Haematology Flashcards
What are the components of the HAS-BLED score?
- 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
What is the DDx for spur cells (acanthocytes)?
Liver failure Alcoholism Hypothyroidism Anorexia nervosa Congestive splenomegaly Abetalipoproteinaemia Chronic granulomatous disease
What is the DDx for burr cells (echinocytes)?
Uraemia Hypomagnesaemia Hypophosphataemia Pyruvate kinase deficiency Haemolytic anaemia
What are the complications of polycythaemia rubra vera?
Stroke Bleeding (acquired Von Willebrand's Disease) Erythromelalgia Gout Pruritus Diaphoresis
What are the examination findings in polycythaemia vera?
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)
Which investigations should be considered in polycythaemia rubra vera?
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
What are the treatment options in polycythaemia rubra vera?
Phlebotomy Aspirin for all Hydroxyurea IFN (younger or may become pregnant) Ruxolitinib (severe symptoms) Allopurinol (hyperuricaemia) Splenectomy
Which malignancies are associated with elevated ferritin?
Breat cancer
Pancreatic cancer
HCC
Haematological cancers
Which non-malignant conditions are associated with elevated ferritin?
HLH Still's Disease Sideroblastic anaemia Fatty liver disease Obesity EtOH excess
What are the favourable prognostic factors in multiple myeloma?
Albumin > 35 g/L Normal LDH β2-microglobulin < 3.5mg/L Absence of: - t(4;14) - t(14;16) - del(17p)
What is the pharmacological therapeutic ladder in multiple myeloma?
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
How are transplant-eligible patients treated?
- 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
How is Minimal Residual Disease (MRD) tested in multiple myeloma and why is it tested?
Next generation flow cytometry OR
Next generation sequencing
What supportive Rx is available in multiple myeloma?
- Osteoporosis: denosumab, zoledronic acid
- Cast nephropathy: proteasome inhibitors
What immunodeficiencies are associated with CLL?
Hypogammaglobulinaemia IgG subclass deficiency (IgG3 in particular)
What are the anticoagulant options in HITS?
- Fondaparinux or danaparoid or argatroban or rivaroxaban
- Argatroban in renal impairment
- Bivalirudin IV infusion if requiring urgent surgery
How is high risk myeloma defined?
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) ]
What are alternatives to CT or MRI for detecting myelomatous bony lesions?
Sestamibi (sensitivity 92%, specificity 96%)
PET (sensitivity 85%, specificity 92%)
What is the monitoring regime for MGUS?
- 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
What is the most common induction regime for mutiple myeloma in Australia?
CyBorD
- Cyclophosphamide
- Bortezomib (subcut)
- Dexamethasone
What supportive measures are available for localised bony myeloma lesions?
- Radiotherapy
- Internal fixation where fracture risk of long bones exists
- Balloon kyphplasty for painful compression fractures
- Bisphosphonates
Does VTE risk increase with thalidomide or lenalidomide?
Only when in combination with dexamethasone
When is recombinant Epo used in myeloma?
Those with renal failure and anaemia
Which myeloma patients are eligible for regular IVIG?
Recurrent infections (≥2 chest infections per year) and hypogammaglobulinaemia
What infection prophylaxis is recommended in myeloma?
- 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.
What is the prognostic value of the presence of myeloma biomarkers? What are the biomarkers?
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
What are the functions of immunomodulatory drugs and proteasome inihibitors in myeloma?
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
Which medications may interact with warfarin to increase bleeding risk?
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
Also dan shen, a herbal medicine
Which medications may interact with warfarin to increase clotting risk?
St John's Wort Infliximab Azathioprine/6-MP Dicloxacillin Aprepitant
Also green leafy vegetables, beetroot
