Traumatic Hemarthrosis of the Knee Secondary to Hemophilia A in a Collegiate Soccer Player: A Case Report

Abstract

Objective: To present the case of a collegiate soccer player who suffered from a traumatic knee hemarthrosis secondary to hemophilia A. This case presents an opportunity to discuss the participation status of athletes with hemophilia.

Background: Hemophilia is a hereditary blood disease characterized by impaired coagulability of the blood. Hemophilia A is the most common of the severe, inherited bleeding disorders. This type, also called classic hemophilia, is due to a deficiency of clotting factor VIII. The athlete with hemophilia A reported pain and loss of function of his knee during a soccer game despite the absence of injury.

Differential Diagnosis: Anterior cruciate ligament tear, intra-articular fracture, meniscus tear, capsular tear, hemarthrosis.

Treatment: After the injury, the athlete was admitted to the hospital, where his knee joint was aspirated and he was infused with factor VIII. Later, he participated in traditional knee rehabilitation and was returned to play at the discretion of the orthopaedist and the hematologist.

Uniqueness: In past participation guidelines, individuals with bleeding disorders were disqualified from athletic participation; however, with advances in medical care, these individuals may be permitted to participate in accordance with the law.

Conclusions: Individuals with hemophilia participate in athletics; therefore, team physicians and athletic trainers must be prepared to care for these individuals.

Until recently, individuals with blood coagulation disorders were not permitted to participate in athletics. Table 1 shows the 1990 sports participation recommendations for athletes with hemophilia. Medical advancements have aided in the care of individuals with hemophilia and may allow some individuals with hemophilia to participate in athletic sports, depending on the severity of their illness and the type of sport. Also in 1990, the Americans with Disabilities Act (ADA) was passed, requiring that no individual be discriminated against based on disability alone. Because individuals with hemophilia often participate in athletics, team physicians and athletic trainers must learn to prevent and treat sports injuries in hemophilic athletes.

Table 1. 1990 Sports Participation Guidelines for Athletes with Hemophilia*†

CASE REPORT

A 21-year-old male collegiate soccer player reported pain and loss of function in his left knee after an away soccer match. He denied any mechanism of injury or pain during the game. The athletic trainer noted marked edema of the lower leg and joint-line effusion of the knee. The athlete was unable to bear weight on his left leg because of pain. Orthopaedic tests could not be completed due to the intensity of the acute symptoms. Ice was placed on the athlete's knee, and the athlete asked to be taken to the hospital because of a history of bleeding problems. There was no documentation of any chronic medical condition in the athlete's medical records.

Upon arrival at the emergency room, the athlete informed the physician that he had mild hemophilia A with no inhibitors and did not use any prophylactic medication before athletic participation, such as desmopressin acetate (DDAVP) or recombinant factor VIII (rFVIII) infusions. Desmopressin acetate and rFVIII may result in increased circulating factor VIII. The emergency room physician noted that the left knee was markedly swollen and range of motion was severely restricted. There was no pain on palpation of the tibia, fibula, or distal femur. Valgus and varus ligamentous stress tests were negative. A Lachman test could not be performed because of pain and inability of the knee to flex. An x-ray of the left knee showed a joint effusion but no fracture. Clotting factor assay revealed a factor VIII level of 23%, which classified this athlete as a mild hemophiliac. The athlete's knee was aspirated, and the amount of rFVIII necessary to raise the circulating factor VIII to 100% was calculated and transfused. The athlete was fitted for crutches and instructed to elevate the knee and use a compression wrap. The emergency room physician made arrangements for follow-up with a hematologist at the home site. The athlete was then released from the hospital and returned home on the team bus.

The next day, a local hematologist examined the athlete and recommended infusions of factor VIII to maintain 100% level for the next 3 days. After the 6-day immobilization period, he started a rehabilitation program that included passive range of motion, quadriceps sets, and heel slides. Rehabilitation progressed slowly, as if the athlete had been immobilized for an extended period of time. He advanced to pain-free range of motion as tolerated. As the effusion subsided, the rehabilitation program became more aggressive. In order to decrease patellofemoral joint-distraction forces and prevent irritation to the patellofemoral joint, open kinetic chain knee extensions were performed on the KinCom dynamometer (Chattanooga Group Inc, Hixson, TN) from 50° to 90° and closed kinetic chain exercises were performed from 0° to 30°. Throughout the rehabilitation process, the athlete was monitored daily for increased swelling. Overall rehabilitation time was increased (compared with other athletes with hemarthrosis) because of swelling and concern for reinjury. Six weeks later, the athlete was cleared to play after the effusion completely subsided, range of motion and strength were restored, and the orthopaedist and hematologist cleared the athlete.

As a result of this injury, the soccer team physician, athletic trainer, and athletic training student developed an emergency plan for the safe participation of this athlete and other athletes with hemophilia. The plan included referral to a hematologist for preparticipation physical clearance and as a result of injury. Other components of the plan were DDAVP effectiveness testing, transportation of factor VIII to all away contests, and knowledge of hemophilia treatment centers for away competition sites (Table 2).

