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RACP Written Exam Cram Series > Haem Cram > Flashcards

Haem Cram Flashcards

1
Q

Microcytosis

A 
Thalassemia
Anaemia of chronic disease
Iron deficiency
Lead poisoning
Sideroblastic anaemia
Hereditary spherocytosis
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2
Q

Normocytic

A

Blood loss
Mixed nutritional def
Chronic disease
Transient erythroblastopenia of childhood

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3
Q

Macrocytosis

A 
B12/folate def
Brisk haemolysis
Myelodysplasic syndrome
Fanconi
Diamond-Blackfan
Osteoporosis
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4
Q

Spherocytes

A

Hereditary spherocytosis
Haemolytic anaemia
Liver disease
Post splenectomy

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5
Q

Acanthocytes/Spur cells

A

Liver disease
Storage diseases
Abetalipoproteinaemia

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6
Q

Target cells

A

Liver disease

Thalassemia

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7
Q

Bizarre cells

A

Burns
Vit E deficiency in neonates
Hereditary poikilocytosis

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8
Q

Cigar/Pencil cells

A

Iron def

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9
Q

Bite cells/Heinz bodies

A

G6PD

Haemolytic anaemia

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10
Q

Serum iron utility?

A

Minimal, biphasic and unreliable except to monitor compliance w therapy

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11
Q

Iron studies findings in iron def anaemia?

A

Low serum iron
High transferrin but low transferrin saturation
Low ferritin

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12
Q

How to investigate for iron def anaemia in pt with inflammation?

A

Soluble transferrin receptors

Transferrin receptor:ferritin ratio >2 suggestove of IDA

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13
Q

Stages of embryonic haematopoiesis?

A

Yolk sac up to 8 weeks gestation
Liver 6 weeks onwards
Spleen/thymus 9 weeks onwards
Bone marrow from 28 weeks

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14
Q

Increased HbA2 on haemoglobin electrophoresis?

A

B-Thalessemia Minor

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15
Q

Increased HbA2 and HbF on haemoglobin electrophoresis?

A

B-Thalessemia Intermedia

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16
Q

> 90% HbF/Hb2A on haemoglobin electrophoresis with NO HbA?

A

B-Thalessemia Major

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17
Q

Intrinsic pathway factors and test?

A

VIII, IX, XI, XII

APTT

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18
Q

Extrinsic pathway factors and test?

A

VII

PT

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19
Q

Vitamin K dependant clotting factors?

A

II, VII, IX, X

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20
Q

Causes of prolonged APTT?

A

Heparin
Lupus anticoagulant
Haemophilia A/B

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21
Q

Causes of prolonged PT?

A

Factor VII deficiency/inhibitor
Vit K def
Warfarin

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22
Q

Correction with mixing test?

A

Full correction = clotting factor deficiency

No correction = inhibitor present (EG lupus anticoagulant)

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23
Q

Nucleated red blood cells?

A

Always abnormal outside neonatal period

  • high physiologic stress (hypoxia)
  • infiltrative/malignant
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24
Q

When is macrocytosis within limits of normal?

A

Neonates
T21
Antiepileptic medication use

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25
Q

Anaemia
Thrombocytopenia
Hypersegmented neutrophils

A

Folate/B12 deficiency

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26
Q

Infant with microcytic anaemia and absent RBC precursors in marrow
Increased RBC adenosine deaminase
Craniofacial/clef palate/thumb/upper limb deformity

A

Diamond-Blackfan anaemia

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27
Q

Diamond-Blackfan pathogenesis?

A

Inherited defect of ribosomal protein RPS gene

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28
Q

Difference between Diamond-Blackfan and Transient Erythroblastopenia of childhood?

A

TEC usually >1yr old, have antecedent viral illness and not associated with congenital abnormalities

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29
Q

Cafe au lait
Short stature
Anaemia
Horseshoe kidney

A

Fanconi Anaemia

30
Q

Dystrophic nails
Oral leukoplakia
Reticular pigmentation
Pancyotpenia

A

Ectodermal dysplasia

31
Q

Eosin-5-maleimide binding

Osmotic fragility

A

Hereditary spherocytosis

32
Q

Thalassemia physical findings?

A 
Skeletal abnormalities
 - frontal bossing
 - hair on end radiographs
 - short limbs
Hepatosplenomegaly
33
Q

Complications of Beta-Thalassemia Major?

A

Jaundice
Gallstones
Hepatosplenomegaly req splenectomy
Skeletal changes
Iron overload - deposits liver (1yr), endocrine (3yrs), cardiac (10yrs)
Infections - yersinia, salmonella, listeria

34
Q

Treatment/prevention of iron overload?

A

Chelation therapy -Desferrioxamine

Commence 1 year after transfusion therapy begins

35
Q

SEs of desferrioxamine?

A

Risk increases as iron stores decrease
Ototoxicity
retinal changes
Bone dysplasia - truncal shortening

Desferrioxamine makes iron more available for Yersinia infections!

36
Q

Most common genetic cause of alpha thalassemia?

A

Deletion mutations

Non-deletional mutations: “Constant Spring”

37
Q

Hb Barts?

A

Newborns with alpha thal mutations

38
Q

Management of alpha thal?

A

Folate supposed
Splenectomy
Transfusion
Avoid oxidative medications

39
Q

Genetic cause of sickle cell anaemia?

A

Autosomal recessive

Single base pair change - GAG to GTG (valine instead of glutamine at 6th codon)

40
Q

Treatment of sickle cell?

