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Haem Flashcards

1
Q

How to replace B12/folate?

A

1mg B12 injections 3 times per week for 2 weeks, then once every 3 months

5mg folate tablets also, but not before B12 as risks SACD

think BeFore - B before F

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2
Q

Causes of high ferritin without iron overload?
With iron overload?
How to tell if there is iron overload?

A

Without (90%):

  • inflammation (acute phase protein)
  • alcohol excess
  • liver disease
  • CKD
  • malignancy

With:

  • Haemochromatosis
  • repeated transfusions

Normal values of transferrin saturation normally excludes iron overload (<45% females and <50% males)

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3
Q

Difference between direct and indirect Coombs test?

A

Direct (DAT):
To test for haemolytic anaemia
Patients RBC are mixed with anti-human Ig (antibodies against human antibodies)
If they are present the cells will agglutinate

Indirect:
Tests for Ig in patient’s plasma (e.g. prenatal for anti-D and before blood transfusion)
Patient’s plasma mixed with donor blood
If Ig present, these will attach to RBC surface
As before, anti-human Ig added, causing agglutination if Ig have attached to RBC surface

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4
Q

If suspected DVT what should you do if:

  • DVT likely?
  • DVT unlikely?
A

2 level wells score

If score 2+ (likely):

  • USS doppler within 4 hours
  • if cannot be done within 4 hours, D-dimer and give anticoagulation; ensure USS within 24 hours
  • if scan negative but D-dimer positive, stop anticoagulation and repeat scan in 6-8 days

If score <2 (unlikely):

  • D-dimer
  • if neg, other diagnosis
  • if pos, doppler USS within 4 hours - again if cannot be done anticoagulate in meantime
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5
Q

Rx for DVT?

If renal failure or anti-phospholipid syndrome?

A

Normally:

  1. DOAC (apixaban/rivaroxaban)
  2. LMWH
  3. Dabigatran
  4. Warfarin

If eGFR<15 or antiphospholipid syndrome:

  1. LMWH
  2. Warfarin
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6
Q

4 most sensitive clinical signs of PE?

A

Tachypnoea (96%)
Crackles (58%)
Tachycardia (44%)
Fever (43%)

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7
Q

If suspected PE what should you do to investigate?

A

PE rule out criteria (PERC)
then 2 PE level Wells score

If >4 points (likely):
- arrange immediate CTPA - if there is a delay then anticoagulate

CTPA +ve - diagnosis
CTPA -ve - leg doppler USS

If <=4 (unlikely):
- D-dimer
+ve then CTPA and anticoagulate
-ve then stop any anticoagulation and consider alternative

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8
Q

Apart from Wells, CTPA etc, what else should be done for suspected PE?

A

ECG:

  • sinus tachycardia
  • S1, Q3, T3 in 20%
  • RBBB or RAD may occur

CXR:

  • Recommended for all patients whilst waiting on CTPA to rule out other pathology
  • Typically normal in PE - may show wedge-shaped opacification
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9
Q

Treatment of PE?
Haemodynamic instability?
Repeat PE?

A

Normally:

  1. DOAC (apixaban, rivaroxaban)
  2. LMWH
  3. Dabigatran
  4. Warfarin

If eGFR <15 or anti-phospholipid syndrome:

  1. LMWH
  2. Warfarin

If haemodynamically unstable:
thrombolysis
OR
thrombectomy (if facilities available)

In repeat PE, IVC filters may be used

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10
Q

Length of anticoagulation in DVT/PE?

A

If provoking factor e.g. immobilisation following surgery, COCP - this transient risk will pass:
3 months

If unprovoked or cancer:
6 months and calculate HASBLED score

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11
Q

Monitoring of unfractioned heparin?

Monitoring of LMWH?

