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Medicine > Haem > Flashcards

Haem Flashcards

1
Q

symptoms of anaemia?

A

fatigue

SOB

faintness

palpitations

headache

tinnitus

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2
Q

signs of anaemia

A

pallor

hyperdynamic circulation: tachycardia, flow murmur (apical ESM), cardiac enlargement

ankle swelling w heart failure

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3
Q

types of microcytic anaemia?

A

Haem defect:

IDA

Anaemia of chronic disease

Sideroblastic/ Lead poisoning

Globin defect:

thalassaemia

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4
Q

causes of normocytic anaemia?

A

recent blood loss

bone marrow failure

renal failure

early anaemia of chronic disease

pregnancy (raised plasma volume)

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5
Q

causes of macrocytic anaemia?

A

megaloblastic:

folate/ B12 deficiency

anti folate drugs: methotrexate, phenytoin

cytotoxics: hydroxycarbamide

non-megaloblastic:

reticulocytosis

alcohol or liver disease

hypothyroidism

myelodysplasia

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6
Q

Causes of IDA?

A

increased loss:

GI bleeding, menorrhagia, hookworms

decreased intake:

poor diet

malabsorption: coeliac, crohns

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7
Q

Ix of Iron deficiency anaemia?

A

haematinics: low ferritin, high TIBC, low transferrin saturation, low Fe

Blood film: anisocytosis, poikilocytosis, pencil cells

OGD + Colonoscopy

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8
Q

Mx of Iron deficiency anaemia?

A

Ferrous Sulphate 200mg PO TDS

(se: GI upset)

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9
Q

what is sideroblastic anaemia?

A

ineffective erythropoiesis

  • increased iron absorption
  • iron loading in bone marrow -> ringed sideroblasts
  • haemosiderosis: endo, liver and cardiac damage
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10
Q

causes of sideroblastic anaemia?

A

congenital

acquired: myelodysplasia/ myeloproliferative disease
drugs: chemo, anti-TB, lead

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11
Q

Ix of sideroblastic anaemia?

A

microcytic anaemia and not responsive to iron

high ferritin, high serum Fe, normal TIBC

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12
Q

mx of sideroblastic anaemia?

A

tx underlying cause

pyridoxine may help

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13
Q

featuers of Beta thalassaemia trait?

A

mild anaemia - usually harmless

low MCV

high HbA2 (a2d2) and high HbF (a2y2)

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14
Q

Features of Beta thal major?

A

features develop from 3-6mo

  • severe anaemia, jaundice (haemolysis), FTT

extramedullary erythropoiesis: frontal bossing, maxillary overgrowth, hepatosplenomegaly

Hameochromatosis after 10 yrs (transfusions)

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15
Q

Ix of Beta thal major?

A

low Hb, low MCV, high HbF, high HbA2

Blood film: target cells and nucleated RBCs

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16
Q

Mx of Beta thal major?

A

lifelong transfusions

+ desferrioxamine for iron chelation

BM transplant may be curative

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17
Q

features of alpha thal trait?

A

asymptomatic

hypochromic microcytes

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18
Q

HbH disease?

A

type of alpha thal

moderate anaemia: may need transfusions

haemolysis: jaundice, hepatosplenomegaly

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19
Q

Hb Barts?

A

Fatal

hydrops fetalis -> death in utero

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20
Q

Blood film findings of B12/ Folate deficiency?

A

Hypersegmented PMN -> megaloblastic

oval macrocytes

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21
Q

Mx of Folate deficiency?

A

assess for underlying cause

give B12 first! (unless B12 level known to be normal)

-> may precipitate or worsen SACD if folate prescribed when b12 is low

Folate 5mg/d PO

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22
Q

Sources of Folate?

A

Diet: Green veg, nuts, liver

stores for 4mo in the body

absorption: proximal jejunum

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23
Q

Causes of Folate deficiency anaemia?

A

low intake: poor intake

increased demand: pregnancy, haemolysis, malignancy

malabsorption: coeliac, crohns

Drugs: anti folates e.g. phenytoin, methotrexate, alcohol

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24
Q

Bloods to Ix macrocytic anaemia?

A

LFTs: mild raised Br in folate/ B12 deficiency

TFT

Serum B12

Red cell folate: reflects body stores over 2-3 mo

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25
Q

Source of Vit B12?

A

meat fish and dairy (vegans likely deficient)

stores for 4 yrs

absorbed in terminal ileum bound to intrinsic factor (released from gastric parietal cells)

role: DNA and myelin synthesis

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26
Q

Causes of B12 deficiency?

A

low intake: vegans

low intrinsic factor: post gastrectomy, pernicious anaemia

terminal ileum: crohns, ileal resection, bacterial overgrowth

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27
Q

features of B12 deficiency?

A

general: symptoms of anaemia

lemon tinge- pallor + mild jaundice

glossitis (beefy, red tongue)

Neuro:

parasthesia

peripheral neuropathy

SACD

optic atrophy

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28
Q

what is subacute combined degeneration of the cord?

A

usually only caused by pernicious anaemia

combined symmetrical dorsal column loss and corticospinal tract loss

  • > distal sensory loss: esp joint position and vibration
  • > ataxia w wide gait and Rombergs +ve

Mixed UMN and LMN signs

  • spastic paraparesis
  • brisk knee jerks, absent ankle jerks

upgoing plantars

pain and temp remain intact

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29
Q

Ix of Vit B12 deficiency?

A

low Hb, high MCV

low WCC and platelets if severe

intrinsic factor Abs: specific

Parietal cell Abs: sensitive

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30
Q

Mx of Vit B12 deficiency?

A

Malabsorption: Parenteral B12 (hydroxycobalamin)

Dietary -> oral B12 (cyanocobalamin)

Parenteral B12 reverses neuropathy but not SACD

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31
Q

What is pernicious anaemia?

A

autoimmune atrophic gastritis caused by autoabs vs parietal cells or IF

-> low IF and low H+

assoc w other AI disease and Ca: 3x risk of gastric adenoca

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32
Q

myositis

interstitial lung disease

thickened and cracked skin of the hands (mechanic’s hands)

Raynaud’s phenomenon

A

Antisynthetase syndrome

subtype of dermatomyositis

anti Jo1 +ve

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33
Q

Causes of cold AIHA?

