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MRCP Part 1 > Hematology & Oncology > Flashcards

Hematology & Oncology Flashcards

1
Q

In Sideroblastic anaemia
Which stain should be applied to a blood film to show ring sideroblasts ?

A

Perl’s stain

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2
Q

Causes of Sideroblastic anaemia

A

Congenital cause:
-delta-aminolevulinate synthase-2 deficiency

Acquired causes
- MDS
- alcohol
- lead
- anti-TB medications

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3
Q

Treatment of Sideroblastic anaemia?

A

Management
- supportive
- treat any underlying cause
- pyridoxine may help

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4
Q

Vit B12 Absorbed in …….1…. After binding to …….2…. Which secreted from ……….3…….

A
  1. absorbed in the terminal ileum
  2. intrinsic factor (3. secreted from parietal cells in the stomach)
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5
Q

Causes of vitamin B12 deficiency

A
  1. pernicious anaemia: most common cause
  2. post gastrectomy
  3. vegan diet or a poor diet
  4. disorders/surgery of terminal ileum (site of absorption)
    Crohn’s: either diease activity or following ileocaecal resection
  5. metformin (rare)
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6
Q

Features of vitamin B12 deficiency?

A
  1. macrocytic anaemia
  2. sore tongue and mouth
  3. neurological symptoms
    the dorsal column is usually affected first (joint position, vibration) prior to distal paraesthesia
  4. neuropsychiatric symptoms: e.g. mood disturbances
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7
Q

Management of B12 deficiency

A

if no neurological involvement 1 mg of IM hydroxocobalamin 3 times each week for 2 weeks, then once every 3 months

if a patient is also deficient in folic acid then it is important to treat the B12 deficiency first to avoid precipitating subacute combined degeneration of the cord

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8
Q

Causes of Microcytic anaemia

A
  1. iron-deficiency anaemia
  2. thalassaemia*
  3. congenital sideroblastic anaemia
    anaemia of chronic disease (more commonly a normocytic, normochromic picture)
  4. lead poisoning
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9
Q

In normal haemoglobin level associated with a microcytosis. In patients not at risk of thalassaemia, this should raise the possibility of ……

A

polycythaemia rubra vera which may cause an iron-deficiency secondary to bleeding.

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10
Q

New onset microcytic anaemia in elderly patients should be urgently investigated to exclude……..

A

underlying malignancy

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11
Q

Megaloblastic causes of macrocytic anaemia

A
  1. vitamin B12 deficiency
  2. folate deficiency
  3. secondary to methotrexate
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12
Q

Normoblastic causes of macrocytic anaemia

A
  1. alcohol
  2. liver disease
  3. hypothyroidism
  4. pregnancy
  5. reticulocytosis
  6. myelodysplasia
  7. drugs: cytotoxics
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13
Q

Causes of normocytic anaemia include

A
  1. anaemia of chronic disease
  2. chronic kidney disease
  3. aplastic anaemia
  4. haemolytic anaemia
  5. acute blood loss
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14
Q

Vitamin B12 deficiency can be classified into the following three groups using serum vitamin B12 level:
Deficiency is likely: < ….

Deficiency is probable: ……

Deficiency is unlikely: >…..

A
  • Deficiency is likely: <148 picomole/L
  • Deficiency is probable: 148 to 258 picomole/L
  • Deficiency is unlikely: >258 picomole/L
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15
Q

What is the mechanism of anemia of chronic disease?

A

Decreased availability of iron , relatively decreased level of erythropoietin and mild decreased in the lifespan of RBC

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16
Q

Increased in Hepcidin level leads to

A

Reduce release of iron from macrophages + dietary iron absorption

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17
Q

Hepcidin synthesized in

A

Liver

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18
Q

Hepcidin
1. High in ………
2. Low in …….

A
  1. Inflammation&raquo_space;> decreased serum iron
  2. Hemochromatosis > low Hepcidin level&raquo_space;> iron overload
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19
Q

Hepcidin inhibits iron transport by binding to …….

A

the iron export channel ferroportin

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20
Q

CKD anemia when GFR <

A

GFR < 35

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21
Q

Metabolic acidosis inhibits conversion of ferric iron (FE 3+) to absorbable form …..

