Bleeding Disorders Flashcards
What is important to ask in history of bleeding disorder
HPC Easy bruising / how long to disappear Epistaxis - normal = <15 minutes Post surgical - dental / tonsils / vaccinations Menorrhagia PPH Post-trauma Any joint pain / limp
PMH
Drug history
Symptoms of anaemia
Systemic Sx - B symptoms / recent infection
FH of bleeding disorder / heavy bleeding / menorrhagia
FH of autoimmune
What is platelet type bleeding / vWF
Mucosal Epistaxis Purpura Petechiae Menorrhagia GI bleeding Retinal haemorrhage Ecchymosis Easily bruising
What is coagulation factor type bleeding
Bleeding into articular joints
Unprovoked muscle haematoma
Brain haemorrhage
How does muscle haematoma present
Swollen hot leg
What is important in Hx to determine if congenital or acquired / type of inheritance
Previous episodes Age of first event Previous challenges with surgery FH Sex
What is haemorrhagic diathesis
Unusualy susceptibility to bleed
What causes
Inhibtion of function of
Platelets
vWF
Coagulation factor
What is haemophilia
X-linked disorder causing coagulation factor deficiency
A = factor 8 (VII) deficiency = most common
B = factor 9 (IX)
What are the clinical features of haemophilia
Risk of severe bleeding in response to minor trauma or spontaneous Haemarthrosis - bleed into joint Muscle haematoma - calf + biceps CNS bleeding Retroperitoneal bleeding Post surgical bleeding
What are the complications of haemophilia
Synovitis Destruction of cartilage Chronic haemophilic arthropathy Neurovascular compression + compartment syndrome Haemorrhagic stroke
How and when does haemophilia present
6 months - 2 years when starting to walk Fall over Stop walking as bleed into knee Bruising Swollen painful leg
How do you Dx haemophilia
Reduced factor 8 or 9
Prolonged APTT
Normal PT
Genetic test but unlikely to have FH
How do you treat haemophilia bleeding
Coagulation factor replacement
DON’t transfuse FFP unless no concentrate factor
DDAVP - releases stored factor 8 + VWf
Tranexamic acid if minor bleed
Recombinant factor if major bleed
Gene therapy
Prophylaxis if severe
When do you give prophylaxis factor concentrate
Severe haemophilia to keep level of factor >2% so don’t bleed spontaneously
Contine for 45 weeks of the year
How do you manage haemophilia
Splints Physio ANalgesia Synovectomy Joint replacement Give vaccines SC instead of IM
What are the complications of treatment
Viral infection - HIV, HBV, HCV, CJD
Development of inhibitors to factor
What are the CI of DDAVP
Elderly as stroke / MI risk
<3 as hyponatraemia / fits
What does severity depend on
Amount of residual coagulation factor in the circulation
What is severe
<1%
Will bleeding spontaneously into muscles and joints every few weeks
What is moderate
1-5%
DONT bleed spontaneous
What is mild
5-30%
Don’t come to harm but may need procedures in place for surgical procedures
What is vWF
AD defect in vWF leading to platelet type bleeding (factor VIII in clotting cascade)
What are the types of vWF
Type 1 = quantitative - don’t make enough
Type 2 = qualitative - abnormal function
Type 3 = severe complete deficiency RARE
How do you Dx and Rx and what should you check
What vaccine
Dx = low levels of vWF in blood
Check blood group as O blood has low levels of vWF
Rx
Tranexamic acid if mild e.g. menorrhagia
DDAVP raises level of vWF by stimulating release = 1st line
vWF concentrate / factor VII for minority if severe
Immunisation against hep A and B
Iron if deficient
What are acquired causes of bleeding
Thrombocytopenia
Liver and renal failure
DIC
Anti-coagulants + platelets
What causes severe thrombocytopenia due to increased destruction
ITP
DIC
TTP
Malignancy
