Haem - step 2 Flashcards
(63 cards)
apart from vWF what other factor is abnormal in von willebrands disease
factor VIII
management of follicular lymphoma
conservative with close follow-up
management of CLL
is asymptomatic - watch and wait
if asymptomatic - bruton tyrosine kinase inhibitors
deficiency of what co-factor is found in vW disease
ristocetin
1st line treatment for symptomatiic von willebrands
desmopressin (promotes release of vW from endothelial cells)
risk factors for AML
male sex
age > 60yrs
prior chemotherapy or radiotherapy
down syndrome
smoking
antidote for tissue plasminogen actiavtor (TPa) toxicity
aminocaproic acid
tranexamic acid
FFP
if someone develops heparin induced thromocytopenia, what alternative is often used
direct throbin inhibitor - Argatroban
antidote for DOAC
andexanet alfa for factor Xa inhibitor
idacrizumab for dabigatran
platelet count levels indicative of platelet replacement
plt < 10,000 if not bleeding
plt < 20,000 if bleeding
plt < 50,000 prior to procedure
1 bag of plt increases plt by 6000
inheritance of haemophillia
haemophillia A - factor VIII deficiency (most common) X linked recessive
haemophillia B - factor IX deficiency - x linked recessive
haemophillia C - factor XI deficiency - autosomal recessive. most common in jews.
best initial and most accurate tests for haemophillia
best initial: mixing study (mix patients plasma with normal plasma which will correct deficiency).
- if it doesnt correct then its an autoimmune/accquired haemophilia due to the presence of antibodies that will target the normal plasma factors
most accurate: specific factor assays (VII)
what is the blood product of choice if severe bleeding in haemophilia and the specific factor is not available
cryoprecipitate
more concentrated source of factor VIII and fibronectin compared to FFP
what is Heyde syndrome
multi-system disorder characterised by aortic stenosis, GI bleeding and acquired von willebrands disease
vWd occurs from the increased circulatory shear forces and subsequent cleavage and loss of vW factor
what medication can worsen von willebrands
aspirin
what type of von willebrands doesnt respond to desmopressin and how is it treated
type 2 (qualitative defects in vW factor)
treat with replacement therapy of vW factor and factor VIII
inheritance pattern of von willebrands disease
type 1 and 2 - autosomal dominant
type 3 - autosomal recessive
how can liver cirrhosis can a hypercoaguable state
liver is responsible for the synthesis of protein C and S
protein C and S deficiency = hypercoaguable
APC resistance test
activated protein C resistance test
used for diagnosing factor V leiden
point mutation in factor V renders it resistant to being activated/broken down by activated protein C
patient is started on dalteparin due to immobility as an inpatient. 5 days later develops skin necrosis at the site of infusion and has now a sore swollen calf. ?diagnosis ?treatment
heparin induced thrombocytopenia II
(HIT type 2)
autoimmune reaction against platelet factor 4 antibody against heparin-prostaglandin 4 antigen
symptoms occur 5-10 days after starting heparin
rarely causes bleeding. usually causes PE/DVT’s
manage with discontinuing heparin and start a non-heparin anticoagulant
best initial and most accurate tests for heparin induced thromboytopenia
best initial: evidence of HIT platelet factor 4 antibody
accurate: functional assay with serotonin release assay
APTT and PT in antiphospholipid syndrome
normal PT but paradoxical prolonged PTT
PTT doesnt correct with mixing study due to presence of antibodies
heparin induced thrombocytopenia type I vs type II presentation
type I occurs 1-2 days after starting heparin, smaller decrease in plt, usually mild
type II occurs 5-10 days after starting, more profound drop in plt, can cause skin necrosis and thrombosis. due to platelet factor 4 antibody against heparin prostaglandin 4
liver disease and DIC have similar lab values. how can it be differentiated?
DIC will have a low factor VIII level
