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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
Anaemia Thrombocytopenia Hypersegmented neutrophils
Folate/B12 deficiency
26
Infant with microcytic anaemia and absent RBC precursors in marrow Increased RBC adenosine deaminase Craniofacial/clef palate/thumb/upper limb deformity
Diamond-Blackfan anaemia
27
Diamond-Blackfan pathogenesis?
Inherited defect of ribosomal protein RPS gene
28
Difference between Diamond-Blackfan and Transient Erythroblastopenia of childhood?
TEC usually >1yr old, have antecedent viral illness and not associated with congenital abnormalities
29
Cafe au lait Short stature Anaemia Horseshoe kidney
Fanconi Anaemia
30
Dystrophic nails Oral leukoplakia Reticular pigmentation Pancyotpenia
Ectodermal dysplasia
31
Eosin-5-maleimide binding | Osmotic fragility
Hereditary spherocytosis
32
Thalassemia physical findings?
``` Skeletal abnormalities - frontal bossing - hair on end radiographs - short limbs Hepatosplenomegaly ```
33
Complications of Beta-Thalassemia Major?
Jaundice Gallstones Hepatosplenomegaly req splenectomy Skeletal changes Iron overload - deposits liver (1yr), endocrine (3yrs), cardiac (10yrs) Infections - yersinia, salmonella, listeria
34
Treatment/prevention of iron overload?
Chelation therapy -Desferrioxamine | Commence 1 year after transfusion therapy begins
35
SEs of desferrioxamine?
Risk increases as iron stores decrease Ototoxicity retinal changes Bone dysplasia - truncal shortening Desferrioxamine makes iron more available for Yersinia infections!
36
Most common genetic cause of alpha thalassemia?
Deletion mutations | Non-deletional mutations: "Constant Spring"
37
Hb Barts?
Newborns with alpha thal mutations
38
Management of alpha thal?
Folate supposed Splenectomy Transfusion Avoid oxidative medications
39
Genetic cause of sickle cell anaemia?
Autosomal recessive | Single base pair change - GAG to GTG (valine instead of glutamine at 6th codon)
40
Treatment of sickle cell?
``` Hydroxyurea - reduces interaction between RBC & endothelium, reduces painful episodes Folic acid Transfusion Fe chelation Prophylactic penicillin until age 5 Immunisations! ```
41
Complications of sickle cell?
``` Iron overload, iron deposition in organs Splenic sequestration crisis Stroke Retinopathy Renal disease including night time bed wetting Cardiac deposition/Pulm HTN Gallstones ```
42
``` Child with sickle cell Fever Dyspnoea Chest pain Pulm infiltrates ```
Acute chest syndrome 50% of sickle cell pts Blood transfusion as treatment Hydroxyurea as prevention
43
Acute, severe pain of femoral/humeral head or mandible in pt with sickle cell?
Avascular necrosis
44
Infections more common in sickle cell and what abs not to use?
Salmonella, neisseria, strep pneumo | Do not use ceftriaxone - risk of haemolysis
45
Blood product with highest risk of bacterial contamination?
Platelets | Stored at room temp for up to 5 days
46
FFP and indications?
All clotting factors | Warfarin, liver disease, DIC, massive transfusion, deficiency of 2, 5, 10, 11
47
Cryoprecipitate and indications?
Fibrinogen, vWF, VIII and IX | Dysfibrinogenemia, DIC, vWF (last line)
48
Prothrombinex and indictions?
2, 9, 10, 11 | Warfarin reversal
49
Extrinsic pathway?
Activated by tissue factor (damage to tissue wall) Tissue factor -> factor VII TF & VIIa = X
50
Intrinsic pathway?
Triggered by contact activation platelet receptor 2b3a Activated Factor XII -> Factor XI XI -> IX IX + VIII + phospholipids = Factor X
51
Common pathway?
Activation of Factor X Xa + Ca + phospholipid + V = prothrombinase -> thrombin Thrombin cleaves fibrinogen to fibrin Factor 13 stabilises the clot
52
DIC investigation findings?
``` Thrombocytopenia, anaemia Fragmented, burr, helmet cells on film Reduced coag factors APTT and PT/INR both prolonged Increased D-Dimer ```
53
DIC mangement?
Treat trigger - sepsis, acidosis, hypoxia Platelet fusion (thrombocytopenia) Cryoprecipate (hypofibrinogenemia) FFP (coag factors/inhibitors)
54
Liver disease vs DIC?
Factor VIII levels in liver disease normal or high, in DIC LOW D-Dimer in liver disease normal, in DIC dramatically elevated
55
Haemophilia A, B and C?
``` A = Factor VIII deficiency B = Factor IX deficiency C = Factor XI deficiency ```
56
Genetic cause of Haemophilia A/B?
X-linked | Genes for both VIII and IX on the long arm of the X chromosome
57
Common presentations of Haemophilia?
Intracranial haemorrhage as newborn Haemarthrosis Psoas haemorrhage
58
Management of Haemophilia?
``` Avoid trauma Avoid NSAIDs/aspirin Desmopressin if Haemophilia A Recombinant Factor VIII & IX Rituximab if inhibitor present Activated Factor VIIa if inhibitor present ```
59
Chronic complications of Haemophilia?
Arthropathy - inflammatory response can result in target joint Development of inhibitor (IgG antibodies) Blood transfusion borne infection Obesity
60
Ashkenazi Jewish child with bleeding post dental surgery and prolonged PTT?
Haemophilia C
61
Treatment of von Willebrands?
Desmopressin - increases VWF release from endothelial cells Tranexamic acid PLASMA DERIVED Factor VIII
62
Risk factors for early onset haemorrhage disease of the newborn?
Maternal phenobarbital, phenytoin, warfarin, rifampicin
63
Risk factors for classic (2-7 days) haemorrhage disease of the newborn?
Vit K deficiency | Breastfeeding
64
Risk factors for late onset (1-6mths) haemorrhage disease of the newborn?
Cholestasis (vit K malabsorption) Biliary atresia Can be idiopathic in Asian breastfed infants
65
Thrombocytopenia Normal sized platelets Absent radius
Thrombocytopenia with absent radii
66
Mucosal bleeding Thromobocytopenia Large sized platelets
Bernard-Soulier
67
Bleeding Normal platelets Platelet aggregation normal with ristocetin but low with other agents
Glannzman Thrombasthenia
68
Sudden onset petechiae and mucosal bleeding Preceding viral illness Generally well
Idiopathic Thrombocytopenic Purpura
69
Risk of ICH in ITP?
<1%
70
Treatment of ITP?
``` 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
``` Fever Microangiopathic haemolytic anaemia Thrombocytopenia Abnormal renal function CNS changes ```
Thrombotic Thrombocytopenic Purpura

RACP Written Exam Cram Series (19 decks)

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