Haematology Flashcards

1
Q

What are the clinical features of multiple myeloma?

A
  • Bone disease: bone pain, osteoporosis + pathological fractures (typically vertebral), osteolytic lesions
  • Lethargy
  • Infection
  • Hypercalcaemia
  • Renal failure
    other features: amyloidosis e.g. Macroglossia, carpal tunnel syndrome; neuropathy; hyperviscosity

CRAB
Calcium high
Renal failure
Anaemia
Bone lesions

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2
Q

What is seen on x-ray in a multiple myeloma case?

A

X-rays: ‘rain-drop skull’ likened to the pattern rain forms after hitting a surface and splashing, where it leaves a random pattern of dark spots

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3
Q

What is the cause of idiopathic thrombocytopenic purpura?

A

Immune (or idiopathic) thrombocytopenic purpura (ITP) is an immune-mediated reduction in the platelet count caused by antiplatelet autoantibodies.

Acute ITP
more commonly seen in children
may follow an infection or vaccination
usually runs a self-limiting course over 1-2 weeks

Chronic ITP
more common in young/middle-aged women
tends to run a relapsing-remitting course of bleeding, purpura, epistaxis and menorrhagia.

No treatment if mild, platelets <20x10^9 prednisolone.

  • First-line treatment for ITP is oral prednisolone.
  • Human immunoglobulin (IVIG) may 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
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4
Q

What is the mode of inheritance of G6PD deficiency?

A

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the commonest red blood cell enzyme defect. It is more common in people from the Mediterranean and Africa and is inherited in an X-linked recessive fashion

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5
Q

How is G6PD deficiency diagnosed?

A

Diagnosis is made by using a G6PD Enzyme assay
- levels should be checked around 3 months after an acute episode of hemolysis as young RBCs may have enough enzyme so results normal.

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6
Q

What is seen in G6PD blood film?

A

Heinz bodies on blood films. Bite and blister cells may also be seen

Heinz (denatured haemglobin) –> think Fava BEANS

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7
Q

What can precipitate a crises in G6PD deficiency?

A

Drugs:
- anti-malarials: primaquine
- ciprofloxacin
- sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas

  • Broad (fava) beans
  • Infections
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8
Q

Management for DVT/PE in pregnanat woman?

A

Pregnancy is a hypercoagulable state
- warfarin contraindicated
- S/C low-molecular weight heparin preferred to IV heparin (less bleeding and thrombocytopenia)

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9
Q

What is the characteristic histological cell type found in Hodgkin’s lymphoma?

A

Reed-Sternberg cells

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10
Q

What are the causes of macrocytic anaemia?

A

Megaloblastic causes:
- Vitamin B12 deficiency
- Folate deficiency

Normoblastic causes:
- Alcohol abuse
- Liver disease
- Hypothyroidism
- Pregnancy
- Reticulocytosis (haemolysis)
- Myelodysplasia
drugs: Cytotoxics

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11
Q

What is the appropriate treatment for a patient who is both vitamin B12 and folic acid deficient?

A

Intramuscular vitamin B12 + start oral folic acid when vitamin B12 levels are normal.

It is important in a patient who is also deficient in both vitamin B12 and folic acid to treat the B12 deficiency first to avoid precipitating subacute combined degeneration of the cord.

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12
Q

What are the complications of tumour lysis syndrome?

A
  • Hyperkalaemia
  • Hyperphosphataemia
  • Hypocalcaemia
  • Hyperuricaemia
  • Acute renal failure
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13
Q

What is the most common inherited bleeding disorder?

A

Von Willebrand’s disease

The majority of cases are inherited in an autosomal dominant fashion (Type 1) and characteristically behaves like a platelet disorder i.e. epistaxis and menorrhagia are common whilst haemoarthroses and muscle haematomas are rare

Role of von Willebrand factor:
- promotes platelet adhesion to damaged endothelium
- carrier molecule for factor VIII

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14
Q

What is the mode of inheritance for haemophilia?

A

Haemophilia A and B are both X-linked recessive disorders

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15
Q

What is Haemophilia? Features? blood test results?

A

Haemophilia A is due to a deficiency of factor VIII.
Haemophilia B (Christmas disease) there is a lack of factor IX

  • haemoarthroses, haematomas
  • prolonged bleeding after surgery or trauma
  • prolonged APTT
  • bleeding time, thrombin time, prothrombin time normal!!
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16
Q

How is a suspected DVT investigated? What is the scoring system used? Which further tests are needed to confirm the diagnosis?

A

If a patient is suspected of having a DVT a two-level DVT Wells score should be performed.

