Hematology factoids

Question 1

How many base pairs in the human genome and how many are variable? How many genes?

Answer 1

3 billion base pairs with 4 million variable. 25000 genes.

Question 2

Baseline annual risk of VTE

Answer 2

0.2% per year

Question 3

20 year “incremental” risk for MAJOR bleeding in average patient on anticoagulants

Answer 3

16% (0.8% per year)

Question 4

Name 3 VTE recurrence predictor score tools

Answer 4

HERDOO2 (for women)
DASH
Vienna

Question 5

RR for VTE in FVL heterozygous and homozygous

Answer 5

3-8 fold in heterozygous

9-80 fold in homozygous

Question 6

Describe the VWD subtypes and inheritance

Answer 6

75% Type I - heterozygous mild decrease in antigen (AD)
Type III - homozygous severe decrease in antigen (AR)
2A - Reduced HMW multimers
2B - Increased affinity for GPIb (low platelets) (AD)
2M - Reduced affinity for GPIb
2N - Reduced FVIII binding capacity (AR)

Question 7

Which Granules in Gray Platelet Syndrome?

Answer 7

Alpha Granules

Large platelets seen

Question 8

What is in platelet alpha granules?

Answer 8

VWF, PF4, IGF-1, TGF-B, PDGF, others

Question 9

Bernard Soulier defect, clinical and inheritance?

Answer 9

GP1b, large platelets, low platelets

AR, 1 in a million

Question 10

Glanzmanns defect and consequence

Answer 10

GP IIb/IIIa

Platelets cannot bind VWF

Question 11

May hegglin defect and clinical

Answer 11

Large platelets
Low platelets
Inclusions in granulocytes only (dohle like)
MYH9 gene (probably)

Question 12

Which coag factors are membrane dependent?

Answer 12

TF
V
VIII
XI

Question 13

What does aspirin do to PFA?

Answer 13

Prolongs Coll/Epi, not Coll/ADP

Question 14

Ristocetin in platelets aggs for bernard soulier and glanzmanns?

Answer 14

Ristocetin agglutinates platelets via gp1b and VWF (both needed) so will be prolonged in Bernard soulier (and will be the only agg test normal in glanzmans)

Question 15

Describe the IPI criteria

Answer 15

No - >1 Extranodal site
S - Stage III or IV
L - LDH >normal
A - Age >60
P - Performance score 2 or more

Question 16

Describe the HANS criteria for DLBCL cell of origin

Answer 16

GEP is gold standard
Hans uses CD10, bcl6 and MUM1 immunohistochemistry
CD10 pos = GCB
CD10 neg, bcl6 pos, MUM1 neg = GCB
CD10 neg, bcl6 pos, MUM1 pos = non GCB
CD10 neg, bcl6 neg = non GCB

Question 17

Describe ECOG performance scores

Answer 17

0 - normal
1 - Light work, all self care
2 - All self care, unable to work. <50% bed
3 - Limited self care, unable to work, >50% in bed
4 - Bedbound, no self care

Question 18

Which DLBCL patients might be OK with 4 RCHOP +2R instead of 6 RCHOP?

Answer 18

[IPI] of 0;
age ≤ 60 years;
no bulky disease
-see German FLYER trial ASH abstract 781 (2018)