A 
Hydroxyurea - reduces interaction between RBC & endothelium, reduces painful episodes
Folic acid
Transfusion
Fe chelation
Prophylactic penicillin until age 5
Immunisations!
41
Q

Complications of sickle cell?

A 
Iron overload, iron deposition in organs
Splenic sequestration crisis
Stroke
Retinopathy
Renal disease including night time bed wetting
Cardiac deposition/Pulm HTN
Gallstones
42
Q 
Child with sickle cell 
Fever
Dyspnoea
Chest pain
Pulm infiltrates
A

Acute chest syndrome
50% of sickle cell pts
Blood transfusion as treatment
Hydroxyurea as prevention

43
Q

Acute, severe pain of femoral/humeral head or mandible in pt with sickle cell?

A

Avascular necrosis

44
Q

Infections more common in sickle cell and what abs not to use?

A

Salmonella, neisseria, strep pneumo

Do not use ceftriaxone - risk of haemolysis

45
Q

Blood product with highest risk of bacterial contamination?

A

Platelets

Stored at room temp for up to 5 days

46
Q

FFP and indications?

A

All clotting factors

Warfarin, liver disease, DIC, massive transfusion, deficiency of 2, 5, 10, 11

47
Q

Cryoprecipitate and indications?

A

Fibrinogen, vWF, VIII and IX

Dysfibrinogenemia, DIC, vWF (last line)

48
Q

Prothrombinex and indictions?

A

2, 9, 10, 11

Warfarin reversal

49
Q

Extrinsic pathway?

A

Activated by tissue factor (damage to tissue wall)
Tissue factor -> factor VII
TF & VIIa = X

50
Q

Intrinsic pathway?

A

Triggered by contact activation platelet receptor 2b3a
Activated Factor XII -> Factor XI
XI -> IX
IX + VIII + phospholipids = Factor X

51
Q

Common pathway?

A

Activation of Factor X
Xa + Ca + phospholipid + V = prothrombinase -> thrombin
Thrombin cleaves fibrinogen to fibrin
Factor 13 stabilises the clot

52
Q

DIC investigation findings?

A 
Thrombocytopenia, anaemia
Fragmented, burr, helmet cells on film
Reduced coag factors
APTT and PT/INR both prolonged
Increased D-Dimer
53
Q

DIC mangement?

A

Treat trigger - sepsis, acidosis, hypoxia
Platelet fusion (thrombocytopenia)
Cryoprecipate (hypofibrinogenemia)
FFP (coag factors/inhibitors)

54
Q

Liver disease vs DIC?

A

Factor VIII levels in liver disease normal or high, in DIC LOW
D-Dimer in liver disease normal, in DIC dramatically elevated

55
Q

Haemophilia A, B and C?

A 
A = Factor VIII deficiency
B = Factor IX deficiency
C = Factor XI deficiency
56
Q

Genetic cause of Haemophilia A/B?

A

X-linked

Genes for both VIII and IX on the long arm of the X chromosome

57
Q

Common presentations of Haemophilia?

A

Intracranial haemorrhage as newborn
Haemarthrosis
Psoas haemorrhage

58
Q

Management of Haemophilia?

A 
Avoid trauma
Avoid NSAIDs/aspirin
Desmopressin if Haemophilia A
Recombinant Factor VIII & IX
Rituximab if inhibitor present
Activated Factor VIIa if inhibitor present
59
Q

Chronic complications of Haemophilia?

A

Arthropathy - inflammatory response can result in target joint
Development of inhibitor (IgG antibodies)
Blood transfusion borne infection
Obesity

60
Q

Ashkenazi Jewish child with bleeding post dental surgery and prolonged PTT?

A

Haemophilia C

61
Q

Treatment of von Willebrands?

A

Desmopressin - increases VWF release from endothelial cells
Tranexamic acid
PLASMA DERIVED Factor VIII

62
Q

Risk factors for early onset haemorrhage disease of the newborn?

A

Maternal phenobarbital, phenytoin, warfarin, rifampicin

63
Q

Risk factors for classic (2-7 days) haemorrhage disease of the newborn?

A

Vit K deficiency

Breastfeeding

64
Q

Risk factors for late onset (1-6mths) haemorrhage disease of the newborn?

A

Cholestasis (vit K malabsorption)
Biliary atresia
Can be idiopathic in Asian breastfed infants

65
Q

Thrombocytopenia
Normal sized platelets
Absent radius

A

Thrombocytopenia with absent radii

66
Q

Mucosal bleeding
Thromobocytopenia
Large sized platelets

A

Bernard-Soulier

67
Q

Bleeding
Normal platelets
Platelet aggregation normal with ristocetin but low with other agents

A

Glannzman Thrombasthenia

68
Q

Sudden onset petechiae and mucosal bleeding
Preceding viral illness
Generally well

A

Idiopathic Thrombocytopenic Purpura

69
Q

Risk of ICH in ITP?

A

<1%

70
Q

Treatment of ITP?

A 
Conservative/expectant
Pred if active mucosal bleed
IVIG if more rapid treatment req
IV anti-D if Rh pos patient
Splenectomy if severe chronic or life threatening haemorrhage
71
Q 
Fever
Microangiopathic haemolytic anaemia
Thrombocytopenia
Abnormal renal function
CNS changes
A

Thrombotic Thrombocytopenic Purpura

RACP Written Exam Cram Series (19 decks)

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