What leads to decreased production of platelet
Marrow failure - leukaemia / myeloma / myelodisplastic Sepsis B12 / folic acid deficiency Liver failure Cytotoxic drugs / RT Aplastic anaemia SLE Anti-phospholipd syndrome
What causes increased consumption
ITP / TTP / heparin induced DIC HUS Viral infection - EBV / HIV / hepatitis Pregnancy B12 deficiency Alcohol Drugs Hypersplenism
How does hypersplenism cause low platelets
Platelet sticks to big spleen
What is ITP
Immune mediated reduction in platelet count
Type 2 hypersensitivity where Ab destroy platelet
Isolated blood thrombocytopenia
What is associated with ITP
Common after viral infection / vaccinations Collagenosis Lymphoma / CLL Drug induced Can be idiopathic
What type of ITP do you get
Acute
Chronic relapsing remitting
Who tends to get acute
More common in children following infection or vaccination
Usually self limiting
How does it present and what are complications
Isolated blood thrombocytopenia
Platelet type bleeding - petehiae/ bruising
Well in themselves
Normal APTT
Chronic ITP
Anaemia
Haemorrhage / GI Bleed
How do you Dx
History PROCESS OF EXCLUSION FBC - thrombocytopenia Do blood film to make sure its okay / exclude ALL (tests at bottom) Must exclude heparin induced Rarely look at marrow
How do you Rx
Usually self limiting
None if no active bleeding
Advise against contact sport / IM injection / anti-coagulant / NSAID
Do repeat bloods to check resolved
If bleeding / severe thrombocytopenia <10
Steroids + PPI
IV IgG
May need transfusion but doesn’t work as Ab still destroy - only give if severe bleeding
Other if unresponsive Splenectomy Ritixumab - Thrombopoetin analogue Azahthioprine - 1st line in pregnancy
What is Evan’s syndrome
ITP + autoimmune haemolytic anaemia
How does liver cause bleeding disorder
Liver produces clotting factors and fibrinogen
What are lab results in liver failure
Prolonged PT + APTT
Reduced fibirnogen
Abnormal LFT
Where do most bleeding come from
Structural lesions e.g. varices
May have other features of liver failure
What is associated
CHolestasis
Vit K dependent factor deficiency
How do you Rx
Replace FFP
Give vit K
What causes haemorrhagic disease of the new born
Immature coagulation system
Vit K deficient diet
What does it lead too
Fatal haemorrhage
How do you Rx
Give IM vit K at birth
What causes acquire haemophilia
Autoimmune
Elderly
Malignancy
Pregnancy
How do you Dx and Rx
Raised APTT
Steroids
CI to platelet
Chronic bone marrow failure
TTP
Autoimmune thrombocytopenia
What is heparin induced thrombocytopenia
Development of Ab to platelet due to exposure to heparin
Usually 5-10 days after
Causes a hyper coagulable state and can cause thrombosis even though low platelet
Also break down platelet = thrombycotpenia
So you get a patient on heparin with low platelet forming blood clots
How do you Dx
HIT Ab
How do you Rx
Stop heparin
What are other causes of drug induced thrombocytopenia
Quinine Abiximab NSAID Diuretic - furosemide Ax - penicillin, sulphonamide, rifampicin AED - carbamazepine + valproate
What should you do for petechiae / non-blanching rash
Urgent FBC if no definite cause known e.g. viral infection / changes to medication / PMH
Does normal WBC rule out infection
No because infection stimulates recruitment but also uses up WBC so can be normal
What investigations after blood shows thrombocytopenia
Blood film - malignancy / b12 / folate / haemolytic
Reticulocyte count
- If high = haemolytis / bleed
- If low = marrow failure
U+E - HUS / TTP / DIC can affect renal
Clotting screen - synthetic function of liver and DIC
B12 / folate - can cause cytopenia’s
How could pancreatic mass cause prolonged PT
Vit K deficeincy due to biliary obstruction