Clinical probability simplified score
DVT likely: 2 points or more
DVT unlikely: 1 point or less

If a DVT is ‘likely’ (2 points or more)
- a proximal leg vein ultrasound scan should be carried out within 4 hours.
- if the result is positive then a diagnosis of DVT is made and anticoagulant treatment should start
- if the result is negative a D-dimer test should be arranged. A negative scan and negative D-dimer makes the diagnosis unlikely and alternative diagnoses should be considered

  • if a proximal leg vein ultrasound scan cannot be carried out within 4 hours a D-dimer test should be performed and interim therapeutic anticoagulation administered whilst waiting for the proximal leg vein ultrasound scan (which should be performed within 24 hours)
  • interim therapeutic anticoagulation used to mean giving low-molecular weight heparin. NICE updated their guidance in 2020. They now recommend using an anticoagulant that can be continued if the result is positive. this means normally a direct oral anticoagulant (DOAC) such as apixaban or rivaroxaban!

if the scan is negative but the D-dimer is positive:
stop interim therapeutic anticoagulation
offer a repeat proximal leg vein ultrasound scan 6 to 8 days later

If a DVT is ‘unlikely’ (1 point or less)
- perform a D-dimer test
- this should be done within 4 hours. If not, interim therapeutic anticoagulation should be given until the result is available
- if the result is negative then DVT is unlikely and alternative diagnoses should be considered
- if the result is positive then a proximal leg vein ultrasound scan should be carried out within 4 hours
- if a proximal leg vein ultrasound scan cannot be carried out within 4 hours interim therapeutic anticoagulation should be administered whilst waiting for the proximal leg vein ultrasound scan (which should be performed within 24 hours)

17
Q

How is a DVT managed? What drug is given?

A

Apixaban or rivaroxaban (both DOACs) should be offered first-line following the diagnosis of a DVT

Instead of using low-molecular weight heparin (LMWH) until the diagnosis is confirmed, NICE now advocate using a DOAC once a diagnosis is suspected, with this continued if the diagnosis is confirmed

If neither apixaban or rivaroxaban are suitable then either LMWH followed by dabigatran or edoxaban OR LMWH followed by a vitamin K antagonist (i.e. warfarin)

18
Q

In cases of DVT, how long should the anticoagulation be given for?

A
  • All patients should have anticoagulation for at least 3 months. Continuing anticoagulation after this period is partly determined by whether the VTE was provoked or unprovoked.
  • A provoked VTE is due to an obvious precipitating event e.g. immobilisation following major surgery. The implication is that this event was transient and the patient is no longer at increased risk.
  • An unprovoked VTE occurs in the absence of an obvious precipitating event, i.e. there is a possibility that there are unknown factors (e.g. mild thrombophilia) making the patient more at risk from further clots.
  • If the VTE was provoked the treatment is typically stopped after the initial 3 months (3 to 6 months for people with active cancer)
  • If the VTE was unprovoked then treatment is typically continued for up to 3 further months (i.e. 6 months in total)

NICE recommend that whether a patient has a total of 3-6 months anticoagulant is based upon balancing a person’s risk of VTE recurrence and their risk of bleeding. The HAS-BLED score can be used to help assess the risk of bleeding
NICE state: ‘Explain to people with unprovoked DVT or PE and a low bleeding risk that the benefits of continuing anticoagulation treatment are likely to outweigh the risks. ‘. The implication of this is that in the absence of a bleeding risk factors, patients are generally better off continuing anticoagulation for a total of 6 months

19
Q

What is the empirical antibiotic of choice for neutropenic sepsis?

A

Neutropenic sepsis is a relatively common complication of cancer therapy, usually as a consequence of chemotherapy. It most commonly occurs 7-14 days after chemotherapy. It may be defined as a neutrophil count of < 0.5 * 109 in a patient who is having anticancer treatment and has one of the following:
- a temperature higher than 38ºC or
- other signs or symptoms consistent with clinically significant sepsis

Management:
- Antibiotics must be started immediately, do not wait for the WBC
- NICE recommends starting empirical antibiotic therapy with piperacillin with tazobactam (Tazocin) immediately!

20
Q

Symptoms and signs of anaemia?

A

Symptoms:
- Fatigue
- Dyspnoea
- Faintness
- Palpitations
- Headache
- Tinnitus
- Anorexia
- Angina (if pre-existing coronary artery disease)

Signs:
- Pallor (conjunctiva)
- In severe anemia, there may be hyperdynamic circulation (Tachycardia, flow murmurs, cardiac enlargement)

21
Q

What are the causes of microcytic anaemia?

