HAEM/ONC
BLEEDING DISORDERS
PromptList clotting disorders (things that make you clot)
ResponseInherited
Factor V leiden
Prothromin gene mutation (G20210A to be precise)
Protein C or Protein S deficiency
Hyperhomocystineaemia
Sticky platelet syndrome
Acquired
Antiphospholipid syndrome (Autoimmune condition attacking phospholipids)
DIC
Factor V leiden
Prothromin gene mutation (G20210A to be precise)
Protein C or Protein S deficiency
Hyperhomocystineaemia
Sticky platelet syndrome
Acquired
Antiphospholipid syndrome (Autoimmune condition attacking phospholipids)
DIC
PromptList bleeding disorders (that you bleed more)
ResponseInherited
Haemophilia A factor VIII, B Factor Ix, C Factor XI
Von Willebrand disease
Heridatroy haemorrhagic telangectasia (HHT)
Acquired
DIC
Liver disease
Vit K deficiency
Von willebrand disease (usually inherited though)
Factor deficiencies
Haemophilia A factor VIII, B Factor Ix, C Factor XI
Von Willebrand disease
Heridatroy haemorrhagic telangectasia (HHT)
Acquired
DIC
Liver disease
Vit K deficiency
Von willebrand disease (usually inherited though)
Factor deficiencies
PromptHaemophilia
ResponseHaemophilia: Either of two hereditary disorders in which the blood clots very slowly, due to a deficiency of either of two coagulation factors. Haemophila A due to deficiency of factor VIII, or haemophilia B due to deficiency of factor Ix. The patient may experience prolonged bleeding folowing any injury or wound, and in severe cases ther is spontaneous bleeding into muscles and joints. Bleeding in haemophilia may be treated by recombinant DNA derived factor VIII or plasma factor VIII concentrate. Alternatively concentrated preparations of factor VIII or factor Ix, obtained by freezing fresh plasma, may be administered (Cryoprecipitate). Haemophilia is controlled by a sex linked gene which means that it is almost exclusively restricted to males. Women can cary the gene - and pass it on to their sons - without being affected themselves. The genes encoding factors VIII and IX have been used in gene therapy trials for haemophilia.- Oxford med dictionary
PromptImportant historic features of haemophilia
ResponseHome management plan - has patient already administed factor replacement for this episode - guides ongoing factor replacement
Prophylaxis - guides ongoing factor replacement
Severity of haemophilia - guides urgency of factor replacement, predicted clinical course
Patient weight - for factor replacement dosing
Central access - Preferred IV access for patient
Prophylaxis - guides ongoing factor replacement
Severity of haemophilia - guides urgency of factor replacement, predicted clinical course
Patient weight - for factor replacement dosing
Central access - Preferred IV access for patient
PromptList common locations of bleeding in haemophiliacs following trauma
ResponseCNS/Head trauma
Deep muscle haematoma
Traumatic haemarthrosis
Intra Abdominal visceral injury
Compartment syndrome
Epistaxis
Haematuria
Intra-abdominal bleeding.
Deep muscle haematoma
Traumatic haemarthrosis
Intra Abdominal visceral injury
Compartment syndrome
Epistaxis
Haematuria
Intra-abdominal bleeding.
PromptManagement of bleeding in haemophilia
ResponseHaemophila A: Recominant factor VIII replacement (50 U/kg trauma, 75 U/kg CNS/TBI)
Haemophilia B: Recombinant Factor IX (75 U/kg trauma, 125 U/kg CNS/TBI)
Urgent discussion with haematologist
Treat injury if present (+/- retrieve to trauma/neurosurgical centre)
Haemophilia B: Recombinant Factor IX (75 U/kg trauma, 125 U/kg CNS/TBI)
Urgent discussion with haematologist
Treat injury if present (+/- retrieve to trauma/neurosurgical centre)
- Doses from RCH, other guidlines hard to find