haem

Question 1

myelodysplastic syndromes

Answer 1

< 20 blasts in plasma
30% leads to AML
more susceptible to infection

give antibiotics
blood transfusion w iron chelation

Question 2

AML

Answer 2

> 20 blasts
bone failure- anaemia, thrombocytopenia, infections
FBC, bone marrow aspirate- blast cells, blood film- auer rods
supportive care
chemo
remission- <5% blasts

Question 3

haemolytic disease of newborn

Answer 3

anti D prophylaxis at 28 weeks and 34 weeks
jaundice
neg mum pos baby
indirect coombs test
Positive Direct Antiglobulin Test (DAT)
Anaemia, Jaundice

Question 4

hyposplenism

Answer 4

howell jolly bodies
pitted erythrocytes
antibiotic prophylaxis
vaccinations

Question 5

G6PD defic

Answer 5

normocytic, intravascular haemolytic anaemia

low haptoglobin
high retic
raised

antimalarials
fava beans
infection

spherocytes
heinz bodies
bite/ blister cells

negtaive coombs

treat folic acid

Question 6

haemorrhagic disease newborn

Answer 6

vit K deficient
bulging fontanelle
prolonged PT AND APTT
caused by drugs in pregnancy
give vit K
Intracranial bleeding
Gastrointestinal bleeding
Post-circumcision bleeding
Umbilical stump bleeding

Question 7

thalassemia

Answer 7

most common- beta 1
autosomal recessive
hair on end appearance on CT

Question 8

clotting cascade

Answer 8

Xa they join
subendothelial contact is intrinsic
cellular damage is extrinsic
APTT is intrinsic
PT is extrinsic
Xa turns prothrombin into thrombin

Question 9

anaemia of chronic disease

Answer 9

normocytic or microcytic
cancers
COPD
infection
RA

low ferritin
high total binding iron

give iron 3 months- doesnt work then anaemia of chronic disease

treat underlying cause
EPO

Question 10

folate or B 12

Answer 10

IM B12 and folate
first B12 because paraesthesia gets worse if u give folate first

Question 11

MGUS

Answer 11

<9% of bone marrow is plasma one cell
>9% is myeloma

Question 12

haemophilia

Answer 12

bleeding after trauma
haemarthroma
big bulges

A- vonwillebronjames associated. 8. more common and more severe
B- 9

drainage

IV recombinant factor
give fresh frozen plasma
tranexamic acid

Question 13

lemon yellow tinge

Answer 13

b12 and folate
macrocytic
megaloblastic

Question 14

ALL

Answer 14

tumour marker CD19
blast cells on blood film
blast cells >20% of bm
neuropathic symptoms more than CML

chemo 4-6 weeks
methotrexate 18 months

Question 15

CLL

Answer 15

lymphocytes >5
B cells
smudge and smear cells
binet
watch and wait
chemo
rituximab- monoclonal

Question 16

treatment ET

Answer 16

aspirin
hydroxycarbamide

Question 17

PRV

Answer 17

extra cells
itchy in shower
venesection weekly
aspirin

Question 18

primary myelofibrosis

Answer 18

everything decreased
tear drop poikolyctes
dry tap aspirate

give epo
give platelets
give hydroxycarbamide

cure w allogeneic stem cells

Question 19

hereditary spherocytosis

Answer 19

normocytic haemolytic
spherocytes
anaemia
pigment gallstone
DAT/ coombes - pos. extravascular
autoimmune
autosomal recessive

folic acid
transfusion

aplastic
parvovirus B19

Question 20

treatment von willedbrand

Answer 20

Type 1: Tranexamic acid or Desmopressin (DDAVP) more please. stimulation

Type 2 and 3: Factor VIII and von Willebrand factor (vWF). theres is useless or they have none

type 1 quantitative
2 qualitative
3 none

OCP for menorrhagia

Question 21

sickle cell

Answer 21

autosomal recessive
HbS trait
HbSS is the disease
manage crisis conservatively
prophylaxis for malaria- proguanil
vaccines
hydroxycarbamide- get rid of bad cells
bone marrow transplant

but protective for malaria weirdly

Question 22

aplastic crisis

Answer 22

breathlessness
fatigue
unwell and random

Question 23

factor V leiden

Answer 23

autosomal dominant
too much clotting
protein C is like no stop but he doesnt listen to C he keeps clotting
recurrent miscarriage
shortened APTT

preventation anti coags
doacs

Question 24

heparin induced thrombocytopenia

Answer 24

lose 30% within 5-10 days

Question 25

TTP

Answer 25

hereditary or aquired anti adam fatigue purpura organ damage too many thrombi when u actually need it u dont have enough platelets stops the inactivation of VWD antibodies against adam ts 13 plasma aspirin heparin

Question 26

HUS

Answer 26

haemolysis thrombocytopenia AKI bloody diarrhoea abdo pain caused by gastroenteritis treat- monitor kidneys give saline avoid toxic drugs

Question 27

DIC

Answer 27

prolonged PT and APTT increased D dimer decreased fibrinogen thrombocytopenia schistocytes Microvascular thrombosis leading to tissue ischaemia Mucosal bleeding, poor healing and bleeding from wounds ulceration and gangrene formation fresh frozen plasma supportive treat underlying cause

Question 28

APLS

Answer 28

miscarriages recurrent ≥3 recurrent miscarriages Coagulation Screen (Partial Thromboplastin Time: aPTT) Anticardiolipin antibodies Control cardiovascular risk if had venous event lifelong Warfarin (INR 2-3) and consider a higher INR if its inadequate History of Recurrent miscarriage: Active Monitoring along with Low Molecular Weight Heparin (LMWH) and Aspirin throughout pregnancy Systemic Lupus and APLS: Hydroxychloroquine (thromboprotective)

Question 29

universal donor of platelets

Answer 29

AB universal recipient of other stuff

Question 30

myeloma is normally paraproteins for what

Answer 30

IgG and IgA waldenstrom IgM

Question 31

myeloma

Answer 31

chemo, steroids, bisphosphonates serum electrophoresis bone aspirate bence jones protein in urine

Question 32

neutropenic sepsis

Answer 32

rigor fever source of infection neutropenia

Question 33

ITP

Answer 33

caused by vaccinations children chicken pox CLL sick FBC low platelets purpura coagulopathy screen steroids first line IV immunoglobulins

Question 34

non hodgkins

Answer 34

not got reed sternberg not painful lymphoma mid life chemo and CD20 straight away burkitts- starry nighst sky on aspiration

Question 35

lymphoma risk factors

Answer 35

EBV HIV immunosuppression smoking ann arbour

Question 36

hodgkins

Answer 36

hurts when drinking alcohol reed sternberg chemo first then CD30

Question 37

haemochromatosis

Answer 37

recessive accumulation of iron in the body bronze pigmentation of the skin pearls prussian blue positive in liver defective hepcidin (he usually says im done in give me no iron) stop eating iron venesection iron chelation