A

Mycoplasma/ EBV, CLL

idiopathic

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34
Q

Mx of cold AIHA?

A

avoid cold

Rituximab (anti-CD20) to deplete B cells

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35
Q

Ix of Cold AIHA?

A

Direct antiglobulin test +ve

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36
Q

Features of cold AIHA?

A

igM-mediated, bind @<4 degrees

often fix complement -> intravascular haemolysis

may cause agglutination -> acrocynanosis or Raynauds

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37
Q

Features of warm AIHA?

A

IgG mediated, bind @37 degrees

extravasc haemolysis and spherocytes

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38
Q

Ix of warm AIHA?

A

Direct antiglobulin test +ve

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39
Q

Causes of warm AIHA?

A

idiopathic

SLE

RA

CLL

Medications: NSAIDs

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40
Q

Mx of Warm AIHA?

A

immunosuppression

splenectomy

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41
Q

IgG Donath-Landsteiner Abs

A

Paroxysmal Cold Haemoglobinuria

rare

assoc w measles, mumps, chicken pox

IgG Donath-Landsteiner Abs bind RBCs in the cold and

-> complement mediated lysis on rewarming

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42
Q

Paroxysmal Cold Haemoglobinuria

?

A

assoc w measles, mumps, chicken pox

IgG Donath-Landsteiner Abs bind RBCs in the cold and

-> complement mediated lysis on rewarming

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43
Q

Features of Paroxysmal nocturnal haemoglobinuria?

A

Visceral viscous thrombosis (hepatic, mesenteric, CNS)

haemolysis and haemoglobinuria

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44
Q

Visceral viscous thrombosis (hepatic, mesenteric, CNS)

haemolysis and haemoglobinuria

absence of RBC anchor molecule -> lysis of RBCs

may also cause low Platelets + PMN

A

Paroxysmal nocturnal haemoglobinuria

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45
Q

Ix of Paroxysmal nocturnal haemoglobinuria?

A

Anaemia +/- thrombocytopenia +/- neutropenia

FACS: low CD55 and CD59

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46
Q

Mx of Paroxysmal nocturnal haemoglobinuria

A

Chronic disorder -> long term anticoagulation

Eculizumab (prevents complement MAC formation)

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47
Q

what organism is most assoc w HUS?

A

E coli O157:H7

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48
Q

features of Haemolytic Uraemic Syndrome?

A

Bloody diarrhoea and abdo pain precedes:

  • MAHA
  • Thrombocytopenia
  • Renal failure
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49
Q

Ix of HUS?

A

schistocytes (MAHA)

low Platelets

normal clotting

U+E, Ur/Cr deranged (renal impairment)

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50
Q

Mx of HUS?

A

usually resolves spontaneously

Exchange transfusion and dialysis may be needed

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51
Q

Deficiency of what causes TTP?

A

Genetic/ acquired deficiency of ADAMTS13

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52
Q

Pentad of Thrombotic Thrombocytopenic Purpura?

A

Fever

CNS signs: confusion, seizures

MAHA

thrombocytopenia

renal failure

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53
Q

Ix of TTP?

A

schistocytes

low PL

normal clotting

U+E/ UR/Cr deranged: renal impairment

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54
Q

Mx of Thrombotic Thrombocytopenic Purpura?

A

Plasmapheresis

Immunosuppression

Splenectomy

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55
Q

What is Pyruvate Kinase deficiency?

A

autosomal recessive defect in ATP synthesis

  • > rigid red cells phagocytosed in spleen
    features: splenomegaly, anaemia +/- jaundice
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56
Q

Ix of pyruvate kinase deficiency?

A

Pyruvate kinase enzyme assay

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57
Q

Mx of Pyruvate Kinase deficiency?

A

not needed

or transfusion +/- splenectomy

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58
Q

Pathology of G6PD deficiency?

A

X linked disorder

g6PD in pentose phosphate pathway -> low NADPH production -> low glutathione which is used to mop up free radicals that cause oxidative damage

-> increased RBC oxidative damage

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59
Q

Triggers of haemolysis in G6PD deficiency?

A

Fava beans

Mothballs (naphthalene)

infection

Drugs: antimalarials, henna, dapsone, sulphonamides

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60
Q

ix of G6Pd deficiency?

A

blood film: irregularly contracted cells, bite cells, Heinz bodies

G6PD assay after 8 wks of acute haemolysis

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61
Q

mx of G6PD deficiency?

A

treat underlying infection

stop and avoid precipitants

transfusion may be needed

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62
Q

mx of hereditary elliptocytosis?

A

most pts asymptomatic

auto dom defect-> elliptical RBCs

Folate (increased utilisation), rarely splenectomy

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63
Q

pathophysiology of hereditary spherocytosis?

A

auto dom defect in RBC membrane

spherocytes trapped in spleen -> extravasc haemolysis

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64
Q

features of hereditary spherocytosis?

A

Splenomegaly

Pigment gallstones

Jaundice

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65
Q

complications of hereditary spherocytosis?

A

aplastic crisis

megaloblastic crisis

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66
Q

Ix of hereditary spherocytosis?

A

Blood film: spherocytes

DAT -ve

increased osmotic fragility

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67
Q

Mx of hereditary spherocytosis?

A

folate

rarely -> splenectomy

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68
Q

pathogenesis of sickle cell?

A

point mutation in B globin gene: glu -> val

SCA: HbSS

Trait: HbAS

HbSS insoluble when deoxygenated -> sickling

sickle cells have lower life span -> haemolysis

sickle cells get trapped in microvasc -> thrombosis

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69
Q

Ix of sickle cell?

A

Hb 6-9, high reticulocytes/ Br

Blood film: sickle cells and target cells

Hb electrophoresis

Neonatal screening at birth: Guthrie Heel prick

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70
Q

presentation of sickle cell anaemia?

