Haematology Flashcards

1
Q

What are the components of the HAS-BLED score?

A
  • HTN: SBP > 160
  • Renal disease: Dialysis, transplant, Cr>200
  • Liver disease: cirrhosis or bili > 2x ULN PLUS AST/ALT/ALP > 3x ULN
  • Stroke Hx
  • Prior major bleeding or predisposition to bleeding
  • Labile INR
  • Age > 65
  • Bleedy meds: aspirin, clopidogrel, NSAIDs
  • EtOH excess: 8 or more drinks/wk
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2
Q

What is the DDx for spur cells (acanthocytes)?

A
Liver failure
Alcoholism
Hypothyroidism
Anorexia nervosa
Congestive splenomegaly
Abetalipoproteinaemia
Chronic granulomatous disease
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3
Q

What is the DDx for burr cells (echinocytes)?

A
Uraemia
Hypomagnesaemia
Hypophosphataemia
Pyruvate kinase deficiency
Haemolytic anaemia
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4
Q

What are the complications of polycythaemia rubra vera?

A
Stroke
Bleeding (acquired Von Willebrand's Disease)
Erythromelalgia
Gout
Pruritus
Diaphoresis
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5
Q

What are the examination findings in polycythaemia vera?

A
Digital infarcts secondary to erythromelalgia
HTN
Easy bruising
Plethora
Gouty tophi
Neurological deficits
Retinal venous engorgement
Splenomegaly
Splenic infarction
Portal HTN (portal vein thrombosis or Budd-Chiari syndrome)
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6
Q

Which investigations should be considered in polycythaemia rubra vera?

A

Hb and red cell mass (should be normal in secondary polycythaemia
Epo level (low in PRV)
SaO2 to exclude secondary to hypoxia
Overnight oxymetry to exclude OSA causing polycythaemia
Uric acid (elevated)
B12 levels (elevated)
Iron studies (low)
Abdominal US to exclude Epo-secreting mass
Bone marrow Bx
Cytogenetics
JAK2

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7
Q

What are the treatment options in polycythaemia rubra vera?

A
Phlebotomy
Aspirin for all
Hydroxyurea
IFN (younger or may become pregnant)
Ruxolitinib (severe symptoms)
Allopurinol (hyperuricaemia)
Splenectomy
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8
Q

Which malignancies are associated with elevated ferritin?

A

Breat cancer
Pancreatic cancer
HCC
Haematological cancers

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9
Q

Which non-malignant conditions are associated with elevated ferritin?

A
HLH
Still's Disease
Sideroblastic anaemia
Fatty liver disease
Obesity
EtOH excess
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10
Q

What are the favourable prognostic factors in multiple myeloma?

A
Albumin > 35 g/L
Normal LDH
β2-microglobulin < 3.5mg/L
Absence of:
- t(4;14)
- t(14;16)
- del(17p)
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11
Q

What is the pharmacological therapeutic ladder in multiple myeloma?

A

Thalidomide + bortezomib (proteasome inhibitor)*

  • *Lenalidomide (with dexamethasone) first line in non-transplant-eligible, second line in transplant-eligible
  • Carfilzomib second line proteasome inhibitor
  • Pomalidomide where bortezomib and lenalidomide fail
  • Daratumumab (anti-CD38) where all PBS options have failed

Newer agents:

  • Ixazomib (proteasome inhibitor)
  • Elotuzumab (SLAMF7 inhibitor)
  • CAR-T cells in trials
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12
Q

How are transplant-eligible patients treated?

A
  • Proteasome inhibitor induction
  • Autologous stem cell transplant
  • Maintenance Rx with thalidomide
  • In young patients with recurrence after auto-HSCT, consider allo-HSCT from HLA-matched donor
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13
Q

How is Minimal Residual Disease (MRD) tested in multiple myeloma and why is it tested?

A

Next generation flow cytometry OR

Next generation sequencing

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14
Q

What supportive Rx is available in multiple myeloma?

A
  • Osteoporosis: denosumab, zoledronic acid

- Cast nephropathy: proteasome inhibitors

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15
Q

What immunodeficiencies are associated with CLL?

