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0:52 Intro and table of contents

2:36 CASE 1- Infection, due surgery and a prolonged APTT ft. a refresher on APTT prolongation

08:00 Mixing studies- definition and uses ft. Hari’s exam nugget

17:00 Factor assays (1 stage, 2 stage and chromogenic assays) ft. David’s humorous humility

43:30 David applies his new-found knowledge to our first case

45:45 CASE 2- Infection, hx of weight loss and bleeding and a prolonged APTT

47:36 Summary

https://practical-haemostasis.com/Factor%20Assays/1_stage_pt_factor_assay.html

https://practical-haemostasis.com/Factor%20Assays/chromogenic_factor_assays.html

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00:00 Intro

04:25 Automated Methods of Measuring a Clot

05:50 Scenario 1 & Pre-Analytical Variables

11:50 HIL Index & Patient Factors

15:55 Blood Tube Basics

21:10 Nitty Gritties- What Happens When We Send a PT?

23:20 PT vs INR for Warfarin- Going Down The Rabbit Hole...

26:35 Heparin Neutralising Buffer

29:00 APTT

33:05 Summary (& an honorable mention)

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0:00 Intro

2:45 What is Haemostasis?

3:55 Stages of Haemostasis (summary)

4:45 Primary Haemostasis

8:35 Secondary Haemostasis

11:10 The Clotting Cascade

12:20 Common + Extrinsic Pathway

13:50 Intrinsic Pathway (TwelvEleveNinEight)

14:25 Clotting Tests

16:30 Hari Pops The Bubble

18:05 In-Vivo vs. In-Vitro

22:20 Isolated PT Prolongation- causes

25:46 Isolated APTT Prolongation- causes

27:43 Paired PT/APTT Prolongation- causes

28:50 Best Test for Bleeeding (David makes Hari proud)

30:24 Bleeding History Pearls and Questionnaires

33:55 When The PT/APTT Screen Doesn't Work

37:25 NICE Guidelines Reference for Surgery

38:15 David's Scenarios

42:00 Summary

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Polycythaemia- red cell #
Erythrocytosis – in red cell mass

Absolute Erythrocytosis
- M: Hct >0.60 or >0.52 + RCM >25% of mean
- F: Hct >0.56 or >0.48 + RCM >25% of mean

Apparent Erythrocytosis
- Men: Hct >0.52 + normal RCM
- Women: Hct >0.48 + normal RCM

Relative erythrocytosis
-Normal RCM + Reduced plasma volume (pathological dehydration)

M>F
Median >60yo

2' PRV: treat underlying cause +/- venesection (higher hct threshold)

Classification of Absolute:
EPO dependent
- Appropriate: High altitude, chronic hypoxia, localised hypoxia, congenital
- Inappropriate: Tumors, EPO doping, Testosterone replacement, diabetic meds
EPO independent:
- Acquired: Primary PRV (low EPO level, feedback)
- Congential Polycythemia= mutations in EPO receptors

Inv:
- Tumor Hunt
- Hx + Exam: ?True vs. Apparent
- FBC, U+E, LFTs, Ca2+
- Blood film
- Ferritin: low in 1’ PRV
- EPO
- Imaging
- NB: Normal Hct + High Red Cell # + Low MCV + Low ferritin –> Masked PRV
- Molecular Testing:
JAK2 (V617F)(96-97%)...SAMURAI JACK=BLOODY)
EXXON 12 (3%)
Del (13q), Del (20q), Del (1q), Tris. 8/9
- *SV thrombus 50% chance MPN
- BMBx: Tri-lineage myeloid expansion
- Familial screen for congenital(young)

Sx of primary PRV:
- Arterial*+ Venous clot (splanchnic*)
- Hyperviscosity sx
- Splenic sx
- Gout

Indications for urgent venesection...Hyperviscosity sx

BSH diagnostic:
JAK2 Pos
- Hct M >0.52, F > 0.48. Or RCM >25% above baseline OR Splanchnic vein thrombus
- JAK2 positive

JAK2 Neg= A1-4 + either ≥ 1 A or 2 B’s
A1: Hct M >0.60, F > 0.56. Or RCM >25% above baseline
A2: No JAK2
A3: No 2' cause
A4: BMBx pos
A5: Palpable splenomegaly
A5: Acq. genetics in BM cells
B’s: Plt >450, Neut >10 (>12.5 in smokers), Radiological splenomegaly, Low EPO

Congenital testing

Risk Stratification:
- Thrombi..
ECLAP study
High Risk:>65 + prev clots
Low Risk: <65 + no prev clot
- Malignant Transformation to MF (5-15% in 10 years), AML (2% at 10 year) markers:
Splenomegaly, LDH, HVAF burden >50% at diagnosis

Management:
- Lifestyle...CV factors decrease
- Aspirin +PPI for all (after confirmed)- decrease CV events 60% (ECLAP)
- Venesection first line (?isovolemic)- sx***
CYTO-PV trial: Hct aim <0.45, make iron def.
400-450ml off
Weekly -> 3-4x/year
- If previous clots: Lifelong anticoag (w/out aspirin)
- NB: if plt>1000 (acq. VWF) bleeding risk, 1st cytoreduce

Cytoreduction: (once confirmed primary PRV)
- High risk
- Progressive Hepatosplenomegaly
- Plts >1500
- WCC >15
- Constitutional Sx
- Poor tolerance of venesection

1st line: (OHC then/or IFN)
OHC
- Risk: Macrocytosis, ulcers, SCC, Malignant transformation
NB: pregnancy
Peg IFN-A
Young + fertile
Lowers HVAF
PROUD PV study (2020)
Continuation PV study (2022)
SE: flu like sx, AI disease (*thyroid), mood disturbances

2nd line
Rux: JAK2i (works for EXXON)
RESPONSE + RESPONSE 2 trial
MAJIC PV study
SE: immunosuppression, skin cancer, wean dont stop

Older
Busulfan: 1 dose (w monitoring) vs intermittent
Risk: leuk transformation, pneumonitis**

Pregnancy: inc. DVT
OHC not safe (stop 3 months prior)
IFN 1st
Aspirin
Uterine Doppler from 20wks of gestation?flow
LMWH 6 wks postpartum