A

Ferrous iron ( Fe2+) in the duodenum&raquo_space;> reduced iron absorption

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22
Q

Patient on Erythropoietin or hemodialysis is require ? Which of the following

  1. Iv iron
    Or
  2. Oral iron
A

IV iron

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23
Q

Target Hb in CKD ?

A

Hb 10-12

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24
Q

In aplastic anemia bone marrow assessment is best made on …

A

Trephine biopsy, which often shows replacement of the normal cellular marrow by ( fatty marrow)

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25
Q

Hypocelullar bone marrow seen in …

A

Aplastic anemia

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26
Q

Causes of aplastic anemia

A

Idiopathic
Fanconi anemia
Dyskeratosis congenita
Drugs: cytotoxic, chloramphenicol, sulphonamides , phenytoin, GOLD

Infection: parvovirus, hepatitis

Radiation

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27
Q

Treatment of aplastic anemia

A
  • Supportive: blood products, prevention and treatment of infection
  • anti thymocyte and anti lymphocyte globulin
  • immunosuppressive agents ( ciclosporin) may be given
  • Allogeneic stem cell transplantation
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28
Q

anti thymocyte and anti lymphocyte globulin can cause

A

Serum sickness ( fever , rash , arthralgia )

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29
Q

Intravascular haemolysis: causes

A
  1. mismatched blood transfusion
  2. G6PD deficiency*
  3. red cell fragmentation: heart valves, TTP, DIC, HUS
  4. paroxysmal nocturnal haemoglobinuria
  5. cold autoimmune haemolytic anaemia
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30
Q

Extravascular haemolysis: causes

A
  1. haemoglobinopathies: sickle cell, thalassaemia

hereditary spherocytosis
haemolytic disease of newborn
warm autoimmune haemolytic anaemia

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31
Q

6 features of Fanconi anemia ?

A
  • Autosomal recessive
  • Aplastic anemia
  • Risk of AML
  • Neurological manifestation
  • Skeletal abnormalities
  • Skin pigmentation ( café au lait spots )
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32
Q

Normal types of Hb

A
  1. Adult HbA ( 2 alpha & 2 Beta) normal > 95%
  2. Adult HbA2 ( 2 alpha & 2 gamma ) normal < 3.5 %
  3. Fetal Hb ( 2 alpha & 2 delta ) normal < 1.5 %
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33
Q
  1. Alpha globulin gene are located on …..
  2. Beta globulin gene are located on …..
A

I. Chromosome 16

  1. Chromosome 11
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34
Q

Types of alpha thalassemia

A
  1. Silent carrier: single alpha gene mutation ( patient is Healthy)
  2. Alpha thalassemia trait ( minor ) 2 gene mutation ( Hb ~ 10 & microcytosis& normal Hb Electrophoresis )
  3. HbH: 3 gene mutation: severe anemia
  4. Alpha thalassemia major ( Hb barts or hydrops fetalis ) 4 gene mutation&raquo_space;> death in utero.
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35
Q

Beta thalassemia trait

A
  • Autosomal recessive
  • Mild hypochromic , microcytic anemia
  • Asymptomatic
  • HbA2 raised > 3.5 %
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36
Q

Beta thalassemia features

A
  1. Microcytic anemia
  2. HbA2 & HbF raised
  3. HbA absent
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37
Q

Beta thalassemia management

A

Repeated transfusion

  • this leads to iron overload&raquo_space; organ failure
  • iron chelation therapy is therefore important
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38
Q

Norma serum gastrin level excludes …..

A

Pernicious anemia

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39
Q

Causes of warm AIHA

A

idiopathic

autoimmune disease: e.g.systemic lupus erythematosus*

neoplasia

lymphoma

chronic lymphocytic leukaemia

drugs: e.g. methyldopa

*MAN CIL

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40
Q

Management of warm AIHA

A

treatment of any underlying disorder

steroids (+/- rituximab)are generally used first-line

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41
Q

The antibody in cold AIHA is usuallyIgMand causes haemolysis best at ….. degree C.

A

at 4 degree Celsius.

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42
Q

In which type of autoimmune hemolytic anema,Patients respond less well to steroids ?