Table 2. Emergency Plan Considerations for Dealing with Hemophilia

DISCUSSION

When blood vessels are damaged, a series of biochemical reactions occurs to form a blood clot. The Figure shows the coagulation cascade, including the intrinsic, extrinsic, and common coagulation pathways. A clot is formed via the intrinsic pathway in response to an abnormal vessel wall in the absence of tissue injury. The process is initiated when contact occurs between blood and exposed endothelial cell surfaces. The name “intrinsic” implies that all the components are in the plasma. The extrinsic pathway results in fibrin clot formation in response to tissue injury. Tissue factor, which is extrinsic to plasma, is activated as a result of vascular injury. The 2 pathways converge at the activation of factor X and ultimately lead to the blood clot.¹

Fig. 1.

Blood coagulation mechanism. *HMWK indicates high molecular-weight kininogen; F, factor; Ca⁺⁺, calcium.

Hemophilia is a hereditary blood disease characterized by impaired coagulability of the blood.¹ The following information has been compiled to explain the factors that should be considered when making decisions regarding the athletic participation status of individuals with hemophilia. Particular attention should be given to developing a proper emergency plan for immediate care of the injured hemophilic athlete.

Types of Hemophilia

Hemophilia A is the most common hereditary coagulation disorder. This disorder is due to a partial or complete deficiency of factor VIII coagulant activity, which is the result of a gene defect on the X chromosome. Hemophilia A is an X-linked recessive disorder. Because males have only one X chromosome, affected males exhibit characteristics of the disorder. Females have two X chromosomes and the disorder is recessive; therefore, they only show characteristics of hemophilia if the unaffected X chromosome is inactivated, and usually their disease is less severe. One in every 10 000 males is affected with hemophilia A. Although hemophilia A is known as an inherited disorder, nearly 30% of individuals with the disorder have no prior family history,^1,2 and the condition is the result of spontaneous gene mutations.

Hemophilia B (Christmas disease) is a partial or complete deficiency of factor IX. The inheritance patterns and clinical presentation of hemophilia B are similar to those of hemophilia A. Hemophilia B is a sex-linked recessive disorder with clinical symptoms including hemorrhages and hemarthrosis. This disorder, however, is not as common as hemophilia A, occurring in 1 in 100 000 males.¹

Hemophilia C (Rosenthal syndrome) is a hereditary bleeding disorder caused by a deficiency of factor XI. It affects 1 of 8 in the Ashkenazi Jewish population. Hemophilia C is inherited as an autosomal recessive trait. Its incidence is 1 in 100 000.¹

Von Willebrand disease is an autosomal dominant coagulation disorder caused by a deficiency of a component of factor VIII. This condition is characterized by consistently prolonged bleeding times and mucocutaneous bleeding rather than hemarthroses and deep muscle hemorrhages. Other signs and symptoms include epistaxis, ecchymosis, easy bruisability, gastrointestinal bleeding, menorrhagia, and hemorrhage after surgery. The incidence of von Willebrand disease is 1 in 80 000.

History of Athletic Participation by Individuals with Hemophilia

Few publications describe the extent to which boys and adults with hemophilia participate in sports. A recent review article provided an overview of sports participation by hemophiliac athletes.³ It reported that only 3 other studies provide data on seasonal or yearly athletic participation of individuals with hemophilia.

In the first study, Glomstein⁴ reported participation of Norwegians with hemophilia involved in Nordic sports. Questionnaires were sent to 298 people, and 179 were returned. Of the 179 respondents, 178 indicated that they participated in Nordic winter sports during their lifetime, and 132 of 178 were still active.

In the second and third studies, Heijnen et al⁵ and Rodriguez-Merchant⁶ reported participation in physical activities and sports by 166 Dutch people with hemophilia in 1996 and 209 in 1997. In their most recent article, Heijnen et al³ reported the results of a self-administered questionnaire for 293 Dutch individuals who visited the clinic for their yearly check-up.^5,6 They considered type of sport and severity of hemophilia. Most individuals were severe hemophiliacs (217) and participated in 44 different sports, including cycling, fitness, skating, skiing, swimming, and tennis.

National Collegiate Athletic Association Division I team physicians were surveyed to determine if they would allow athletes with hemophilia to participate in sports. The survey questions were specific to the type of sport and the severity of the hemophilia. An additional purpose of the study was to determine if individuals with hemophilia A were currently participating in Division I athletics. Team physician were willing to allow participation under certain circumstances, and hemophilic athletes were currently participating.⁷

Factors to Consider When Making Participation Decisions

Many factors should be taken into consideration when making a decision to permit or disqualify an individual with hemophilia from athletic participation. Among these factors are the severity of the hemophilia, the type of sport, treatment, medication, the presence or absence of inhibitors (antibodies that may destroy rFVIII), and consultation with a specialist.