A

aPTT

LMWH - no monitoring

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12
Q

Polycythaemia rubra vera:

  • What is it?
  • features?
  • management?
  • prognosis?
A

Clonal proliferation leading to increased red cells. May also be accompanying increased neutrophils and PLT

Features:

  • itch - worse when hot
  • ‘ruddy complecion’
  • Splenomegaly
  • hyperviscosity
  • JAK2 +ve

Rx:

  • aspirin
  • venesection (keep Hb normal)
  • chemo - hydroxyurea (slight increased risk of secondary leukaemia)

Prognosis:

  • thrombotic events common
  • 10% progress to myelofibrosis
  • 10% progress to acute leukaemia (risk increased with chemo)
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13
Q

Sickle cell crises:

  • thrombotic?
  • sequestration?
  • acute chest?
  • aplastic?
  • haemolytic?
A

Thrombotic: vaso-occlusive, caused by infection/dehydration. Clinically diagnosed from pain. Infarcts occur in various organs including bones (AVN)

Sequestration: pooling in spleen or lungs with worsening anaemia

Acute chest: chest pain, dyspnoea, bilateral infiltrates on CXR, low pO2. Commonest cause of death after childhood.

Aplastic: caused by parvovirus B19, sudden fall in Hb

haemolytic: rare, increased rate of haemolysis

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14
Q

Features of G6PD deficiency?
What is seen on film of G6PD deficiency?
Diagnosis?
Inheritance?

A

Features: intravascular haemolysis, gallstones, splenomegaly (neonatal jaundice common)

Heinz bodies
Bite cells
Blister cells

Diagnosis: G6PD enzyme assay - check levels 3 months after acute haemolysis

X-linked recessive

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15
Q

Hereditary spherocytosis inheritance?
features?
Diagnosis?
Management acute and long-term?

A

AD

Failure to thrive, jaundice, gallstones, splenomegaly, MCHC elevated
Parvovirus causes aplastic crisis
spherocytes on film

Diagnosis: EMA binding test

Management:
acute - supportive, transfusion
long term - splenectomy, folate supplements

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16
Q

What is seen in hyposplenism on film?

A

Howell-Jolly bodies
Pappenheimer bodies
Target cells

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17
Q

Can LMWH cause heparin-induced thrombocytopaenia?

How can it cause a PE?

A

Yes

Heparin/LMWH binds to a platelet factor on the surface and causes an immune response. Some people form IgG which bind to the heparin-PF4 complex, which can actually activate the platelet, causing a clot

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18
Q

Primary haemostasis?

A

Vessel wall damage exposes collagen which platelets bind to via vWF

Platelets activated and change shape, release ADP and TXA2 which stimulate aggregation,

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19
Q

Extrinsic pathway?

Measurement?

A

Most important

Tissue factor (factor 3) is released from damaged vessel walls and binds to factor 7

TF/7 converges on common pathway and activates factor 10

Measure: PTT

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20
Q

Intrinsic pathway?

Measurement?

A

Amplification of response

Exposed collaged activates factor 12
12-11-9

9a binds and activates 8

9a/8a converge on common pathway

Measure: aPTT

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21
Q

Common pathway?

A

TF/7 or 9/8 activate 10

10a binds 5a to activate prothrombin (2) to thrombin (2a)

This converts fibrinogen to fibrin

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22
Q

Control of haemostasis?

A

ATIII - protease that blocks all pathways of the cascade

Protein C/S - proteins that are on the surface of platelets and inhibit factor 5/8

Fibrinolysis - breakdown of fibrin by plasmin, which is activated from plasminogen by tissue plasminogen factor

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23
Q

ITP?

A

Autoimmune condition against platelets in adults or children

Isolated low platelets

Kids: aged 2-6, self-limiting post-infection

Adults: variable and fluctuating course
1st line - steroids
2nd - pooled IVIG

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24
Q

TTP:

  • what is it?
  • what gene?
  • features?
  • bloods?
  • film?
  • management?
A

Emergency characterised by thrombocytopaenia + MAHA - widespread micro-thrombi and intravascular haemolysis, caused by a trigger

ADAMTS-13

Fever, bleeding, AKI (haematuria + proteinuria), fluctuating CNS signs (drowsy, confusion, seizures)

Bloods:
profound thrombocytopaenia
MAHA: low Hb, high LDH, high bili, low haptoglobin
NORMAL COAGULATION

