Presentation of haemophilia

A

haemoarthrosis
haematomas
increased bleeding after surgery/trauma

39
Q

Blood marker for haemophilia

A

raised APTT

39
Q

How would ITP present

A

epistaxis, petechia, purpura
thrombocytopenia, antiplatelet autoantibodies
Mx: pred, IVIG

40
Q

How would TTP present

A

The terrible pentad
1) thrombocytopenia
2) anaemia
3) fever
4) renal failure
5) neuro signs

41
Q

Pathophysio TTP

A

deficiency ADAMTS13 which breaks down vWF therefore increased vWF leads to platelet aggregation

42
Q

Causes of TTP

A

post infectious
pregnancy
tumours
SLE
HIV
ciclosporin, OCP, epn, clopi, acic

43
Q

What would schistocytes on blood film indicate

A

TTP
DIC

44
Q

Management of TTP

A

plasma exchange
steroids, immunosup

45
Q

What is DIC

A

coagulation and fibrinolysis disregulation resulting in clotting with bleeding
transmembrane glycoprotein (TF) release

46
Q

Diagnosis of DIC

A

low: platelets, fibrinogen
High: PT APTT D-dimer
Schistocytes

47
Q

Examples of microcytic anaemia

A

IDA
thalassemia
sideroblastic

48
Q

Examples of macrocytic anaemia

A

Vit B12 def*
folate def*
MTX*
alcohol
liver
hypothyroid

*megaloblastic

49
Q

Examples of normocytic anaemia

A

chronic disease
CKD
aplastic
haemolytic
acute blood loss

50
Q

Types of Sideroblastic anaemia

A

congenital: delta aminolevulinate syn 2 def
acquired: myelodys, alcohol, lead

51
Q

Pathophy sideroblastic anaemia

A

red cells fail to form completely

52
Q

Blood results of sideroblastic anaemia

A

microcytic anaemia
high ferritin, iron, transferritin
Bone marrow: sideroblasts
blood film: basophilic stippling

53
Q

Management of sideroblastic anaemia

A

supportive

54
Q

What is pernicious anaemia

A

antibodies to intrinsic factor and parietal cells causing reduced B12

55
Q

Presentation of pernicious anaemia

A

anaemia: tired, pale, SOB
neuro: peripheral neuropathy, weakness, neuro psych
lemon tinge (jaundice + anaemia)
sore tongue

56
Q

Investigations of pernicious anaemia

A

macrocytic anaemia
blow b12
Blood film: hypersegmented polymorphs
anti intrinsic factor antibodies - specific
anti parietal antibodies

57
Q

Management pernicious anaemia

A

IM vit B12

58
Q

Cause of aplastic anaemia

A

autoimmune destruction haematopoeitic stem cells (pancytopenia)
mostly unknown
radiation, chemo, Fanconis (autosome rec, short, cafe au lait, increased risk AML)

59
Q

presentation aplastic anaemia

A

RBC: fatigue, pallor
WBC: infections
Platelets: bleeding, petechiae

60
Q

Diagnosis aplastic anaemia

A

pancytopenia
raised erythropoietin
bone marrow: low haematopoietic stem cells

61
Q

Management aplastic anaemia

A

blood products
ATG ALG (+steroids)
stem cell transplant

62
Q

Types of haemolytic anaemia

A

warm: IgG, SLE, Mx steroids
Cold: IgM, lymphoma, EBV, Raynaurds, less steroid responsive

63
Q

What would +ve Coombs test indicate

A

Haemolytic anaemia

64
Q

What is SCD

A

autosomal rec, abnormal Hb synthesis

65
Q

Describe possible types of SCD

A

trait: HbAS
disease: HbSS
Mild disease: HbSC

66
Q

Pathophysio SCD

A

HbS polymerises at low o2 and sickles -> haemolyse -> block vessels

67
Q

Sickle cell diagnosis

A

haemoglobin electrophoresis

68
Q

Sickle cell management

A

crisis: rehydrate, o2, blood, exchange
long term: hydroxyurea (increases HbF)
Pneumococcal vaccine

69
Q

Presentation of Hodgkins lymphoma

A

lymphadenopathy: asyn, pain with alcohol
B symptoms
medistinal mass

70
Q

Ix Hodgkins lymphoma

A

normocytic anaemia
Reed sternberg cells (owl eye)
*most common type nodular sclerosing

71
Q

Staging used for lymphoma

A

Ann arbor
1: single lymph node
2: 2+ unilateral
3: bialteral
4: beyond lymph nodes

A: no systemic features
B: systemic features

72
Q

Management of Hodgkins

A

Chemo
Radio
Combo

73
Q

Prognostic features of Hodgkins

A

Bad: b symptoms
Good: lymphocyte predominant type

74
Q

Presentation NHL

A

lymphadenopathy
B symptoms
extranodular: pancytopenia, gastro, nerve palsies

75
Q

Management NHL

A

watchful waiting
Chemo or radio
Vaccines

76
Q

What causes myeloma

A

genetic mutations which occur as B-lymphocytes differentiate into mature plasma cells.

77
Q

Presentation of myeloma

A

CRABBI
Calcium, high, constipation, nausea, confusion
Renal damage
Anaemia
Bleeding
Bone pain (back)
Infections

78
Q

Investigations for myeloma

A

protein electrophoresis: blood IgA IgG
urine: Bence Jones
Bone marrow: increased plasma cells
Whole Body MRI
XR skull: rain drop skull

79
Q

Rain drop skull

A

Myeloma

80
Q

Diagnosis of myeloma

A

1 major + 1 minor OR 3 minor
Major: plasmacytoma, BM 30% plasma cells, increased M protein

Minor: osteolytic lesions, BM 10-30% plasma cells, minor increased M protein, low levels Abs bood

81
Q

CML important points

A

Philadelphia Ch T(9:22)
Granulocytes at different stages of maturation
Mx: imatinid (inhibits tyrosine kinase)

82
Q

AML important points

A

symptoms relating to BM failure
acute promyelocytic leukaemia
-t(15:17)
- 25yo
-DIC
- good prognosis

83
Q

ALL important points

A

kids 2-5
good prog: del (9p), FAB L1
bad prog: philadelphia t(9:22), FAB L3

84
Q
A

MRCP (19 decks)

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