A
  1. Iron deficiency anaemia (the most common cause)
  2. Thalassemia
  3. Sideroblastic anaemia
22
Q

What are the causes of normocytic anaemia?

A
  1. Acute blood loss
  2. Anaemia of chronic disease (low mcv also can)
  3. Bone marrow failure
  4. Renal failure
  5. Hypothyroidism (high mcv also can)
  6. Haemolysis (high mcv also can)
  7. Pregnancy
23
Q

What is the management in a patient on warfarin who presents with major bleeding?

A
  1. Stop warfarin
  2. Give intravenous vitamin K 5mg
  3. Prothrombin complex concentrate - if not available then FFP
24
Q

Why is Heparin usually given at the same time with warfarin?

A

Heparin must be given by injection, whereas warfarin is available orally. Warfarin has a long half-life and need only be given once a day.

Warfarin is slower-acting than the common anticoagulant heparin. It takes several days for warfarin to reach the therapeutic effect. Warfarin’s long half-life means that it remains effective for several days after it is stopped.

If given initially without additional anticoagulant cover, it can increase thrombosis risk, when warfarin is newly started, it may promote clot formation temporarily. For these main reasons, hospitalised patients are usually given heparin with warfarin initially, the heparin covering the 3–5-day lag period and being withdrawn after a few days, in order to have the benefit of anticoagulation from heparin until the full effect of warfarin has been achieved.

25
Q

Which medications are associated with increased risk of VTE?

A
  • Combined oral contraceptive pill: 3rd generation more than 2nd generation
  • Hormone replacement therapy: the risk of VTE is higher in women taking oestrogen + progestogen preparations compared to those taking oestrogen only preparations
  • Raloxifene and tamoxifen
  • Antipsychotics (especially olanzapine) have recently been shown to be a risk factor
26
Q

Investigation of urine in multiple myeloma?

A

Monoclonal proteins (usually IgG or IgA) in the serum and Bence Jones proteins in urine

27
Q

What is the blood picture in DIC?

A

A typical blood picture includes:
- low platelets
- prolonged APTT, prothrombin and bleeding time
- fibrin degradation products are often raised

28
Q

What are the B symptoms?

A

fever greater than 38 C
weight loss > 10% in last 6 months
night sweats

B symptoms refer to systemic symptoms of fever, night sweats, and weight loss which can be associated with both Hodgkin lymphoma and non-Hodgkin lymphoma. The presence or absence of B symptoms has prognostic significance and is reflected in the staging of these lymphomas.

29
Q

What is the cause of pernicious anaemia?

A

Pernicious anaemia is a vitamin B12 deficiency anaemia that is due to a lack of intrinsic factor. A special protein, called intrinsic factor (IF), binds vitamin B12 so that it can be absorbed in the intestines. This protein is released by parietal cells in the stomach. When the stomach does not make enough intrinsic factor, the intestine cannot properly absorb vitamin B12.

Lack of intrinsic factor is most commonly due to an autoimmune disease which causes antibodies to attack gastric parietal cells or the intrinsic factors resulting in B12 deficiency.

  • more common in females, typically develops in middle to old age
  • Associated with thyroid disease, diabetes, Addison’s, rheumatoid and vitiligo
  • Predisposes to gastric carcinoma
  • Treatment of the disorder is with 3 monthly treatment of vitamin B12 injections. Folic acid supplementation may also be required.
30
Q

Triad of symptoms seen in vitamin B12 deficiency?

A
  • Fatigue
  • Paraesthesia
  • Tongue soreness (glossitis) (beefy red tongue)
31
Q

Which condition is Heinz bodies seen?

A

Heinz bodies are inclusions within red blood cells due to a number of conditions, including alpha-thalassaemia and glucose-6-phosphate dehydrogenase deficiency.

32
Q

Reversal agent for Dabigatran?

A

Idarucizumab is a reversal agent for dabigatran.

33
Q

Reversal agent for Apixaban and Rivaroxaban?

A

Andexanet alfa.
Andexanet alfa is a recombinant form of human factor Xa protein.

34
Q

Clotting profile seen in Von Willebrand?

A

Von Willebrand’s disease is the most common inherited bleeding disorder. The majority of cases are inherited in an autosomal dominant fashion.

  • Epistaxis and menorrhagia are common whilst haemoarthroses and muscle haematomas are rare

Prolonged bleeding time
Prolonged APTT
Normal platelets

35
Q

Auer rods is seen in which condition?

A

Acute promyelocytic leukaemia.

36
Q
A