Film: schistocytes

Management: Plasmapheresis

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25
Management of HUS?
Supportive - no role for abx
26
Causes of severe thrombocytopaenia? | Mild?
ITP TTP DIC Malignancy Mild: - heparin-induced - drugs - diuretics, aspirin, thiazides - alcohol, liver disease - hypersplenism - pregnancy - SLE/APS - B12 def
27
Causes of thrombocytosis?
Reactive - acute phase reactant Malignancy Hyposplenism Essential thrombocytosis
28
What is essential thrombocytosis? Features? Mutation? Management?
Myeloproliferative disorder which overlaps with CML and polycythaemia rubs vera and myelofibrosis. Megakaryocyte overproduction causing increased PLT Both arterial or venous thrombus or haemorrhage can be seen Characteristic symptom - burning sensation in hands JAK2 mutation Management - hydroxyurea aspirin interferon a in younger people
29
What is seen on coag of vWD?
increased aPTT and bleeding time | low vWF and factor 8
30
Haemophilia A and B? | Seen on coag?
``` A = factor 8 def B = factor 9 def ``` Coag: increased aPTT Factor infusions
31
Seen on coag of liver disease?
Increased PTT/aPTT May be increased TT if production of prothrombin reduced
32
Causes of abnormal platelets?
Drugs: NSAIDs, aspirin, anti-platelets Systemic: renal/liver disease Haem: paraprotein/myeloproliferative disease
33
Bloods for DIC? | Management?
``` D dimers - high PLT - low Bleeding - high PTT, aPTT, TT - long Fibrin degradation products - high Film: schistocytes ``` Management: - Treat underlying cause - PLT transfusion - FFP - Cryoprecipitate - RBC infusion
34
General what causes an increase in: - PTT? - aPTT? - TT? - Everything?
PTT - liver disease, warfarin aPTT - haemophilia, vWD, heparin TT - heparin, advanced liver disease everything - DIC
35
Coag for ITP?
low PLT
36
Coag for TTP?
low PLT | low Hb, high LDH, low haptoglobin, schistocytes
37
Coag for vWD?
increased aPTT | low factor 8
38
Coag for haemophilia?
increased aPTT
39
Coag for liver disease?
Increased PTT, aPTT, late on increased TT as well
40
Coag for vitK def/warfarin?
Very high PTT, high aPTT
41
Coag for heparin?
high PTT, very high aPTT and TT
42
``` MOA heparin? Excretion? Coag? Monitoring? When to stop before surgery? ```
potentiates ATIII, inhibits factor Xa (LMWH Xa only, unfractioned also 2, 9, 11 and 12) Renal Coag - increased PTT, very high aPTT and TT Monitor - aPTT Before surgery - stop the day before
43
Fondaparinux MOA?
Also potentiates ATIII to inhibit factor Xa
44
Warfarin change in coag? When starting warfarin? Monitoring? When to stop before surgery?
Very high PTT, high aPTT Cover with heparin for a couple of days INR - modified PTT compared to international normal 2-3 = DVT, PE, AF 3-4 = VET on warfarin, APS, metallic valve - check INR daily until within range 2 days in a row, then check twice weekly then weekly. Once stable check every 6-12 weeks 5 days before surgery
45
What can potentiate warfarin?
Liver disease Cranberry juice P450 inhibitors (cipro, amiodarone, valproate) NSAIDs
46
Management of warfarin if: - major bleeding? - INR 5+, minor bleeding? - INR >8, no bleeding? - INR 5-8, no bleeding?
Major: stop warfarin, IV vit K and prothrombin complex concentrate INR 5+, minor bleed: Stop warfarin, IV vit K, restart when INR <5 INR >8, no bleeding: stop warfarin, oral VitK using IV preparation, restart when INR <5 INR 5-8, no bleeding: withhold 1-2 doses of warfarin, reduce maintenance dose
47
MOA Dabigatran?
2a inhibitor
48
Causes of inherited thrombophilia?
Factor V leiden (protein C resistance) - most common Prothrombin mutation - 2nd commonest Others: - ATIII, protein C or protein S deficiency
49
Causes of acquired thrombophilia?