A

clinical features manifest 3-6 mo due to drop in HbF

triggers: infection, cold, hypoxia, dehydration

Splenomegaly -> may get sequestration crisis

Infarction: Stroke, spleen

Crises: pain, pulmonary

Kidney, Liver, Lung disease

Erection

Dactylitis

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71
Q

Complications of Sickle Cell Disease?

A

Sequestration crisis:

splenic pooling -> shock + severe anaemia

Splenic infarction: atrophy and hyposplenism

increased risk of infection: e.g. osteomyelitis

Aplastic crisis: parvovirus B19

Gallstones

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72
Q

Mx of chronic Sickle cell disease?

A

Folate

Pen V + immunisations if no spleen (pneumococcal, meningococcus, Hib)

hydroxycarbamide if freq crises

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73
Q

Mx of Acute Sickle cell crises?

A

A->E, call for senior help

Analgesia: Opioids IV

Good hydration

Give O2

keep warm

Ix for cause: e.g infection

Abx if suspect infection

Transfusion: exchange if severe

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74
Q

Features of DIC?

A

thrombosis and bleeding

increased APTT/PT/TT

low Platelets/ fibrinogen

high fibrin degradation products

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75
Q

Causes of DIC?

A

sepsis

malignancy esp APML

trauma

obstetric complications

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76
Q

Mx of DIC?

A

FFP

Platelets

Heparin

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77
Q

Haemophilia A vs B?

A

A: fVIII

B: fIX deficiency

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78
Q

Haemophilia features?

A

X-linked

affects males

present w haemarthroses, bleeding after surgery/ extraction

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79
Q

Ix of Haemophilia?

A

low fVIII: A, fIX: B assay

prolonged APTT, normal PT

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80
Q

Mx of Haemophilia A?

A

avoid IM Injections and NSAIDs

minor bleeds: desmopressin + tranexamic acid

Major bleeds: fVIII

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81
Q

von willebrands disorder features?

A

vWF: stabilises fVIII

binds platelets via Gp1b to damaged epithelium

if mild, APTT and bleeding time normal

if not: high APTT, bleeding time, normal Pl and decreased vWF

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82
Q

Mx of Von Willebrands Disease?

A

Desmopressin: raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells

tranexamic acid for mild bleeding

fVIII concentrates

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83
Q

Causes of thrombocytopenia?

A

decreased production:

BM failure: aplastic, infiltration, drugs

Megaloblastic anaemia

increased destruction:

immune - ITP, SLE, CLL, Heparin, viruses

non immune: DIC, TTP, HUS, PNH, anti-phospholipid

Splenic pooling:

portal HTN, sickle cell

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84
Q

features of idiopathic thrombocytopenic purpura?

A

children commonly post URTI

self limiting

in adults: F>M, long term

Anti-platelet abs present

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85
Q

mx of ITP?

A

conservative

steroids

IVIg

splenectomy

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86
Q

Causes of Functional platelet defects?

A

drugs: aspirin, clopidogrel

secondary to: uraemia, paraproteinaemias

Hereditary: bernard-soulier (Gp1b deficiency), Glanzmanns (GpIIb/IIIa deficiency)

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87
Q

features of coagulation disorders e.g. haemophilia/ VWD?

A

deep bleeding: muscles, joints, tissues

delayed but severe bleeding after injury

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88
Q

features of vascular or platelet disorder?

A

Bleeding into skin: purpura, petechiae, ecchymoses

Bleeding into mucous membranes: epistaxis, menorrhagia, gums

Immediate, prolonged bleeding from cuts

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89
Q

Thrombin time tests?

A

Fibrinogen function

Increased w Heparin, DIC

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90
Q

What does bleeding time test?

A

platelet function

increased if decreased pl number/ function, VWD, aspirin, DIC

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91
Q

What does APTT test?

A

intrinsic pathway: 12, 11, 9, 8

+ common: 10, 5, 2, 1

Increased:

haemophilia, VWD, unfractionated heparin, DIC, hepatic failure, antiphospholipid

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92
Q

What does PT test?

A

extrinsic pathway: 7

+ common: 10, 5, 2, 1

increased:

warfarin/ Vit K deficiency, hepatic failure, DIC

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93
Q

Types of thrombophilia?

  • predisposing to thrombosis
A

Inherited:

Factor V Leiden

Prothrombin Gene mutation

Protein C and S deficiency

Antithrombin III deficiency

Acquired:

Antiphospholipid syndrome

OCP

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94
Q

Pathophysiology of Factor V Leiden -> thrombosis?

A

Factor V Leiden is resistant to protein C

Protein C normally deactivates fV and fVIII w protein S and thrombomodulin cofactors

-> increased risk of VTE

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95
Q

indications for thrombophilia screen?

A

arterial thrombosis < 50

venous thrombosis < 40 w no RFs

familial VTE

unexplained recurrent VTE

unusual site: portal, mesenteric

recurrent foetal loss

neonatal

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96
Q

Ix of thrombophilia?

A

FBC, clotting, [fibrinogen]

Factor V Leiden/ APC resistance

Lupus anticoagulant and anti-cardiolipin Abs

Assays for ATIII, Protein C and S

PCR for prothrombin gene mutation

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97
Q

Mx of thrombophilias?

A

tx acute thrombosis as normal

anticoagulate to INR 2-3

consider lifelong warfarin

if recurrence occurs on warfarin -> INR 3-4

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98
Q

prevention of thrombosis in pts w thrombophilia?

A

lifelong anticoagulation may be needed

avoid OCP/HRT

prophylaxis in high risk situations e.g. surgery, pregnancy

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99
Q

ix of Aplastic Anaemia?

A

Bone marrow biopsy: Hypocellular marrow

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100
Q

Mx of aplastic anaemia?

A

supportive; transfusion

immunosuppression: anti-thymocyte globulin

Allogeneic Bone marrow transplant: may be curative

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101
Q

Causes of pancytopenia?