A

Cold AIHA

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43
Q

Causes of cold AIHA

A

neoplasia: e.g. lymphoma

infections: e.g. mycoplasma, EBV

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44
Q

FFP indications

A
  1. PT ratio or activated partial thromboplastin time (APTT) ratio > 1.5
  2. can be used prophylactically in patients undergoing invasive surgery where there is a risk of significant bleeding.
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45
Q

Cryoprecipitate contains ?

A

concentrated Factor VIII:C,
von Willebrand factor,
fibrinogen,
Factor XIII and
fibronectin, produced by further processing of Fresh FFP.

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46
Q

HbA1C higher then expected in

A

Due to increased RBC span

  1. B12 deficiency
  2. Folate deficiency
  3. IDA
  4. Splenectomy
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47
Q

neonatal jaundice is often seen

intravascular haemolysis

gallstones are common

splenomegaly may be present

Heinz bodieson blood films.Bite and blister cellsmay also be seen

A

G6PD deficiency

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48
Q

The diagnosis of G6pD deficiency is made by

A

G6PD enzyme assay

Should be checked 3 months after acute episode of hemolysis

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49
Q

Drugs causing hemolysis in G6pD deficiency

A

anti-malarials: primaquine

ciprofloxacin

sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas

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50
Q

Drugs safe in G6pD deficiency

A

penicillins

cephalosporins

macrolides

tetracyclines

trimethoprim

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51
Q

Diagnosis of hereditary spherocytosis by

A

EMA binging test and the cryohaemolysis test

for atypical presentations electrophoresis analysis of erythrocyte membranes is the method of choice

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52
Q

Management of hereditary spherocytosis

A

acute haemolytic crisis:

  • treatment is generally supportive
  • transfusion if necessary

longer term treatment:

  • Folate replacement
  • splenectomy
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53
Q

PNH diagnosis by

A

Flow cytometry of blood to detect low levels of CD 59 & 55

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54
Q

treatment of PNH

A

blood product replacement

anticoagulation

eculizumab, a monoclonal antibody directed against terminal protein C5, is currently being trialled and is showing promise in reducing intravascular haemolysis

stem cell transplantation

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55
Q

Blood film
In Hyposplenism e.g. post-splenectomy,coeliac disease

A

target cells

Howell-Jolly bodies

Pappenheimer bodies

siderotic granules

acanthocytes

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56
Q

Blood film in IDA

A

target cells

‘pencil’ poikilocytes

if combined with B12/folate deficiency a ‘dimorphic’ film occurs with mixed microcytic and macrocytic cells

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57
Q

Blood film in MF

A

‘tear-drop’ poikilocytes

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58
Q

Blood film in Intravascular haemolysis

A

Schistocytes

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59
Q

Blood film in megaloblastic anaemia

A

Hypersegmented neutrophils

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60
Q

Heinz bodies in

A

G6PD
Alpha thalassemia

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61
Q

Howell jolly bodies in

A

Hyposplenism

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62
Q

Burr cells ( echinocytes ) in

A

Uremia
Pyruvate kinase deficiency

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63
Q

Acanthocytes in

A

Abetalipoproteinemia

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64
Q

Basophilic stippling

A

Lead poisoning

Thalassaemia

Sideroblastic anaemia

Myelodysplasia

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65
Q

Spherocytes seen in

A

Hereditary spherocytosis

Autoimmune hemolytic anaemia

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66
Q

Target cells in

A

Sickle-cell/thalassaemia

Iron-deficiency anaemia

Hyposplenism

Liver disease

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67
Q

Schistocytes (‘helmet cells’) in

A

Intravascular haemolysis

Mechanical heart valve

DIC

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68
Q

McArdle’S Disease

A

Muscle pain
Cramps
Autosomal recessive
Deficiency of myophosphorylase
Low lactate
Exercise related

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69
Q

Drug-induced pancytopaenia

A

cytotoxics

antibiotics: trimethoprim, chloramphenicol

anti-rheumatoid: gold, penicillamine

carbimazole*

anti-epileptics: carbamazepine

sulphonylureas: tolbutamide

*causes both agranulocytosis and pancytopaenia

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70
Q

Features of TTP

A

fever

fluctuating neuro signs(microemboli)

microangiopathic haemolytic anaemia

thrombocytopenia

renal failure

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71
Q

Causes of TTP

A

post-infection e.g. urinary, gastrointestinal

pregnancy

drugs: ciclosporin, oral contraceptive pill, penicillin, clopidogrel, aciclovir

tumours

SLE

HIV

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72
Q

TTP MANAGEMENT

A

no antibiotics - may worsen outcome

plasma exchangeis the treatment of choice

steroids, immunosuppressants

vincristine

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73
Q

Features of Wiskott-Aldrich syndrome

A

recurrent bacterial infections (e.g. Chest)

eczema

thrombocytopaenia

low IgM levels

74
Q

Wiskott-Aldrich syndrome causes primary immunodeficiency due to a combination……….

