Hemophilia

Background

• Two types (clinically indistinguishable):
  • Hemophilia A: Factor VIII deficiency
  • Hemophilia B: Factor IX deficiency
• Substantial proportion of both types arise from spontaneous mutations
• ICH is most common cause of hemorrhagic death
• Never give NSAIDs or IM injections
• Consult hematology if pt has h/o inhibitors

Diagnosis

• Easy bruising or bleeding out of proportion to the history of trauma
• Recurrent bleeding into joints and muscles
  • Iliopsoas hemorrhage requires aggressive treatment (80-100% factor replacement)
• Hematuria
  • Common but typically not severe

Work-Up

• Coags
  • Only helpful for making the dx; once established unlikely to yield new information
  • PT - normal
  • PTT - abnormal (unless mild hemophilia)
• Head CT
  • If c/o HA, AMS, sig. flunt head injury
• CT A/P
  • Back, thigh, groin, or abd pain

Factor Replacement

• FFP if dx is unknown (contains VIII and IX)
  • Each bag raises factor levels by 3-5%
• Factor replacement if dx is known
  • Factor VIII required = (Target FVIII – Baseline FVIII)/2 x wt in kg
    • If baseline is unknown assume zero
    • After initial correction give half this dose q8-12hr
  • Factor IX required = (Target FIX – Baseline FIX) x wt in kg
    • After initial correction give half this dose 24 hr later

Specific Therapy

TYPE OF BLEEDING	INITIAL DOSAGE	DURATION	COMMENT
Skin
Abrasion	None	None	Treat with local pressure and topical thrombin
Laceration	Usually none; if necessary, treat as minor	None	Local pressure and anesthetic with epinephrine may benefit; watch 4 hours after suturing; reexamine in 24 hours
Superficial
Deep	Minor bleeding (12.5 mg/kg)	Single-dose coverage	May need hospitalization for observation; repeat may be necessary for suture removal
Nasal epistaxis
Spontaneous	Usually none; may need to be treated as mild bleeding	None	Uncommon; consider platelet inhibition; treat in usual manner
Traumatic	Moderate bleeding (25 mg/kg)	Up to 5–7 days	Trauma-related bleeding can be significant
Oral
Mucosa or tongue bites	Usually none; treat as minor if persists	Single dose	Commonly seen
Traumatic (laceration) or dental extraction	Moderate (25 U/kg) to severe (50 U/kg)	Single dose; may need more	Saliva rich in fibrin lytic activity; oral ε-aminocaproic acid (Amicar) may be given at 100 mg every 6 hr for 7 days to block fibrinolysis; check contraindications; hospitalize patients with severe bleeding
Soft tissue/muscle hematomas	Moderate (25 U/kg) to severe (50 U/kg)	2–5 days	May be complicated by local pressure on nerves or vessels (e.g., iliopsoas, forearm, calf)
Hemarthrosis
Early	Mild (12.5 U/kg)	Single dose	Treat as earliest symptom (pain); knee, elbow, ankle more common
Late or unresponsive cases of early hemarthrosis	Mild to moderate (25 U/kg)	3–4 days	Arthrocentesis rarely necessary and only with 50% level coverage; immobilization is critical point of therapy
Hematuria	Mild (12.5 U/kg)	2–3 days	Urokinase, the fibrinolytic enzyme, is in urine; with persistent hematuria an organic cause should be ruled out
Major bleeding	Major bleeding (50 U/kg)	7–10 days or 3–5 days after bleeding ceases	In head trauma, therapy should be given prophylactically; early CT scan of head recommended for all

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Source

Tintinalli, Rosens