Symptoms

• Ecchymoses

• Hemarthrosis—bleeding into joint spaces* (especially knee, elbow, and ankle)

• Joint pain, swelling and erythema

• Cutaneous warmth

• Decreased range of motion

• Muscle hemorrhage

• Pain with motion of affected muscle

• Signs of nerve compression

• Potential life-threatening blood loss, especially with thigh bleeding

• Mouth bleeding with dental extractions or trauma

• Genitourinary bleeding

• Intracranial hemorrhage (spontaneous or following trauma), with headache, vomiting, change in mental status, and focal neurologic signs

• Excessive bleeding with surgery

*Hallmark of hemophilia; recurrent inadequately managed hemarthrosis leads to deformity and chronic pain.

Table 67-1 Laboratory and Clinical Manifestations of Hemophilia

■:i-v !■■<.- (LtliThih

Madera«

Mild (Mart Than

0.01 lUftnlT

(0.01-0.0 J lU/mU1

O.Oi lUftnl)

Age at on !d

1 VtMi or lew

2 ytjn oJuH

Neorurjl lymplomi

PC&

Usual

Usual

Rare

KH

pcnfloral

Uncommon

Bait

Muscle^oim hemoirhage

Spontaneous

Minor trauma

Minor Uauma

tNS hcmcohJQ?

High risi

HodHjfelltll

Raw

Postsurgical hemorrhage fwlthoul

Frank bleeding, severe

Wound bleed rig.

Wound bleeding, with factor

common

lesi thjnos imii/mL

Oral hemooluge Following trauma.

Usual

Com men

Common

tooth ex1i«lion

CNB, [.onlr-al ntTvoui vyilpi"; ICH inliatrJuiul liemorrlugL1; PCS, poitdrcumtisiundl b4<?Kliny.

ftoimal range of fac Dor VM/K act^Hy levet ls.OS-1.5 lU/mL (SO-ISOitOL 1 lU/mL eoriesporxit to 100% of the factor found Jn I mL of normal plHIft

CNB, [.onlr-al ntTvoui vyilpi"; ICH inliatrJuiul liemorrlugL1; PCS, poitdrcumtisiundl b4<?Kliny.

ftoimal range of fac Dor VM/K act^Hy levet ls.OS-1.5 lU/mL (SO-ISOitOL 1 lU/mL eoriesporxit to 100% of the factor found Jn I mL of normal plHIft

"O.OI UrtnL - OOOOOl uni(/l: 0.05 ILVmL DJ00005 unir/1; 0.5 HJI/hiL. = QQWfi unilA; 1 unil/hiL = I ILVmL = OJMI unit/I: IU/mL 0JB015 unirA

Treatment

Desired Outcomes

Currently, there is no cure for hemophilia A or B. The life expectancy of hemophiliacs was only 8 to 11 years in the 1920s and 1930s. With the development of effective treatment strategies, life expectancy is currently about 65 years, or nearly that of the normal population.

The short-term goals of hemophilia treatment are to:

• Decrease the number of bleeding episodes per year or bleeding frequency

• Normalize or improve clotting factor concentrate levels The long-term goals of hemophilia treatment are to:

• Maintain clinical joint function

• Normalize orthopedic joint score

• Normalize radiologic joint score

• Maintain quality-of-life measurements

General Approach to Treatment

O Intravenous factor replacement with recombinant or plasma-derived products to treat or prevent bleeding is the primary treatment of hemophilia. Primary prophylaxis is defined as the regular administration of factor concentrates with the intention of preventing joint bleeds.5 The rationale for primary prophylaxis is that individuals with factor levels of greater than 0.02 IU/mL (0.00002 unit/L) rarely suffer from spontaneous bleeds and arthropathy. Therefore, to maintain a trough level above this might convert "severe" hemophilia to "moderate" disease, with the abolition of joint bleeds and the associated arthropathy.6

Although primary prophylaxis is expensive, historical cohorts show progressively better outcomes (joint function and radiologic appearances) with its use. The Medical and Scientific Advisory Council of the National Hemophilia Foundation of the United States recommends primary prophylaxis in patients with severe hemophilia A and B (factor VIII or factor IX less than 1%). The optimal duration of prophylactic therapy is unknown.7, 8 Vaccination against hepatitis A and B is recommended in all hemophiliacs.

Nonpharmacologic Therapy Surgery

Surgical arthroscopic synovectomy reduces replacement-therapy-resistant disease and repetitive hemarthrosis of a single joint. This procedure removes inflamed joint tissue. Patients may have decreased range of motion after the surgery.

Orthotics

Joint prostheses do not deal with the deformities directly. Orthotics in hemophilia serve as an important supportive measure before or after surgery.

Pharmacologic Therapy

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