A

B and T cell dysfunction

75
Q

ITP MANAGEMENT

A
  • first-line treatment for ITP isoral prednisolone ( 80 % of patients respond )
  • IVIGmay also be used
    it raises the platelet count quicker than steroids, therefore may be used if active bleeding or an urgent invasive procedure is required
  • splenectomy if plt < 30 after 3 months
  • immunosuppressive drugs : cyclophosphamide
76
Q

What is Evan’s syndrome

A

ITP in association with autoimmune haemolytic anaemia (AIHA)

77
Q

In ITP
Antibodies are directed against…………….?

A

Glycoprotein IIb-Ib complex

78
Q

Plt transfusion in active bleeding

A
  1. Plt < 30 with clinical significant bleeding
  2. Plt < 100 with severe bleeding or bleeding at critical sites , such as CNS
79
Q

Plt transfusion in Pre-invasive procedure (prophylactic)

A

> 50×109/L for most patients

50-75×109/L if high risk of bleeding

> 100×109/L if surgery at critical site

80
Q

What is the threshold of plt transfusion if no active bleeding or no planned invasive procedure

A

10

81
Q

platelet transfusions have the highest risk of ……………… compared to other types of blood product.

A

bacterial contamination

82
Q

Features of hemophilia

A

haemoarthroses

haematomas

prolonged bleeding after surgery or trauma

83
Q

PT and APTT and Bleeding time in hemophilia

A

Prolonged aPTT
Normal PT, Thrombin time and bleeding time

84
Q

Von willebrand’s disease investigation

A

prolonged bleeding time

APTT may be prolonged

factor VIII levels may be moderately reduced

defective platelet aggregation with ristocetin

85
Q

Von willebrand’s disease
Management

A
  • tranexamic acid for mild bleeding
  • desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells
  • factor VIII concentrate
86
Q

desmopressin raises levels of vWF by inducing release of vWF from………?

A

Weibel-Palade bodies in endothelial cells

87
Q

Treatment of DVT if the patient has active cancer

A
  • previously LMWH was recommended
  • the new guidelines now recommend using a DOAC, unless this is contraindicated
88
Q

Treatment of DVT if renal impairment is severe (e.g. < 15/min)

A

LMWH, unfractionated heparin or LMWH followed by a VKA

89
Q

Treatment of DVT if the patient has antiphospholipid syndrome (specifically ‘triple positive’ in the guidance)

A

LMWH followed by a VKA should be used

90
Q

Pathophysiology of DVT/PE in pregnancy?

A
  • increase in factors VII, VIII, X and fibrinogen
  • decrease in protein S
  • uterus presses on IVC causing venous stasis in legs
91
Q

Management of DVT/PE in pregnancy?

A
  • warfarin contraindicated
  • S/C low-molecular weight heparin preferred to IV heparin (less bleeding and thrombocytopenia)
92
Q

what is the most common cause of thrombophilia

A
  • factor V Leiden (activated protein C resistance)
  • prothrombin gene mutation: second most common cause
93
Q

Features of protein C deficiency

A
  • VTE
  • skin necrosis following the commencement of warfarin: when warfarin is first started biosynthesis of protein C is reduced. This results in a temporary procoagulant state after initially starting warfarin, normally avoided by concurrent heparin administration. Thrombosis may occur in venules leading to skin necrosis
94
Q

Antithrombin III inhibits …….

A

thrombin, factor X and IX.

It mediates the effects of heparin

95
Q

Management of Antiphospholipid syndrome in pregnancy

A
  • low-dose aspirin should be commenced once the pregnancy is confirmed on urine testing
  • low molecular weight heparin once a fetal heart is seen on ultrasound. This is usually discontinued at 34 weeks gestation
  • these interventions increase the live birth rate seven-fold
96
Q

The best site for IM adrenaline injection is

A

theanterolateral aspect of the middle third of the thigh.

97
Q

Refractory anaphylaxis defined as

A

respiratory and/or cardiovascular problems persistdespite 2 doses of IM adrenaline

98
Q

Serumtryptaselevels are sometimes taken in such patients as they remain elevated for up to ………….. following an acute episode of anaphylaxis

A

12 hours

99
Q

Types of Cryoglobulinaemia

A

type I (25%):

monoclonal - IgG or IgM
associations: multiple myeloma, Waldenstrom macroglobulinaemia

type II (25%)

mixed monoclonal and polyclonal: usually with rheumatoid factor
associations:hepatitis C, rheumatoid arthritis, Sjogren’s, lymphoma

type III (50%)
- polyclonal: usually with rheumatoid factor
-‘associations: rheumatoid arthritis, Sjogren’s

100
Q

Features of Cryoglobulinaemia

A
  • Raynaud’s only seen in type I
  • cutaneous ( vascular purpura, distal ulceration, ulceration)
  • arthralgia
  • renal involvement (diffuse glomerulonephritis)
101
Q

Investigations of Cryoglobulinaemia

A
  • High ESR
  • Low complement ( especially C4)
102
Q

Treatment of Cryoglobulinaemia

A

treatment of underlying condition e.g. hepatitis C

immunosuppression

plasmapheresis

103
Q

Pulmonary causes of Eosinophilia

A
  1. asthma
  2. allergic bronchopulmonary aspergillosis
  3. Churg-Strauss syndrome
  4. Loffler’s syndrome
  5. tropical pulmonary eosinophilia
  6. eosinophilic pneumonia
  7. hypereosinophilic syndrome
104
Q

Infective causes of Eosinophilia

A
  1. schistosomiasis
  2. nematodes: Toxocara, Ascaris, Strongyloides
  3. cestodes: Echinococcus
105
Q

Other causes of eosinophilia

A
  1. drugs: sulfasalazine, nitrofurantoin
  2. psoriasis/eczema
  3. eosinophilic leukaemia (very rare)
106
Q

Causes of Hyposplenism

A
  1. splenectomy
  2. sickle-cell
  3. coeliac disease, dermatitis herpetiformis
  4. Graves’ disease
  5. systemic lupus erythematosus
  6. amyloid
107
Q

Features of Hyposplenism

A
  1. Howell Jolly bodies
  2. Siderocytes
108
Q

Causes of splenomegaly

A

myelofibrosis

chronic myeloid leukaemia

visceral leishmaniasis (kala-azar)

malaria

Gaucher’s syndrome

portal hypertension e.g. secondary to cirrhosis

lymphoproliferative disease e.g. CLL, Hodgkin’s

haemolytic anaemia

infection: hepatitis, glandular fever

infective endocarditis

sickle-cell*, thalassaemia

rheumatoid arthritis (Felty’s syndrome)

109
Q

Causes of Massive splenomegaly

A
  1. MF
  2. CML
  3. MALARIA
  4. visceral leishmaniasis
  5. Gaucher’s syndrome
110
Q

Methaemoglobinaemia describes haemoglobin which has beenoxidised from ……..

A

Fe2+ to Fe3+.

111
Q

chocolate’ cyanosis

dyspnoea, anxiety, headache

severe: acidosis, arrhythmias, seizures, coma

normal pO2 but decreased oxygen saturation

Features of…..?

A

Methaemoglobinaemia

112
Q

Causes of Methaemoglobinaemia

A

Congenital causes
1. haemoglobin chain variants: HbM, HbH

  1. NADH methaemoglobin reductase deficiency

Acquired causes

  1. drugs: sulphonamides,nitrates (including recreational nitrates e.g. amyl nitrite ‘poppers’),dapsone, sodium nitroprusside, primaquine
  2. chemicals: aniline dyes
113
Q

Treatment of Methaemoglobinaemia

A
  • For NADH methaemoglobinaemia reductase deficiency:»> ascorbic acid
  • IV methylthioninium chloride (methylene blue) if acquired
114
Q

Investigations of Hereditary angioedema

A

C1-INH level is low during an attack

low C2 and C4 levels are seen, even between attacks. Serum C4 is the most reliable and widely used screening tool

115
Q

Management of Hereditary angioedema

A

acute
- HAE does not respond to adrenaline, antihistamines, or glucocorticoids

  • IV C1-inhibitor concentrate, fresh frozen plasma (FFP) if this is not available

prophylaxis: anabolic steroid Danazol may help

116
Q

Selective oEstrogen Receptor Modulators (SERM) is used in the management of……..

