MD1TALK
Bleeding disorders
Hemostasis → Cessation of bleeding
Normal Hemostasis Requires P/O all following:
1) Normal Sub-endothelium (exposed after injury): normal collagen
and tissue factor
2) Normal Platelet and VonWillebrand: in number and function
3) Normal coagulation factors
Process of hemostasis after injured endothelium and exposed
Sub-endothelium:
1) VC: due to nerve reflex and ↓ secretion of NO *VD*
2) PLT plug: VWF secreted from injured sup endothelium →
adhere to injury → PLT adhere on VWP→
3) PLT activation + degranulation: change in shape*star* and
degranulate *Serotonin +Adenosine* to aggregate more PLT and
form cross link through GP2b/3a
4) activation of coagulation system via released tissue factor
→ fibrin mesh + PLT → clot
5) Fibrinolysis through
plasmin
, MD1TALK
Thrombosis risk: protein S, C and plasminogen , factor v liden ,
antithrombin 3 Deficiency
Values: no need to memorize
• PLT No.:150-450,000
• MPV: 7-9
• Average PLT life: 7-9d *thats why aspirin is stopped before surgery in
one weak due life span of PLT
• Thrombopiotin from kidney & liver → megakaryocytic regulator
• PBF:round shape and Flat disc
PLT function : Adhesion→ Activation→ Aggregation→ Activate coagulation
Immune thrombocytopenia purpura (ITP):
Age: Toddler age
Path: Autoimmune disease due auto-antibodies produced by
spleen directed to GP2b/3a → opsinization than destruction by spleen.
• Can be 1ry occurs after viral infection (due to M.M) or secondary.
Symptoms:
• present 1-3 weeks after viral URTI , with mucocutaneous bleeding
Petechia, Purpura and epistaxis
• No adenopathy or HSM
Lab:
1) CBC: ↓ PLT only
2) PBF: large PLT (↑MPV) due to compensatory ↑ in production in BM
3) Coagulation profile: ↑BT , normal PT & PTT
4) BM aspiration: no need only if you suspecting leukaemia
(pancytopenia with HSM)
Management:
• If PLT >30000 + reliable family → only observe
• If PLT <30000 → steroid
• PLT Rx according to clinical not PLT count → ex: unstoppable
bleeding → give PLT.
1- Steroid first line
2- IVIG
3- IV anti D (must be RH+) → abs toward RH → RBC make spleen
busy so platelet is spared
CI in : HA → if no res
4- Immunotherapy : Rituximab
5- Splenectomy last thing
Prognosis:
Bleeding disorders
Hemostasis → Cessation of bleeding
Normal Hemostasis Requires P/O all following:
1) Normal Sub-endothelium (exposed after injury): normal collagen
and tissue factor
2) Normal Platelet and VonWillebrand: in number and function
3) Normal coagulation factors
Process of hemostasis after injured endothelium and exposed
Sub-endothelium:
1) VC: due to nerve reflex and ↓ secretion of NO *VD*
2) PLT plug: VWF secreted from injured sup endothelium →
adhere to injury → PLT adhere on VWP→
3) PLT activation + degranulation: change in shape*star* and
degranulate *Serotonin +Adenosine* to aggregate more PLT and
form cross link through GP2b/3a
4) activation of coagulation system via released tissue factor
→ fibrin mesh + PLT → clot
5) Fibrinolysis through
plasmin
, MD1TALK
Thrombosis risk: protein S, C and plasminogen , factor v liden ,
antithrombin 3 Deficiency
Values: no need to memorize
• PLT No.:150-450,000
• MPV: 7-9
• Average PLT life: 7-9d *thats why aspirin is stopped before surgery in
one weak due life span of PLT
• Thrombopiotin from kidney & liver → megakaryocytic regulator
• PBF:round shape and Flat disc
PLT function : Adhesion→ Activation→ Aggregation→ Activate coagulation
Immune thrombocytopenia purpura (ITP):
Age: Toddler age
Path: Autoimmune disease due auto-antibodies produced by
spleen directed to GP2b/3a → opsinization than destruction by spleen.
• Can be 1ry occurs after viral infection (due to M.M) or secondary.
Symptoms:
• present 1-3 weeks after viral URTI , with mucocutaneous bleeding
Petechia, Purpura and epistaxis
• No adenopathy or HSM
Lab:
1) CBC: ↓ PLT only
2) PBF: large PLT (↑MPV) due to compensatory ↑ in production in BM
3) Coagulation profile: ↑BT , normal PT & PTT
4) BM aspiration: no need only if you suspecting leukaemia
(pancytopenia with HSM)
Management:
• If PLT >30000 + reliable family → only observe
• If PLT <30000 → steroid
• PLT Rx according to clinical not PLT count → ex: unstoppable
bleeding → give PLT.
1- Steroid first line
2- IVIG
3- IV anti D (must be RH+) → abs toward RH → RBC make spleen
busy so platelet is spared
CI in : HA → if no res
4- Immunotherapy : Rituximab
5- Splenectomy last thing
Prognosis:
