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haematology > haem peer teaching > Flashcards

haem peer teaching Flashcards

1
Q

how would you categorise haemoglobinopathies?

and which is the most important

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

Sickle cell

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2
Q

describe sickle cell

A

AR inh

gene on chr11? check..

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3
Q

what is the normal lifespan of a sickle cell

A

20-30 days

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4
Q

describe the pathophys of sickle cell disease

A

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

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5
Q

presentation of sickle cell?

A

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

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6
Q

types of Sickle cell crisis?

A

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

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7
Q

how does hydroxycarbamide work?

A

increases proportion of HbF

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8
Q

management of priapism in sickle cell?

A 
hydration
02
analgesia
warm bath 
if still not sorted - aspirate and irrigate c. cavernosum
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9
Q

complications of sickle cell

A

chronic pain
stroke
AVN of fem head
OTHERs

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10
Q

thallassaemia beta - give some signs

A

anaemia

frontal bossing

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11
Q

describe other symptoms of pernsicious anaemia

late complication

A

parasthesia / glossitis / fatigue

can progress to partial spinal cord degenration

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12
Q

define haemolytic anaemia

b. signs

A

reduction in lifespan of RBC from normal of 120 days

b. signs of destruction
signs of increased production

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13
Q

causes of haemolytic anaemia:

b. commonest drug cause

A

b. cephalosporin ABx

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14
Q

what is the common Px of hered. spherocyt.

A

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

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15
Q

describe pathophys of G6PD

A

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

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16
Q

PRV
Def:
what is the treatment :

A

neopastic prolif of hemapoeitic cells in BM
Aspirin
Venesection

17
Q

What are the 3 biggest causes of massive splenomegaly.

A

CML
Myelofibrosis
Malaria

18
Q

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

A

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

19
Q

how do you measure the following clotting pathways:
intrinsic
extrinsic

A

aptt

pt

20
Q

what do you reverse warfarin immediately with

and is it used for noacs and doacs as well?

A

Beriplex

Yes

21
Q

DOACs

what inhibits what

A

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

22
Q

what are the treatment principles for ALL

A

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

23
Q

give some signs of pancytopenia

A

bruising
infection
mouth sores
anaemia

24
Q

what is important to remmeber about APML?

A

causes a coagulopathy - often the cause of death

25
Q

what is the ann-arbor classification

A

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

26
Q

2 most common types of NHL

A

diffuse large b cell

follicular

27
Q

what is the highest risk regime for neutropenic sepsis?

what is it indicated in

A

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

28
Q

diagnosis of DIC:

A 
2 up
d dimer 
PT 
2 down
platelets 
fibrinogen

nb - in subacute / chronic dic then PT or APTT may be normal

29
Q

presentation of DIC?

A

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

30
Q

management of DIC:

A

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

haematology (1 decks)

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