A

oestrogen receptor-positive breast cancer.

117
Q

Selective oEstrogen Receptor Modulators (SERM)

Side effects

A

menstrual disturbance: vaginal bleeding, amenorrhoea

hot flushes - 3% of patients stop taking tamoxifen due to climacteric side-effects

venous thromboembolism

endometrial cancer

118
Q

Anastrozole and letrozole arearomatase inhibitors that reduces peripheral oestrogen synthesis.
Side effects?

A

osteoporosis

NICE recommends a DEXA scan when initiating a patient on aromatase inhibitors for breast cancer

hot flushes

arthralgia, myalgia

insomnia

119
Q

IgG4-related disease

A

Riedel’s Thyroiditis

Autoimmune pancreatitis

Mediastinal and Retroperitoneal Fibrosis

Periaortitis/periarteritis/Inflammatory aortic aneurysm

Kuttner’s Tumour (submandibular glands) & Mikulicz Syndrome (salivary and lacrimal glands)

Possibly sjogren’s and primary biliary cirrhosis

120
Q

Severity of neutropenia

A

Mild : 1.0 - 1.5

Moderate: 0.5 - 1.0

Severe: < 0.5

121
Q

Causes of neutropenia

A
  1. viral
    - HIV
    - Epstein-Barr virus
    - hepatitis

2.drugs
- cytotoxics
- carbimazole
- clozapine

  1. benign ethnic neutropaenia
    - common in people of black African and Afro-Caribbean ethnicity
    - requires no treatment
  2. haematological malignancy
    - MDS
    - aplastic anemia
  3. rheumatological conditions
    - SLE, RA: in Felty’s syndrome
  4. severe sepsis
  5. haemodialysis
122
Q

Definition of neutropenic sepsis

A

Neutrophil coun < 0.5 plus one of the following

  1. T > 38
  2. Signs or symptoms consistent with clinically significant sepsis
123
Q

Aetiology of neutropenic sepsis

A

coagulase-negative, Gram-positive bacteria are the most common cause, particularlyStaphylococcus epidermidis

this is probably due to the use of indwelling lines in patients with cancer

124
Q

Management of neutropenic sepsis

A
  • antibiotics must be started immediately, do not wait for the WBC
  • starting empirical antibiotic therapy withTazocin immediately
  • if patients are still febrile and unwell after 48 hours an alternative antibiotic such as meropenem is often prescribed +/- vancomycin
  • if patients are not responding after 4-6 days the Christie guidelines suggest ordering investigations for fungal infections (e.g. HRCT), rather than just starting therapy antifungal therapy blindly
  • there may be a role for G-CSF in selected patients
125
Q
  1. autosomal dominant
  2. defect in porphobilinogen deaminase
  3. female and 20-40 year olds more likely to be affected
  4. typically present with abdominal symptoms, neuropsychiatric symptoms, HTN and tachycardia.
  5. urine turns deep red on standing

What is the diagnosis?

A

Acute intermittent porphyria (AIP)

126
Q

Acute intermittent porphyria (AIP), defect in

A

porphobilinogen deaminase

127
Q

classically photosensitive rash with bullae, skin fragility on face and dorsal aspect of hands

urine: elevated uroporphyrinogen and pink fluorescence of urine under Wood’s lamp

What is the diagnosis?

A

Porphyria cutanea tarda (PCT)

128
Q

Treatment of Porphyria cutanea tarda (PCT)

A

Chloroquine

129
Q

Porphyria cutanea tarda (PCT), the defect in ……

A

uroporphyrinogen decarboxylase

130
Q

photosensitive blistering rash

abdominal and neurological symptoms

more common in South Africans

What is the diagnosis?