A

congenital: fanconis anaemia

acquired:

idiopathic

BM infiltration

Haemotological: Leukaemia, Lymphoma, Myelofibrosis, Myelodysplasia, Megaloblastic anaemia

infection: HIV

Radiation

Drugs: e.g. cyclophosphamide cytotoxics, chloramphenicol, thiazide, carbimazole, clozapine, phenytoin

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102
Q

what drugs may cause pancytopenia?

A

cytotoxic: cyclophosphamide, methotrexate, azathioprine

chloramphenicol

thiazides

carbimazole

clozapine

phenytoin

103
Q

features of myelodysplastic syndromes?

A

cytopenias

hypercellular BM

defective cells e.g. ringed sideroblasts

30% -> AML

usually in elderly

BM failure: infection, bleeding, bruising, anaemia

splenomegaly

104
Q

Pelger Huet Anomaly?

A

AML/ CML

myelodysplastic syndrome

105
Q

Mx of myelodysplastic syndrome?

A

supportive: transfusions, EPO, G-CSF

Immunosuppression

Allogeneic BMT: may be curative

106
Q

features of essential thrombocythaemia?

A

Thrombosis:

arterial- TIA, strokes, peripheral emboli

venous- DVT, PE, Budd Chiari

bleeding: (abnormal pl fn)
e. g. mucous membranes
erythromelalgia: episodes of burning pain and redness in the feet

107
Q

Ix of essential thromboythaemia?

A

platelets > 600 (often >1000)

BM: increased megakaryocytes

50% JAK2 +ve

108
Q

mx of essential thrombocythaemia?

A

Platelets 400-1000: aspirin alone

Platelets > 1000 or thrombosis: hydroxycarbamide

Anagralide: inhibits pl maturation

109
Q

Essential thrombocythaemia assoc w?

A

progression to AML/ Myelofibrosis

110
Q

Features of polycythaemia vera?

A

hyperviscosity:

headaches, visual disturbances, tinnitus, thrombosis (arterial/ venous)

histamine release:

aquagenic pruritus

erythromelalgia:

sudden severe burning in hands and feet w redness of skin

splenomegaly, hepatomegaly

gout

111
Q

Ix of Polycythaemia vera?

A

99% JAK2 +ve

high RBC, Hb, Hct

high WCC/ Pl

BM: hypercellular w erythroid marrow

low EPO

112
Q

Mx of polycythaemia vera?

A

Aim to keep Hct <0.45 to reduce thrombosis risk

Aspirin 75mg OD

Venesection if young

Hydroxycarbamide if older/ higher risk

113
Q

prognosis of polycythaemia vera?

A

thrombosis and haemorrhage

30% -> myelofibrosis

5% -> AML

114
Q

Differential of polycythaemia?

A

true polycythaemia:

Polycythaemia vera

secondary to hypoxia (smoking, altitude, COPD) or high EPO (renal cyst/ tumour)

Pseudopolycythaemia: low plasma vol

Acute- dehydration, shock, burns

chronic- smoking, diuretics

115
Q

differential for thrombocythaemia?

A

primary: essential thrombocythaemia

secondary to

  • bleeding, infection, chronic inflammation (RA, IBD)

trauma/ surgery, hyposplenism/ splenectomy

116
Q

Features of primary myelofibrosis?

A

elderly

massive hepatosplenomegaly

due to extramedullary haemotopoiesis in liver and spleen

Hypermetabolism: FLAWS

BM Failure: anamia, infection, bleeding

117
Q

Ix of Myelofibrosis?

A

Film: leukoerythroblastic w tear drop poikilocytes

cytopenias

BM: dry tap (neep trephine biopsy)

50% JAK2+ve

118
Q

Mx of primary myelofibrosis?

A

supportive: blood products

Splenectomy

Allogeneic BMT may be curative

119
Q

What Drugs increase risk of VTE?

A

COCP

HRT

Antipsychotics esp olanzapine

120
Q

Risk factors for developing ALL/AML

A

ionising radiation- radiotx

chemotx

benzene

MDS/ myeloproliferative disorders

Downs: signficantly increased risk of AML

121
Q

t(15;17)

excess of abnormal promyelocytes

DIC

PML-RARA fusion protein

A

Acute promyelocytic leukaemia

122
Q

ALL

  • peak age incidence
  • clinical features
A

peak incidence in childhood

BM failure - pancytopenia

hepatosplenomegaly

lymphadenopathy

thymic enlargement

testicular enlargement

bone pain

123
Q

ALL Ix

A

Blood count and film, bone marrow aspirate (>20% blasts)

high WCC (lymphoblasts). low RBC/platelets/ neutrophils

· Immunophenotyping – matters as T (15%) and B-lineage (85%) ALL may be treated differently

· Cytogenetic/molecular genetic analysis - Ph positive need imatinib, treatment must be tailored to the prognosis

· Blood group, LFTs, creatinine, electrolytes, calcium, phosphate, uric acid, coagulation screen

CXR + CT: mediastinal + abdominal LNs

LP: CNS involvement

124
Q

Mx of ALL

A

· Specific therapy – systemic or CNS-directed chemotherapy

· Supportive care – blood products, abx, general medical care, prophylaxis for Pneumocystis jirovecii infection (co trimoxazole)

· Hyperuricaemia: hydration, urine alkalinization and allopurinol or rasburicase

· Hyperphosphataemia; Al(OH)3, calcium

· Hyperkalemia: fluids, diuretics

· Extreme leukocytosis (WBC > 200 × 109/l): leukapheresis, sometimes haemodialysis

BMT: best option for younger adults

125
Q

AML

  • typical features
  • more specific features
  • Blood film features
A

BM failure- effects of pancytopenia

Splenomegaly, hepatomegaly

Gum infiltration if monoblasts/ monocytic

Skin infiltration/ CNS involvement + hypoK

Blood film: circulating blasts (>20%) with granules. auer rods.

if unclear- immunophenotyping

126
Q

AML

  • age of incidence
  • mutations seen
  • pathognomonic features
A

bimodal incidence: peak in under 2s and in 65-70yos

many mutations assoc with AML

t(5;8), inv(16), t(8;21), trisomy 8, trisomy 21

>20% myeloblasts on BM + Auer Rods

127
Q

treatment of AML

A

Supportive care:

blood products, abx, FFP/Cryoprecipitate if DIC, allopurinol, fluid and electrolytes to prevent tumour lysis syndrome

Chemotherapy

Consider Allo BMT in young / autologous HSCs

Specific for acute promyelocytic leukaemia: ATRA

128
Q

Ix in CML?

