4th Year Additions Flashcards
what is irradiated blood?
blood that has been treated with radiation to prevent transfusion associated graft versus host disease
who needs CMV negative blood?
pregnant women and foetus
granulocyte transfusions
what is anisocytosis?
variation in size of RBCs
what are target cells seen in?
iron deficiency anaemia
post-splenectomy
what are heinz bodies seen in?
G6PD deficiency
alpha thalassaemia
what are Howell-Jolly bodies seen in?
spleen problem
when does the reticulocyte increase?
haemolytic anaemia
acute blood loss
what are schistocytes?
fragments of RBCs and indicate damage in their journey through vessels
causes of schistocytes?
DIC HUS TTP MAHA haemolytic anaemia
what are sideroblasts seen in?
myelodysplastic syndrome
sideroblastic anaemia
what are smudge cells seen in?
CLL
what are spherocytes seen in?
hereditary spherocytosis
inherited haemolytic anaemias
hereditary spherocytosis hereditary elloptocytosis thalassaemia sickle cell anaemia G6PD deficiency
acquired haemolytic anaemias
AIHA
PNH
MAHA
prosthetic valve-related
how does tumour lysis syndrome cause AKI?
raised urate
what is MALT lymphoma associated with?
H. pylori
what is smouldering myeloma?
progression of MGUS
investigations for myeloma
BLIP
- BJP
- light chain assay
- immunoglobulins in serum
- protein electrophoresis of serum
what does thalidomide increase the risk of?
VTE
what is TTP (thrombotic thrombocytopenia purpura)?
small blood clots form throughout the body causing thrombocytopenia
what causes clot formation in TTP?
problem with protein ADAMTS13
RFs for development of TTP
obesity
pregnancy
management of TTP
plasma exchange with FFP
what is heparin-induced thrombocytopenia (HITT)?
development of Ab against platelets in response to exposure to heparin
target PF4
management of HITT
use different anticoagulant
anticoagulation choice in pregnancy?
LMWHs
if recurrent PEs consider IVC filter
female iron requirements daily
1.5mg/day
male iron requirements daily
1mg/day
pregnancy iron requirements daily
7.5mg/day
who usually gets Gaucher’s disease?
Jewish inheritance
genetic inheritance of Gaucher’s disease?
AR
what is Gaucher’s disease?
glucocerebrosidase accumulates and lipids cannot be broken down leading to deposits in organs
presentation of Gaucher’s disease
skeletal
blood
abdominal complaints
management of Gaucher’s disease
enzyme replacement therapy of GBA enzyme
who get’s Tay-Sachs disease?
Jewish people
inheritance of Tay-Sachs disease?
AR
what is Tay-Sachs disease?
genetic defect in hexosaminidase A gene which causes a deficit beta-hexosamindase A leading to accumulation of fatty deposits that damage brain and spinal cord
presentation of Tay Sachs
6 months with loss of vision and hearing, dysphagia, muscle weakness
three forms of Tay Sachs
infantile
juvenile
late onset/adult
management of Tay Sachs
supportive + manage complications
what is the inheritance pattern of Niemann-Pick disease?
AR
what is Niemann-Pick disease?
abnormal sphinogmyelin degradation leading to cells being unable to metabolise cholesterol and lipids
presentation of Niemann-Pick disease
contraction
difficulty walking
sleep disturbance
management of Niemann-Pick
measure blood and skin biopsies to assess levels
antibody in warm AIHA?
IgG
antibody in cold AIHA
IgM
types of blood transfusion reactions
- non-haemolytic febrile reaction
- minor allergic reaction
- anaphylaxis
- acute haemolytic reaction
- transfusion-associated circulatory overload (TACO)
- transfusion-related acute lung injury (TRALI)
what causes a non-haemolytic febrile reaction?
antibodies reacting with white cell fragments in the blood product and cytokines that have leaked from blood cells during storage??
presentation of non-haemolytic febrile reaction
fever
chills
management of non-haemolytic febrile reaction
slow or stop transfusion
paracetamol
monitor
presentation of minor allergic reaction
pruritus
urticaria
management of minor allergic reaction
temporarily stop transfusion
antihistamine
monitor
management of anaphylaxis
stop transfusion
IM adrenaline
ABC support - oxygen + fluids
what causes an acute haemolytic reaction?
ABO incompatibility
presentation of acute haemolytic reaction
fever
abdo pain
hypotension
management of acute haemolytic reaction
check blood, patients blood and send for DAT testing, repeat crossmatching
fluid resuscitation
what causes transfusion-associated circulatory overload? (TACO)
excessive rate of transfusion
pre-existing heart failure
presentation of TACO
pulmonary oedema
hypertension
TACO management
slow or stop transfusion
consider IV loop diuretics (furosemide)
oxygen
what causes transfusion-related acute lung injury (TRALI)?
non-cardiogenic pulmonary oedema thought to be secondary to increased vascular permeability by host neutrophils activated by substances in donated blood
presentation of TRALI
hypoxia
pulmonary infiltrates on CXR
fever
hypotension
management of TRALI
stop transfusion
oxygen
supportive care
what does cryoprecipitate contain?
factor VIII:C von willebrand factor fibrinogen factor XIII fibronectin
what is the preferred anticoagulant in renal impairment?
apixaban
can Coeliac disease cause hyposplenism?
yes
what does raised LDH indicate?
haemolysis
transfusion thresholds
Hb <70g/L without ACS
Hb <80g/L with ACS
myeloma management pathway
suitable for transplant= brotezomib + dex
unsuitable= thalidomide + alklayting agent + dex
chemotherapy before stem cell transplant
what is used to prevent relapses in myeloma?
bortezomib
genetic mutation in acute promyelocytic leukaemia
t(15;17)
what is seen on blood film in acute promyelocytic leukaemia
auer rods
what genetic change is in mantle cell lymphoma?
t(11;14)
VTE management if Wells score 0-1
check d-dimer
if negative consider an alternative diagnosis
if positive get scan within 4 hours
d-dimer high but scan negative VTE management
stop interim anticoagulation and repeat 6-8 days later
treatment time length for VTE?
3 months provoked
6 months unprovoked
criteria for platelet transfusion
<10 x 10^9 no bleeding
<30 x10^ 9 minor bleeding
<100 x 10^9 in severe/ critical site
presentation of acute intermittent porphyria
red urine on standing
neuro, abdo, etc
what is Wiskott-Aldrich syndrome?
defect in WASP gene
x-linked recessive
low IgM
types of Hodgkin’s lymphoma
nodular sclerosing (most common - lacunar cells) mixed cellularity lymphocyte predominant (best prognosis) lymphocyte depleted (worst prognosis)
what is the most common type of lymphoma?
large dffuse B cell lymphoma
what is on microscopy in Burtkitt’s?
starry sky
when in hydroxyurea used in sickle cell?
prevent further crisis
what can packed red cells increase?
k+
drug causes of G6PD deficiency
sulph drugs - sulphonamides, sulphasalazine and SUs
malaria prophylaxis
what reverses rivaroxaban and apixaban?
andexanet alfa