A

Variegate porphyria

131
Q

Variegate porphyria, the defect in ……

A

protoporphyrinogen oxidase

132
Q

Management of Acute intermittent porphyria

A
  • avoiding triggers
  • acute attacks
  1. IV haematin/haem arginate
  2. IV glucose should be used if haematin/haem arginate is not immediately available
133
Q

In polycythaemia vera
ESR level is …..
Leukocyte alkaline phosphate…..

A

ESR LOW

Leukocyte alkaline phosphate high

134
Q

Treatment of polycythemia Vera

A
  • aspirin
    reduces the risk of thrombotic events
  • venesection
    first-line treatment to keep the haemoglobin in the normal range
  • chemotherapy
    hydroxyurea - slight increased risk of secondary leukaemia
    phosphorus-32 therapy
135
Q

……….% of patients progress to acute leukaemia

A

5-15

136
Q

Causes of thrombocytosis

A
  1. reactive: platelets are an acute phase reactant - platelet count can increase in response to stress such as a severe infection, surgery. Iron deficiency anaemia can also cause a reactive thrombocytosis

2.malignancy

3.essential thrombocytosis, or myeloproliferative disorder

  1. hyposplenism
137
Q

Management of ET

A
  • hydroxyurea (hydroxycarbamide) is widely used to reduce the platelet count
  • interferon-α is also used in younger patients
  • low-dose aspirin may be used to reduce the thrombotic risk
138
Q

CA 125

A

Ovarian cancer

139
Q

CA 19-9

A

Pancreatic cancer

140
Q

CA 15-3

A

Breast cancer

141
Q

CEA

A

Colorectal cancer

142
Q

S-100

A

Melanoma,schwannomas

143
Q

Bombesin

A

Small cell lung carcinoma, gastric cancer,neuroblastoma

144
Q

Thymoma is Associated with

A

myasthenia gravis (30-40% of patients with thymoma)

red cell aplasia

dermatomyositis

also : SLE, SIADH

145
Q

Causes of death in thymoma

A

compression of airway

cardiac tamponade

146
Q

the most common tumour of theanterior mediastinumis …..

A

Thymoma

147
Q

Prevention of TLS

A

IV fluids

patients are higher risk should receive eitherallopurinol or rasburicase

148
Q

Laboratory tumor lysis syndrome

A

2 or more of the following

  1. uric acid > 475umol/l or 25% increase
  2. K > 6 mmol/l or 25% increase
  3. phos > 1.125mmol/l or 25% increase
  4. Ca < 1.75mmol/l or 25% decrease
149
Q

Cairo-Bishop score

A
  • Laboratory tumor lysis syndrome: abnormality in two or more of the following, occurring within three days before or seven days after chemotherapy.
  1. uric acid > 475umol/l or 25% increase
  2. K > 6 mmol/l or 25% increase
  3. phos > 1.125mmol/l or 25% increase
  4. Ca < 1.75mmol/l or 25% decrease

Clinical tumor lysis syndrome: laboratorytumour lysis syndrome plus one or more of the following:

  1. increased serum creatinine (1.5 times upper limit of normal)
  2. cardiac arrhythmia
  3. sudden death
150
Q

Differentiating features of MGUS from myeloma

A

normal immune function

normal beta-2 microglobulin levels

lower level of paraproteinaemia than myeloma (e.g. < 30g/l IgG, or < 20g/l IgA)

stable level of paraproteinaemia

no CRAB

151
Q

X-rays: ‘rain-drop skull seen in

A

MM

152
Q

Diagnostic criteria of MM
1 major + 1 minor
Or
3 minor

A

Major
1. Plasmacytoma (as demonstrated on evaluation of biopsy specimen)

  1. 30% plasma cells in a bone marrow sample
  2. Elevated levels of M protein in the blood or urine

Minor criteria

  1. 10% to 30% plasma cells in a bone marrow sample.
  2. Minor elevations in the level of M protein in the blood or urine.
  3. Osteolytic lesions (as demonstrated on imaging studies).
  4. Low levels of antibodies (not produced by the cancer cells) in the blood.
153
Q