A

PCR for BCR-ABL (philadelphia chr) fusion gene

Ph Chr +ve in 80%

t(9;22)

high WCC, FBC: low Pl, Hb

Blood film: neutrophils, myelocytes, basophilia

Can monitor disease and treatment response with FBC, BCR-ABL:ABL ratio

129
Q

Treatment of CML

A

Imatinib*

a BCR-ABL tyrosine kinase inhibitor

or

if resistant -2nd line: Dasatinib/ Nilotinib

young patients- possible allogeneic SCT

130
Q

how to monitor disease and response to therapy in CML

A

FBC and WCC count

cytogenetics and detection of Ph chr - reduction in % of Ph Chr expressed

RT-PCR of BCR-ABL fusion transcript. can calculate BCR-ABL: ABL ratio

successful therapy means this will drop

e.g. complete response: 0% Ph +ve

partial response 1-35% Ph +ve

BCR-ABL transcripts reduce from 100% to the best - 0.001%

131
Q

chronic phase vs accelerated phase vs blast phase of CML?

no of blasts in BM/blood

A

chronic (80% of pts): <5% blasts

indolent

Accelerated: 10%-19% blasts

increasing manifestations e.g. splenomegaly

Blast crisis: >20% blasts

median survival 3-6 months

resembles acute leukaemia

132
Q

what translocation is assoc w CML?

A

t (9;22)

Philadelphia chr

  • > formation of BCR-ABL fusion gene
  • > constitutive tyrosine kinase activity
133
Q

Blood film- tear drop poikilocytes (dacrocytes) and leukoerythroblasts

Dry tap on BM biopsy

extramedullary haemopoiesis - massive splenomegaly (budd chiari syndrome may present), hepatomegaly

pancytopenia: anaemia, thrombocytopenia, neutropenia

hypermetabolic state: weight loss, fatigue, night sweats, hyperuricaemia

A

myelofibrosis

134
Q

t(9;22)

A

produces philadelphia chrosome (BCR-ABL gene)

seen in CML

expresses a fusion oncogene with tyrosine kinase activity

can be detected using FISH

135
Q

features of CML?

A

Systemic: FLAWS

massive HSM -> abdo discomfort

Bruising/ bleeding

Gout

Hyperviscosity

136
Q

treatment of CLL

A

many patients undergo watchful waiting if asymptomatic with slowly progressive disease

1st line: alemtuzumab anti-CD52 (depletes lymphocytes)

Ibrutinib - Brutons Tyrosine kinase inhibitor

supportive: abx, vaccination, e.g. aciclovir, PCP prophylaxis, IVIG, pneumovax

young patients: allogeneic SCT

Autoimmune phenomena: 1st line steroids. 2nd line Rituximab

Radiotx - debulking LNs/ splenomegaly

Chemotx: fludarabine, cyclophosphamide, Rituximab

137
Q

what may undergo richter transformation to DLBCL?

A

CLL

hairy cell leukaemia

138
Q

CLL prophylaxis and treatment of infections

A

Aciclovir

PCP prophylaxis for those receiving fludarabine or alemtuzumab

IVIG for those w hypogammaglobulinaemia and recurrent bacterial infections

Immunisation against pneumococcus and seasonal flu

139
Q

CLL autoimmune phenomena e.g. AIHA

mx?

A

1st line steroids

2nd line rituximab

140
Q

Features of CLL?

A

often asymptomatic incidental finding

symmetrical painless Lymphadenopathy

HSM

Anaemia

B symptoms: FLAWS

141
Q

complications of CLL?

A

AIHA, ITP

Infection: bacterial, zoster

Marrow failure/ infiltration

142
Q

Smear cells seen in?

A

CLL

(most often assoc w abnormally fragile lymphocytes)

143
Q

ix of CLL?

A

high WCC, lymphocytes

Smear cells

low serum Ig

+ve DAT: if AIHA

Rai or Binet staging

Diagnosis via immunophenotyping to distinguish from NHL

144
Q

Hodgkins Lymphoma

  • how does it present
  • associated with which virus
  • affects which age groups
A

presents as asymmetrical painless lymphadenopathy.

most often single node and spreads contiguously to adjacent LNs

+/- obstructive/mass effect symptoms -> SVC/ bronchial obstruction

B symptoms may be present: fever>38, night sweats, weight loss (>10% over 6mo)

pain in affected nodes after alcohol

assoc w EBV

bimodal age incidence - 20-30 and >60

itch, Pel Ebstein fever, hepato/splenomegaly

145
Q

Reed sternberg cell

binucleate/ owl eyed cell on a background of lymphocytes and reactive cells

LN/ BM biopsy - cells stain with CD15 and CD30

A

Hodgkins Lymphoma

146
Q

Staging of Hodgkins Lymphoma

A

I; one group of nodes

· II; >1 group of nodes same side of the diaphragm

· III; nodes above and below the diaphragm

· IV; extra nodal spread (other organs)

· Suffix A if none of below, B if any of below

o Fever, night sweats

o Unexplained Weight loss >10% in 6 months

**RMB that spleen is considered a LN

147
Q

Pel Ebstein fever assoc w?

A

fevers which cyclically increase then decrease over an average period of one or two weeks.

Hodgkins lymphoma

148
Q

Ix of Hodgkins Lymphoma

A

FBC, film, ESR, LFT, LDH, Ca

  • high ESR or low Hb = worse prognosis

LN excision biopsy or FNA

  • Reed sternberg cells (Owls eyes nuclei)

Staging: CT/MRI Chest, Abdo, pelvis

BM biopsy if B symptoms of stage 3/4 disease

149
Q

treatment of Hodgkins Lymphoma

Chemotherapy

Radiotherapy

A

Chemotherapy:

ABVD: adriamycin, bleomycin, vinblastine, dacarbazine

2-4 cycles in stage 1/2, 6-8 in stage 3

given at 4 wkly intervals

prognosis excellant esp in the young. preserves fertility but can cause long term pulmonary fibrosis, cardiomyopathy

Radiotherapy:
HL is highly responsive to radiotx

usually given at the end of chemo to small area of diseased nodes

risk of collateral damage: BrCa, Leukaemia/ MDS, lung/skin Ca

150
Q

what are the chemotherapy drugs used in hodgkins lymphoma tx?