A
Hypogammaglobulinaemia
IgG subclass deficiency (IgG3 in particular)
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16
Q

What are the anticoagulant options in HITS?

A
  • Fondaparinux or danaparoid or argatroban or rivaroxaban
  • Argatroban in renal impairment
  • Bivalirudin IV infusion if requiring urgent surgery
17
Q

How is high risk myeloma defined?

A

Overall survival of 2yrs or less despite treatment with immunomodulatory drug and a proteasome inhibitor.

  • ISS uses beta2-microglobulin>5.5mg/L and albumin>35g/L to stratify prognosis
  • Revised ISS uses beta2-microglobulin, albumin, LDH and high risk FiSH profile [ del17p or t(4;14) ]
18
Q

What are alternatives to CT or MRI for detecting myelomatous bony lesions?

A

Sestamibi (sensitivity 92%, specificity 96%)

PET (sensitivity 85%, specificity 92%)

19
Q

What is the monitoring regime for MGUS?

A
  • When serum paraprotein level is ≤15g/l and stable, IgG type, and normal SFLC kappa: lambda ratio, SPEP can be repeated annually.
  • When paraprotein >15g/l or abnormal SFLC kappa:lambda ratio, a bone marrow aspirate and trephine is considered if paraprotein is rising to assess for evidence of MM. If these results are satisfactory, 6 monthly intervals for 1 year, then yearly provided the treating physician is contacted upon any clinical changes
20
Q

What is the most common induction regime for mutiple myeloma in Australia?

A

CyBorD

  • Cyclophosphamide
  • Bortezomib (subcut)
  • Dexamethasone
21
Q

What supportive measures are available for localised bony myeloma lesions?

A
  • Radiotherapy
  • Internal fixation where fracture risk of long bones exists
  • Balloon kyphplasty for painful compression fractures
  • Bisphosphonates
22
Q

Does VTE risk increase with thalidomide or lenalidomide?

A

Only when in combination with dexamethasone

23
Q

When is recombinant Epo used in myeloma?

A

Those with renal failure and anaemia

24
Q

Which myeloma patients are eligible for regular IVIG?

A

Recurrent infections (≥2 chest infections per year) and hypogammaglobulinaemia

25
Q

What infection prophylaxis is recommended in myeloma?

A
  • Valaciclovir, acyclovir or famciclovir for VZV if on proteasome inhibitor
  • Bactrim for PJP if on equivalent of prednisone 20mg daily for ≥ 4wks. Second line are dapsone, pentamidine or atovaquone.
  • Vaccinations against hepatitis B, pneumococcus, influenza.
26
Q

What is the prognostic value of the presence of myeloma biomarkers? What are the biomarkers?

A
80% probability of developing end organ damage in 3 years.
Biomarkers:
- SFLC ratio ≥ 100 (involved:uninvolved)
- Bone marrow plasma cells ≥ 60%
- MRI demonstrating >1 focal bony lesion
27
Q

What are the functions of immunomodulatory drugs and proteasome inihibitors in myeloma?

A

IMiDs:

  • Increase in T-cell costimulation and enhance NK cell activity
  • Induction of apoptosis and antiangiogenesis

PIs:

  • Inhibition of clearance of misfolded proteins
  • Reduction of cytokines that promote MM-cell growth
  • Accumulation of tumour suppressor proteins
28
Q

Which medications may interact with warfarin to increase bleeding risk?

A
Cephalosporins
Penicillins (except dicloxacillin)
Metronidazole
Macrolides
Doxycycline
Quinolones
Sulfamethoxazole
Imidazole antifungals (azoles)
COX-2 inhibitors
Amiodarone
Fenofibrate
Statins (particularly simvastatin)
Valproic acid
SSRIs, SNRIs
Tamoxifen
Testosterone
Imatinib
MTX
Leflunomide
Influenza vaccine

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29
Q

Which medications may interact with warfarin to increase clotting risk?

A
St John's Wort
Infliximab
Azathioprine/6-MP
Dicloxacillin
Aprepitant

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