Markers of poor prognosis in MM

A
  1. High B2 microglobulin
  2. Low Albumin
154
Q

Management of Waldenstrom’s macroglobulinaemia

A

typically rituximab-based combination chemotherapy

155
Q

What is the most common symptom in SVC obstruction

A

Dyspnoea

156
Q

Management of SVC obstruction

A
  1. endovascular stenting is often the treatment of choice to provide symptom relief
  2. certain malignancies such as lymphoma, small cell lung cancer may benefit from radical chemotherapy or chemo-radiotherapy rather than stenting
  3. the evidence base supporting the use of glucocorticoids is weak but they are often given
157
Q

Management of spinal cord compression

A

high-dose oral dexamethasone

urgent oncological assessment for consideration of radiotherapy or surgery

158
Q

Good prognostic factors of ALL

A

French-American-British (FAB) L1 type

common ALL

pre-B phenotype

low initial WBC

del(9p)

159
Q

Poor prognostic factors of ALL

A

FAB L3 type

T or B cell surface markers

Philadelphia translocation, t(9;22)

age < 2 years or > 10 years

male sex

CNS involvement

high initial WBC (e.g. > 100 * 109/l)

non-Caucasian

160
Q

Poor prognostic features of AML

A

> 60 years

> 20% blasts after first course of chemo

cytogenetics: deletions of chromosome 5 or 7

161
Q

Acute promyelocytic leukaemia M3

associated with t(…….)

A

t(15;17)

162
Q

APML is treated with

A

all-trans retinoic acid (ATRA)

163
Q

Most common tumour causing bone metastases

A
  1. prostate
  2. breast
  3. lung

(in descending order)

164
Q

Breast cancer: risk factors

A
  1. BRCA1,BRCA2genes
  2. p53 gene mutations
  3. obesity
  4. OCP
  5. not breastfeeding
  6. nulliparity, 1st pregnancy > 30 yrs
  7. early menarche,late menopause
  8. 1st degree relative premenopausal relative with breast cancer
  9. previous surgery for benign disease (?more follow-up, scar hides lump)
165
Q

Burkitt’s lymphoma is associated with the……… translocation, usuallyt(……..)

A

thec-myc gene translocation, usuallyt(8:14)

166
Q

Complications of CLL

A
  1. Anemia
  2. Hypogammaglobulinaemia
  3. Warm autoimmune hemolytic anemia
  4. transformation to high-grade lymphoma(Richter’s transformation)
167
Q

Ritcher’s transformation is indicated by one of the following symptoms:

A

lymph node swelling

fever without infection

weight loss

night sweats

nausea

abdominal pain

168
Q

Indications for CLL treatment

A
  1. progressive marrow failure
  2. massive (>10 cm) or progressive lymphadenopathy
  3. massive (>6 cm) or progressive splenomegaly
  4. autoimmune cytopaenias e.g. ITP
  5. B symptoms
  6. progressive lymphocytosis: > 50% increase over 2 months or lymphocyte doubling time < 6 months
169
Q

Treatment of CLL

A

fludarabine, cyclophosphamide and rituximab (FCR)

170
Q

del 13q in CLL associated with

A

goodprognosis

171
Q

chromosome 17 (del 17p)in CLL associated with

A

Poor prognosis

172
Q

Cyclophosphamide can cause hemorrhagic…1……

incidence reduced by the use of ……2…

A
  1. Hemorrhagic Cystitis
  2. hydration andmesna
173
Q

t(11;14) in

A

Mantle cell lymphoma

174
Q

t(14;18) seen in

A

follicular lymphoma

175
Q

Malaria can lead to …….. lymphoma

A

Malaria can lead to Burkitt’s lymphoma

176
Q

Management of Hairy cell leukaemia

A

chemotherapy is first-line: cladribine, pentostatin

immunotherapy is second-line: rituximab, interferon-alpha

177
Q

Lead poisoning results indefective ….

A

ferrochelatase and ALA dehydratase function.

178
Q

Leucocyte alkaline phosphatase

Low in

A

CML
INFECTIOUS MONONUCLEOSIS
PNH
PERNICIOUS ANAEMIA

*CIPP

179
Q

Causes of leukaemoid reaction

A

severe infection

severe haemolysis

massive haemorrhage

metastatic cancer with bone marrow infiltration

180
Q

Normal pO2 but decreased oxygen saturation is characteristic of

A

methaemoglobinaemia

MRCP Part 1 (15 decks)

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