A

ABVD

Adriamycin, Bleomycin, Vinblastine, Dacarbazine/DTIC

cycles given at 4 wkly intervals

SE: Adriamycin- cardiomyopathy

Bleomycin- Pulm fibrosis

151
Q

Tx of Hogdkins Lymphoma in relapse patients

A

Autologous SCT

+ Intensive chemo

152
Q
A
153
Q

Of the common B cell non-hodgkin lymphomas,

which are high grade?

which are low grade?

A

High Grade:

V aggressive: Burkitt’s
Aggressive: Diffuse Large B cell, Mantle Cell

Low Grade:

Indolent-

Follicular, small lymphocytic/ CLL, marginal zone lymphoma, mantle zone lymphoma

154
Q

affects middle aged/ elderly

*Large T cell lymphocytes

Aggressive

Often with associated reactive cell population esp eosinophils

Lymphadenopathy and extranodal sites

A

Peripheral T Cell lymphoma

155
Q

what lymphoma is associated with longstanding coeliac disease?

A

EATL

Enteropathy Associated T cell lymphoma

156
Q

what lymphoma is associated with mycosis fungoides?

A

Cutaneous T cell lymphoma

157
Q

Affects mainly children/ young adults

Histology shows large ‘epitheliod’ lymphocytes

t(2;5) and Alk-1 protein expression

Aggressive.

A

Anaplastic large cell lymphoma

158
Q

What immunodeficiency is associated with burkitts lymphoma?

A

HIV / post transplant patients

159
Q

MALT (mucosal associated lymphoid tissue) lymphoma / Marginal zone NHL

  • what sites are normally affected?
  • treatment?
A

mainly at extranodal sites. e.g. gut, lung, spleen, thyroid, parotid

arise in response to chronic antigen stimulation

treatment involves removing the antigenic stimulation e.g. H pylori eradication

160
Q

what is the H pylori triple therapy for eradication?

A

omeprazole, clarithromycin and amoxicillin

161
Q

what causes constant antigenic stimulation, predisposing the patient to small bowel T cell lymphoma?

A

Coeliac disease

-> enteropathy associated T-cell Non-Hodgkins Lymphoma (EATL)

162
Q

a lymphoma caused by HTLV-1 infection

HTLV-1 infects T cells

common in the Carribean and Japanese population

what lymphoma is associated?

A

Adult T cell leukaemia/ lymphoma

*nuclei of ATL cells have characteristic cloverleaf appearance

163
Q

what virus is associated with Adult T cell leukaemia/ lymphoma?

A

HTLV-1

164
Q

Known risk factors of lymphoma:

A

constant antigenic stimulation (will increase risk of DNA replication error)

infection (direct viral infection of lymphocytes e.g. EBV)

loss of T cell function (e.g. HIV)

165
Q

Gastric MALToma

what causes the chronic antigenic stimulation that may lead to gastric MALToma

A

Helicobacter pylori

-> marginal zone non-hodgkins lymphoma of the stomach

166
Q

Lymphoma

  • what is it?
  • where may it be found?
A

Lymphomas are neoplastic tumours of lymphoid cells.

usually found in lymph nodes, bone marrow and or blood (via the lymphatic system)

and in lymphoid organs such as the spleen, MALT

167
Q

why are lymphocytes particularly at risk of neoplasm/ malignancy?

A

rapid cell proliferation in immune response risks DNA replication error

dependent on apoptosis (in the germinal centre) -> apoptosis may get switched off in the germinal centre, there may be acquire DNA mutations in the pro apoptotic genes

DNA molecules are cut and rejoined and undergo deliberate point mutation to generate immunoglobulin and T cell receptor diversity -> potential for recombination errors and new point mutations

168
Q

what causes constant antigenic stimulation that may result in marginal zone lymphoma of the thyroid gland?

A

Hashimoto’s Disease

169
Q

What causes chronic antigenic stimulation that may predispose to developing parotid lymphoma?

A

Sjogren’s syndrome

-> marginal zone non-hodgkins lymphoma of the parotids

170
Q

Spread of neoplasia in Hodgkins vs Non Hodgkins Lymphoma?

A

Hodgkins: spreads contiguously to adjacent LNs

Non Hodgkins: spreads discontinuously

171
Q

Treatment for DLBCL

A

6-8 cycles of Rituximab-CHOP

aim of therapy is curative

if relapse-> 2nd line tx: autologous SCT

-> same regimen for other high grade non hodgkin lymphomas

172
Q

what drugs does R-CHOP consist of?

A

Rituximab (anti CD20)

Cyclophosphamide

Adriamycin

Oncovin aka Vincristine

Prednisolone

173
Q

features of multiple myeloma

A

CRAB

hypercalcaemia - stones, bones, groans, moans

renal impairment

anaemia

bone pain, osteolytic lesions, fractures, osteoporosis

174
Q

Ix of Multiple myeloma

A

serum Ca, high ESR, high Urea/Cr

*NORMAL ALP

MRI whole body

blood film shows Rouleaux formation

Urine electrophoresis: bence-jones protein

serum protein electrophoresis: dense narrow band

BM aspirate: >10% plasma cells

Xray: Punched out lytic lesions, Pepper pot skull, vertebral collapse, fractures

175
Q

Diagnosis of Multiple Myeloma?

A

1 major and 1 minor criteria or 3 minor criteria + signs or symptoms

Major criteria

Plasmacytoma (as demonstrated on evaluation of biopsy specimen)

30% plasma cells in a bone marrow sample

Elevated levels of M protein in the blood or urine

Minor criteria

10% to 30% plasma cells in a bone marrow sample.