APS | COCP
50
when to consider thrombophilia?
Recurrent VTE or miscarriage VTE <40y/o VTE in unusual place e.g. mesenteric/cerebral veins
51
Common lymphoid precursor produces? Common myeloid precursor? Common erythroid precursor?
Lymphoid: T, B and NK cells Myeloid: granulocytes - neutrophils, basophils, eosinophils, macrophages Erythroid: PLT, RBC
52
What drug can cause neutrophilia?
Steroids (due to reduced marginisation)
53
What are atypical lymphocytes in glandular fever?
Increase in cytoplasm and wrapping around the cell
54
What causes conversion to Fe3+?
Oxidative stress
55
What controls iron absorption via down regulation of ferroportin in response to iron load and inflammation?
Hepcidin
56
Iron, ferritin and TIBC in: - haemochromatosis? - iron deficiency anaemia? - AOCD? - thalassaemia?
Haem: iron and ferritin raised, TIBC low Iron def: iron and ferritin low, TIBC high AOCD: iron low, ferritin high, TIBC low Thalassaemia: iron normal or high, ferritin normal or high, TIBC normal
57
Main 3 causes of microcytic anaemia
Iron def Thalassaemia AOCD
58
Iron replacement if uncomplicated?
200mg ferrous fumarate/sulfate TDS for a minimum of 3 months
59
What is seen on film with a thalassaemia trait?
Heinz bodies - accumulations of B globin tetramers
60
What is seen on film in HbH? On HPLC? Management?
Heinz bodies, target cells HPLC - low HbA, HbA2 and HbF High HbH - with B globin tetramers Folic acid Intermittent transfusions May need splenectomy
61
Physical features of B thalassaemia major? CXR? HPLC? Management?
Severe anaemia Extramedullary haematopoiesis - hepatosplenomegaly, skull bossing CXR - hair on end sign HPLC - High HbF, HbA2, low HbA Regular transfusions and iron chelators (desferrioxamine)
62
Where are folate and B12 absorbed in bowel?
Folate - duodenum B12 - ileum
63
In pernicious anaemia which Ig's are sensitive and specific?
Anti-parietal cell - sensitive, not specific Anti-IF - specific, not sensitive
64
What is seen on film of B12 deficiency anaemia?
Hypersegmented neutrophils | Howel Jolly bodies
65
Causes of non-megaloblastic macrocytosis (may not have anaemia)?
Alcohol Pregnancy Hypothyroidism Liver disease - TARGET CELLS Marrow failure - myelodysplasia/aplastic anaemia - unlike the others this will always cause anaemia
66
What causes spurious macrocytosis?
Reticulocytosis Cold agglutination (reticulocytosis - haemolysis, haemorrhage, treatment of anaemia, thalassaemia)
67
3 hallmarks of extravascular haemolysis?
Hepatosplenomegaly Protoporphyrin release (converted to unconjugated bilirubin, causing jaundice and pigment gall stones) Urobilinogen (normal products in excess)
68
Hallmarks of intravascular haemolysis?
Schistocytes Increased LDH Low haptoglobin Haemoglobinaemia
69
Causes of extravascular haemolysis?
Thalassaemia Sickle cell Hereditary spherocytosis Autoimmune haemolytic anaemia
70
Woman returns from Haiti 25 days ago and has fever/chills on alternating days, headache and myalgia. O/E temp 39, hepatomegaly - what is it?
Malaria
71
Platelet transfusion limit in active bleeding?
30 in most cases Can be up to 100 if bleeding at critical site such as CNS
72
Platelet transfusion limit for pre-invasive procedure?
50 for most 50-75 if risk of bleeding 100 if surgery at a critical site
73
Platelet transfusion threshold if no bleeding or planned invasive procedure? Contraindications for platelet transfusion? (4)
10 Contra: - Chronic bone marrow failure - ITP - Heparin-induced thrombocytopaenia - TTP
74
Atypical blood cells seen on film with hyposplenism?
Howell-Jolly bodies Target cells Pappemheimer bodies
75
Seen on film with iron-deficiency anaemia? | If combined iron deficiency/B12 deficiency?
