haem peer teaching

Question 1

how would you categorise haemoglobinopathies?

and which is the most important

Answer 1

1. genes affecting wuality and structure of Hb
2. genes affecting quantities

Sickle cell

Question 2

describe sickle cell

Answer 2

AR inh

gene on chr11? check..

Question 3

what is the normal lifespan of a sickle cell

Answer 3

20-30 days

Question 4

describe the pathophys of sickle cell disease

Answer 4

destroyed quicker
marrow tries to produce more RBCs to replace
hence increased retics
but doesn’t compensate enough so still anaemia..

Question 5

presentation of sickle cell?

Answer 5

6-12 months of age
anemia / splenomegaly
increased risk of overwhelming infection with encapsulated bacteria eg. pneumococcal disease
crises

Question 6

types of Sickle cell crisis?

Answer 6

vaso-occlusive (lots of Hb becomes deox at once) - severe pain from O2 depr of tissues and AVN of bone marrow

aplastic crisis - Parvovirus B19

sequesteration crisis

Question 7

how does hydroxycarbamide work?

Answer 7

increases proportion of HbF

Question 8

management of priapism in sickle cell?

Answer 8

hydration
02
analgesia
warm bath 
if still not sorted - aspirate and irrigate c. cavernosum

Question 9

complications of sickle cell

Answer 9

chronic pain
stroke
AVN of fem head
OTHERs

Question 10

thallassaemia beta - give some signs

Answer 10

anaemia

frontal bossing

Question 11

describe other symptoms of pernsicious anaemia

late complication

Answer 11

parasthesia / glossitis / fatigue

can progress to partial spinal cord degenration

Question 12

define haemolytic anaemia

b. signs

Answer 12

reduction in lifespan of RBC from normal of 120 days

b. signs of destruction
signs of increased production

Question 13

causes of haemolytic anaemia:

b. commonest drug cause

Answer 13

b. cephalosporin ABx

Question 14

what is the common Px of hered. spherocyt.

Answer 14

anemia in childhood, jaundic and splenomeg, parvovirus, pigment gallstones

Question 15

describe pathophys of G6PD

Answer 15

g6pd pathway maintains levels of nadph
maintains levels of glutathione
glutathione protects RBC from oxidative stress
(decreased g6pd activity = ox stress in RBC)

Question 16

PRV
Def:
what is the treatment :

Answer 16

neopastic prolif of hemapoeitic cells in BM
Aspirin
Venesection

Question 17

What are the 3 biggest causes of massive splenomegaly.

Answer 17

CML
Myelofibrosis
Malaria

Question 18

TTP
a. what is the enzyme def? - and why does this lead to problems with increased clotting?

**go over Mx briefly and presentation - acute and serious

Answer 18

a. ADAMTS13 - usually cleaves and gets rid of VWF - so more VWF knocking about - loads of clotting

Question 19

how do you measure the following clotting pathways:
intrinsic
extrinsic

Answer 19

aptt

pt

Question 20

what do you reverse warfarin immediately with

and is it used for noacs and doacs as well?

Answer 20

Beriplex

Yes

Question 21

DOACs

what inhibits what

Answer 21

RivaroXAban - Xa inhi
ApiXAban - Xa inh
dabigatran - direct thrombin inhibitor

Question 22

what are the treatment principles for ALL

Answer 22

induction of remision
consolidation of remission
maintenance for around 3 years
CNS proph with intrathecal chemo

Question 23

give some signs of pancytopenia

Answer 23

bruising
infection
mouth sores
anaemia

Question 24

what is important to remmeber about APML?

Answer 24

causes a coagulopathy - often the cause of death

Question 25

what is the ann-arbor classification

Answer 25

h.lymphoma stages 1-2-3-4 A = absence of b symptoms B = fever / nt sweats /wt loss

Question 26

2 most common types of NHL

Answer 26

diffuse large b cell | follicular

Question 27

what is the highest risk regime for neutropenic sepsis? | what is it indicated in

Answer 27

R-CHOP given for DLBC HNL just think - mix of pred and loads of chemo drugs - clearly a bit risky

Question 28

diagnosis of DIC:

Answer 28

``` 2 up d dimer PT 2 down platelets fibrinogen ``` nb - in subacute / chronic dic then PT or APTT may be normal

Question 29

presentation of DIC?

Answer 29

bleeding from 3 unrelated sites is typical - likely sites include: ears / nose / throat resp tract site of peripheral cannula / venepuncture

Question 30

management of DIC:

Answer 30

TREAT THE UNDERLYING CONDITION in many cases - dic will resolve following treatment of the underlying disorder Best supportive care In bleeding patients with a prolonged PT and APTT (ext-PT and Int-APTT) FFP may be used If severe hypofibronogenaemia persists despite FFP - cryoprecipitate may be indicated