Minor elevations in the level of M protein in the blood or urine.

Osteolytic lesions (as demonstrated on imaging studies).

Low levels of antibodies (not produced by the cancer cells) in the blood.

176
Q

what is the first investigation you want to do when you suspect Multiple myeloma?

A

serum protein electrophoresis

  • paraprotein detected
177
Q

myeloma bone disease: possible complications

A

80-90% have lytic lesions

pathological fractures

spinal cord compression leading to paralysis

hypercalcaemia

bone pain -> affecting QOL, independence, mobility

too many osteoclasts (from increased IL6) and not enough active osteoblasts

178
Q

what investigations for myeloma bone disease

A

X ray - for lytic lesions

MRI- High sensitivity for marrow infiltration, response monitoring possible, expensive & limited availability

CT – full body CT is the standard used in Imperial to diagnose as it detects very small lesions (high sensitivity). Good for radiotherapy planning but higher radiation dose

PET - detects active disease

179
Q

why does multiple myeloma cause renal failure?

A

free light chains cause injury to kidneys - active inflammatory mediators in the proximal tubule epithelium

-> leads to amyloidosis (AL chains)

cast nephropathy

180
Q

treatment of multiple myeloma

A

supportive for CRAB symptoms including bisphosphonates

Auto-SCT - curative, best for younger patients

steroids

Classical cytostatic drugs (relatively low dose) eg. melphalan

Proteasome inhibitors – prevent misfolded proteins from being degraded, which accumulates in myeloma cells (esp effective as myeloma produces a lot of proteins)

e.g. bortezomib

IMIDs – thalidomide, lenalidomide, pomalidomide (also used in MDS). Reduces cell turnover by affecting transcription factor relevant for plasma cells

181
Q

mx of AKI due to Myeloma?

A

rehydration +/- dialysis

182
Q

Mx of hypeviscosity due to myeloma?

A

Plasmapheresis (remove light chains)

183
Q

Poor prognostic indicators of multiple myeloma?

A

high B2-microglobulin

low albumin

184
Q

mx of cord compression secondary to myeloma?

A

Radiotx

+ dexamethasone

185
Q

Mx of high calcium in myeloma?

A

hydration, frusemide, bisphosphonates

186
Q

mx of infections secondary to multiple myeloma?

A

broad spec abx +/- IVIg if recurrent

187
Q

Diagnosis of Multiple Myeloma?

A

Clonal BM plasma cells ≥ 10%

presence of serum and or urinary monoclonal protein

end organ damage: CRAB (1 or more)

Ca high (>2.6 mM)

Renal insufficiency

Anaemia (<10g/dL)

Bone lesions

188
Q

what amyloid is assoc w Multiple Myeloma?

A

AL Amyloid

189
Q

Echo findings of AL amyloidosis?

A

Sparkling appearance on echo

190
Q

Diagnosis of Amyloidosis?

A

Biopsy of affected tissue ->

Apple green birefringence w Congo Red stain under polarized light

191
Q

indications for splenectomy?

A

trauma

rupture e.g. EBV

AIHA

ITP

Hereditary spherocytosis

hypersplenism

192
Q

complications of splenectomy?

A

redistributive thrombocytosis -> early VTE

Gastric dilatation (ileus)

left lower lobe collapse: v common

increased susceptibility to infections:

encapsulated bacteria meningococcus, pneumo, Hib

193
Q

Blood film after splenectomy?

A

Howell-Jolly bodies

Pappenheimer bodies

Target cells

194
Q

Mx of Splenectomy/ hyposplenism?

A

Immunisation: pneumovax, HiB, Men C, yrly flu

Daily Abx: pen V or erythromycin

Warning: Alert card and/or bracelet

195
Q

Causes of hyposplenism?

A

post splenectomy

SCD

coeliac

IBD

196
Q

Haematological causes of Splenomegaly?

A

Haemolysis: HS

Myeloproliferative disease: CML, PV, MF

Lymphoproliferative disease: lymphoma, leukaemia

197
Q

infective causes of splenomegaly?

A

malaria, leishmaniasis, hydatid disease

EBV, CMV

TB, infectious endocarditis

198
Q

Connective tissue causes of splenomegaly?

A

rheumatoid arthritis

SLE

Sjogrens

199
Q

Causes of splenomegaly?

A

Haematological: haemolysis, myeloproliferative, lymphoproliferative

Infection: EBV, malaria, leishmaniasis

Portal HTN: cirrhosis, Budd-Chiari

Connective tissue: RA, SLE, sjogrens

Other: Sarcoid, Amyloid,

200
Q

Causes of massive splenomegaly (>20cm)?

A

CML

Myelofibrosis

Malaria

leishmaniasis

Gauchers (AR, glucocerebrosidase deficiency)

201
Q

Features of the spleen?

A

LUQ

lies anterior to ribs 9-11

Dull to percussion

enlarges to RIF

moves inferiorly on resp

cant get above it

medial notch

202
Q

Function of the spleen?

A

phagocytosis of the old RBCs, WBCs, opsonised bugs

antibody production

haematopoiesis

sequestration of formed blood elements

203
Q

Normal ESR?

A

20 mm/hr

204
Q

what causes raised ESR?

A

plasma factors:

  • increased fibrinogen: inflammation
  • increased globulins: e.g. myeloma

red cell factors:

anaemia

205
Q

differentials for ESR >100?

A

Multiple myeloma

SLE

GCA

AAA

Ca prostate

206
Q

differential for basophilia?

A

parasitic infection

IgE-mediated hypersensitivity: urticarial, asthma

CML

207
Q

Differential for eosinophilia?

A

parasitic infection

drug reactions

allergies: asthma, atopy, churg-strauss

Skin disease, psoriasis, eczema, pemphigus

208
Q

differential of high monocyte count?

A

Chronic infection: TB, Brucella, Typhoid

AML

209
Q

Differential of low lymphoctes?

A

Drugs: steroids, chemo

HIV

210
Q

differential for lymphocytosis?

