Haem

Question 1

Thrombophilia causes

*anticoagulation after 1 VTE

Answer 1

FvL/APC resistance homozygotes*
ATIII deficiency*
Protein C+S deficiency
Prothrombin mutation
Homocysteine
PNH
APS - lupus anticoagulant*, ACL*, B2 glycoprotein
All types should receive prophylaxis for surgery, long flights

Question 2

Causes of polycythaemia

Hct >60% in male, >56% in female

Answer 2

Primary - PRV
Secondary:
1. increased EPO - CKD, hepatoma, cerebellar haemangioma, uterine myoma, virilising syndromes, Cushings, phaeo
2. hypoxaemia - lung disease, HHT, OSA, CHD, CO poisoning, haemoglobinopathy (B thal)

Question 3

Ix for polycythaemia

Answer 3

PRV - JAK2, high Hb/Hct/RCC/WCC/Plt/neutrophil ALP
Splenomegaly
ABG
EPO (note haemangioblastoma, RCC, liver ca excrete EPO)
B12 (high in PRV due to transcobalamin I+III form neutrophils)
EUC
BM to exclude another MPD
*a/w GORD

Question 4

PRV Rx

Answer 4

Lower Hb to <140 male or <120 female
Phlebotomy
Hydroxyurea
IFNa for splenomegaly or severe pruritis
Allopurinol for urate
Low dose aspirin

Question 5

Myelofibrosis causes

Answer 5

1. Malignant:
   CML, MPD, leukaemia, lymphoma, MM, metastatic ca
2. Nonmalignant:
   SLE, HIV, TB, renal osteodystrophy, hyper PTH

Question 6

Myelofibrosis Rx

Answer 6

hydroxyurea for Sx (thrombocytosis, splenomegaly)
allopurinol for urate
ruxolitinib (JAK2 inhibitor) for splenomegaly
Curative: allogeneic BMT for young patients
-10% transform to leukaemia

Question 7

ET Rx

Answer 7

Treat symptomatically (neuro Sx, headache, erythromelalgia)

• aspirin, hydroxyurea, IFNa, anagrelide
• tranexamic acid for bleeding

Question 8

Hodgkins lymphoma types

Answer 8

Lymphocyte predominant
Nodular sclerosing
Mixed
Lymphocyte depleted

Question 9

NHL types

Answer 9

1. low grade - small lymphocytic, follicular mixed
2. int - follicular large cell, diffuse small cleaved cell, diffuse mixed, diffuse large cell
3. high-grade - large cell immunoblastic, lymphoblastic, Burkitts (*a/w AIDS)

Question 10

Hodgkins lymphoma Ann-Arbor staging

Answer 10

1 - single node/lymphatic site
2 - lymph node regions on 1 side of diaphragm +/- spleen
3 - lymph nodes on both sides of diaphragm +/- spleen
4 - diffuse disease involving organs
A or B-symptoms

Question 11

ECOG

Answer 11

0 - fully active
1 - restricted work
2 - unable to work, independent self-care
3 - in bed >50% of the time
4 - bedbound

Question 12

Hodgkins Rx

Answer 12

ABVD or BEACOPP +/- RTx
autologous SCT
*alkylating agents cause later risk of leukaemia

Question 13

NHL Rx

Answer 13

Treat high-grade with R-CHOP +/- MTX
autologous SCT

B-CLL - chloarmbucil or fludarabine + rituximab
MALT - chloarmbucil
Hairy cell - cladrabine
Follicular - RCVP or RCHOP

Question 14

MM Rx

Answer 14

Young - VCD (bortezomib, cyclophosphamide, dex)
Old - MTP (melphalan, thalidomide, pred) or VMP
SCT

Question 15

POEMS syndrome

Answer 15

polyneuropathy
organomegaly
endocrinopathy (prolactin, hypothyroid, T2DM)
monoclonal gammopathy
skin (clubbing, hypertrichosis, thickened skin, pigmentation)
-elevated VEGF in 2/3
Rx as myeloma

Question 16

Indications for allogeneic BMT

Answer 16

Malignant
- acute leukaemia, lymphoma, Hodgkins, MM
Nonmalignant
- AA, sickle cell, Thal, Gauchers, SCID, Fanconi anaemia

Question 17

Indications for autologous BMT

Answer 17

Malignant
-leukaemia, CML, lymphoma, hodgkins, , testicular ca, Wilms
Nonmalignant
-RA, MS