Fe def: - target cells - pencil poikilocytes - microcytic hypochromic Mixed: - microcytic and macrocytic (dimorphic) film - so MCV often normal
76
What to prescribe to people with heart failure who are receiving packed red cells?
Furosemide dose STAT - even if already on it
77
What can polycythaemia rub vera progress to?
AML | Myelofibrosis
78
Management of vWD in mild bleeding? What can raise levels of vWF? What else can be given if severe?
Mild - tranexamic acid Desmopressin can raise levels of vWF by inducing release of vWF from endothelial cells Factor VIII concentrate
79
Commonest ages of Hodgkin's?
Bimodal peak - 30's and 70's
80
Types of Hodgkin's? | Typical symptoms?
- Nodular sclerosing - most common - good prognosis - Mixed cellularity - lymphocyte predominant - best prognosis - lymphocyte depleted - rare, worst prognosis 'B' symptoms - weight loss, fever, night sweats Presence of B symptoms are assoc w a poor prognosis
81
How is tranexamic acid given in major haemorrhage?
IV bolus followed by slow infusion
82
Features of Waldenstrom's Macroglobulinaemia?
Lymphoplasmacytoid malignancy characterised by secretion of monoclonal IgM paraprotein Weight loss, lethargy Hyperviscosity - visual disturbance, stroke etc Hepatosplenomegaly Cryoglobulinaemia e.g. Raynaud's Renal function fine No bone lesions on skeletal survey
83
What is generally given 1st line in DIC after resus measures?
FFP or cryoprecipitate
84
Over how long is a unit of RBC transfused in non-urgent cases?
90-120 mins
85
What malignancy might EBV cause?
Non-hodgkins lymphoma (specifically Burkitt's)
86
Apart from biopsy, what in Hx can help differentiate hodgkin's from non-hodgkin's?
Hodgkins get alcohol-induced pain B symptoms typically occur later in non-hodgkins Extra nodal disease is commoner in non-hodgkins (marrow failure, bone pain, gastric, CNS, testicular)
87
Ix for lymphoma? | What are good prognostic indicators?
1. biopsy 2. CTCAP - staging 3. FBC - rule out leukaemia Offer HIV - RF for non-hodgkin's LDH and ESR - prognostic indicator
88
Staging of lymphoma?
1. one node 2. more than one node on same side of diaphragm 3. nodes on both sides of diaphragm 4. extra-nodal disease
89
Commonest type of non-hodgkin's lymphoma? Specifically associated with immunosuppressed? Rarest? Other types?
Commonest - diffuse large B cell (40%) - high grade but these typically respond better to chemo Burkitt's - immunosuppressed, young, 'starry-sky', c-myc mutation Rarest - Mantle cell Others - follicular - low grade Cutaneous T cell - quite rare
90
What DOAC should be avoided in kidney failure? | What one should be used?
Dabigatran - renally excreted Use Apixaban - faecally excreted (Rivaroxaban is hepatic ally excreted)
91
How long should cancer patients get VTE prophylaxis after DVT/PE?
6 months Exception to the rule
92
Indications for cryoprecipitate use? | What is its major constituent?
Major haemorrhage and haemophilia Major constituent = factor VIII
93
What leukaemia can cause raised platelets?
CML
94
Management of APS in pregnancy?
Aspirin as soon as pregnancy confirmed LMWH once foetal heart is seen (around 6-7 weeks) and discontinued at 34 weeks
95
Are any Ix needed to be performed in sickle cell crises?
No, clinical diagnosis
96
What is seen on film with sickle cell disease?
Howell-Jolly bodies Target cells (due to hyposplenism)
97
How often should sickle cell patients be given the pneumococcal vaccine?
Every 5 years
98
Common presentation of sickle cell in late infancy?
Hand-foot syndrome - both hands and feet get swollen
99
Management of post-thrombotic syndrome?
Stockings

Finals Revision (29 decks)

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