A

Leukaemia, lymphoma esp CLL

Viral infections eg. CMV, EBV

Chronic infections TB, Brucella, hepatitis, toxo

211
Q

Differential for neutrophilia?

A

bacterial infection: left shift, toxic granulation, vacuolation

stress: trauma, burns, surgery, haemorrhage

steroids

inflammation: MI, polyarteritis nodosa

Myeloproliferative disorders e.g. CML

212
Q

differential of neutropenia?

A

viral infection

Severe sepsis

Drugs: chemo, cytotoxics, carbimazole, sulphonamides

Feltys

213
Q

tartrate resistant acid phosphatase +ve

A

Hairy cell leukaemia

214
Q

Sudan Black B, MPO +ve

A

AML

215
Q

Blood film showing target cells?

A

hyposplenism

thalassaemia

liver disease

216
Q

Heinz bodies?

A

G6Pd deficiency

217
Q

Acanthocytes?

A

LAB

Liver disease

Abetalipoproteinaemia

218
Q

Basophilic stippling

A

Lead poisoning

thalassaemia

219
Q

Burr cells?

A

Uraemia

220
Q

Howell Jolly bodies?

A

hyposplenism

221
Q

leukoerythoblastic film?

A

BM infiltration

222
Q

Pappenheimer bodies?

A

hyposplenism

223
Q

Pencil cells?

A

IDA

224
Q

What is the most common inherited bleeding disorder?

A

Von Willebrand’s disease

225
Q

Types of Von Willebrand’s disease?

A

type 1: partial reduction in vWF (80% of patients)

type 2*: abnormal form of vWF

type 3**: total lack of vWF (autosomal recessive)

226
Q

Investigation of Von Willebrands disease?

A

prolonged bleeding time

APTT may be prolonged

factor VIII levels may be moderately reduced

defective platelet aggregation with ristocetin

227
Q

Tx for post-thrombotic syndrome?

A

Compression stockings

228
Q

painful, heavy calves

pruritus

swelling

varicose veins

venous ulceration

following previous DVT

A

Post-thrombotic syndrome

229
Q

What is cryoprecipitate used for?

A

contains concentrated Factor VIII:C, von Willebrand factor, fibrinogen, Factor XIII and fibronectin

Clinically it is most commonly used to replace fibrinogen

e.g. DIC, liver failure

230
Q

universal donor of FFP?

A

AB -ve blood

lacks anti-A or anti-B antibodies

231
Q

What is Prothrombin complex concentrate used for?

A

used for the emergency reversal of anticoagulation in patients with either severe bleeding or a head injury with suspected intracerebral haemorrhage

232
Q

Critical bleeding e.g. CNS

+ Pl < 100

Mx

A

immediate platelet transfusion

normally if other bleeding e.g haematemesis, melaena -> only transfuse if Pl < 30

233
Q

An 88-year-old male has been formally diagnosed with anorectal cancer. Imaging confirms the cancer is localised to a region inferior to the pectinate line.

If untreated which group of lymph nodes will this cancer likely metastasize to?

A

Below the pectinate line cancer spreads to the superficial inguinal lymph nodes.

234
Q

stomach cancer drains to which LN first?

A

Coeliac lymph nodes

235
Q

Ca in the anal canal above pectinate line drains into which LNs?

A

Internal iliac lymph nodes

236
Q

Ca of glans penis drains into which LNs?

A

Deep inguinal lymph nodes

237
Q

Ca of testes/ ovaries/ kidney/ adrenal glands drain into which LNs?

A

Para-aortic lymph nodes

238
Q

Ca of Lateral breast
Upper limb drain into which LNs?

A

Axillary lymph nodes

239
Q

Ca in

Duodenum
Jejunum drains into which LNs?

A

Superior mesenteric lymph nodes

240
Q

ca in Descending colon
Sigmoid colon
Upper part of rectum will drain into which LNs?

A

Inferior mesenteric lymph nodes

241
Q

diagnosis of Hodgkins lymphoma?

A

This is made by excision of a complete lymph node that is then submitted for detailed histological evaluation.

242
Q

poor prognostic markers of Hodgkins lymphoma?

A

Lymphocyte depleted Hodgkins lymphoma, advancing age, male sex and stage IV disease

243
Q

Normal blood transfusion threshold?

Transfusion threshold in pts wihth ACS?

A

Normal: 70g/L

ACS: 80g/L

244
Q

Electrolyte abnormalities in Tumour Lysis syndrome?

A

High K and PO4

and low Ca

+ high uric acid

245
Q

Which one of the types of Hodgkin’s lymphoma carries the best prognosis?

A

Lymphocyte predominant

246
Q

What is the most common type of Hodgkins Lymphoma?

A

Nodular sclerosing

247
Q

What drug class is known to precipitate renal failure in patients with multiple myeloma (MM)?

A

NSAIDs

248
Q

t(11;14)
deregulation of the cyclin D1 (BCL-1) gene

A

Mantle cell lymphoma

249
Q

t(15;17)

fusion of PML and RAR-alpha genes

A

seen in acute promyelocytic leukaemia (M3)

250
Q

t(8;14)

MYC oncogene is translocated to an immunoglobulin gene

A

Burkitt’s lymphoma

251
Q

most common type of Hodgkin’s lymphoma?

A

nodular sclerosing

252
Q

platelet transfusions should not be performed in which conditions?

A

Chronic bone marrow failure

Autoimmune thrombocytopenia

Heparin-induced thrombocytopenia, or

Thrombotic thrombocytopenic purpura.

253
Q

reversal agent for dabigatran?

A

Idarucizumab

  • humanised monoclonal antibody that directly inhibits dabigatran.
254
Q

How to investigate patients w unprovoked clots?

A

Investigate for underlying malignancy:

  • physical exam guided by full hx
  • CXR
  • Blood tests and urinalysis

Consider CT abdo-pelvis in all >40 w unprovoked DVT/ PE

Thrombophilia screening:

consider test for antiphospholipid abs

consider testing for hereditary thrombophilia in pts who have first degree relative who have had DVT/PE

Medicine (16 decks)

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