Severity of the Hemophilia. Hemophilia is classified as mild, moderate, or severe based on the severity of the disorder. Those with mild hemophilia have 5% to 50% of the normal level of concentration of factor VIII. These individuals are at minimal risk for spontaneous hemorrhages, but they are likely to bleed after trauma or surgery. Those with moderate hemophilia have between 1% and 5% of the normal factor VIII level. These individuals hemorrhage after moderate trauma. Those with severe hemophilia have less then 1% of the normal factor VIII level. These individuals frequently bleed after minimal or unrecognizable trauma, especially into joints and muscles.^1,8–10

Type of Sport. Sports are classified into 3 general categories: collision, contact, and noncontact. Collision sports are played with the intent of striking opposing athletes. Sports in this category include football, hockey, and rugby. Contact sports have the possibility of contacting opposing athletes; however, that is not the intent. Examples of these sports are basketball and soccer. In noncontact sports, contact among athletes is highly unlikely. Track and field and tennis are examples of noncontact sports.¹¹ Sports that are associated with a higher degree of inherent risk of musculoskeletal injury might not be appropriate for an athlete with hemophilia.

Treatment and Medication. Advances in treatment for hemophilia have allowed affected individuals to become more active. Recombinant factor VIII permits the athlete to prevent or treat bleeding that occurs as a result of injury. This gives the athlete reassurance that the injury can be managed efficiently. The plasma-derived concentrations currently used carry a low risk of transmitting bloodborne infections. Major efforts to reduce disease transmission have been instituted: for example, selecting low-risk plasma donors, adopting polymerase chain-reaction-based virus-detection methods, and retesting donors 6 months after taking blood to determine if it can be used.¹² In 1983, DDAVP was approved for use in the United States for individuals with mild hemophilia A and von Willebrand disease. Desmopressin acetate is a synthetic antidiuretic hormone that releases factor VIII from endothelial tissue. If more factor VIII is present in the bloodstream before an injury, the hemorrhage may not cause as much damage as it otherwise might^8,13; however, the response to DDAVP is not universal.¹⁴ To determine the effectiveness of DDAVP, treatment centers test baseline levels of circulating factor VIII, administer DDAVP, and then reassess circulating levels of factor VIII. If factor VIII levels increase 3- to 5-fold, the DDAVP treatment is considered effective and may be used in the future to prevent and treat athletic injuries.

In 1992, the first rFVIII was derived. This genetically engineered factor VIII allows for hemorrhage control. However, minimal risk of disease transmission was present because the rFVIII was formulated in human albumin. In 1997, a second generation of rFVIII was produced that does not require the use of human albumin.¹² The creation of rFVIII has made treatment more readily available for patients with hemophilia, and the current form permits treatment with no risk of disease transmission.

Inhibitors. An inhibitor is a type of antibody that destroys substances the body does not recognize. An individual with hemophilia A who has inhibitors to factor VIII neutralizes and inhibits factor VIII, allowing the bleeding to continue. Inhibitors are not inherited but acquired. Individuals with factor VIII levels of less than 5% of normal have a 20% to 33% chance of developing inhibitors. Although individuals with mild hemophilia are not likely to develop inhibitors, patients with severe hemophilia are at a much higher risk of acquiring inhibitors because they are exposed to more factor VIII therapy. Most inhibitors develop during childhood. Fifteen to twenty percent of heavily treated children with hemophilia develop antibodies by the age of 20 years.¹⁵

Until recently, hemophiliacs with inhibitors could not benefit from improvements in treatment strategies because their inhibitors also function to neutralize recombinant factor VIII. Treatments such as immune tolerance and FVIIa replacement are helpful for patients with inhibitors (Figure). Tolerance is built in patients through the repeated administration of high doses of factor VIII, suppressing the production of factor VIII inhibitors. This is achieved in two thirds of the patients exposed to repeated, high-dose administration of factor VIII.¹² By using FVIIa and the extrinsic pathway, the need for factor VIII is bypassed (Figure). Some advantages to FVIIa include a limited risk of virus transmission, absence of severe anaphylactic reactions, and reduced risk of inhibitor development. Disadvantages include the high cost and the need for repeated administration due to the short half-life of FVIIa (3 to 4 hours).

Examination and Consultation with Hematologist. Each patient needs to be evaluated on an individual basis. Past history of injuries, severity level, type of sport, and response to treatments need to be considered before making a final decision regarding athletic participation. Physical and emotional maturity should also be considered.¹⁶ Chronologic age, Tanner stage, and muscle strength can be used to assess physical maturity. Individuals who are aware of their body and who do not take unnecessary risks are more likely to be successful in sports. Risk takers are more apt to incur injury. The decision to allow participation should come with the aid of the patient's hematologist, but the team physician has the final say.

Factors Supporting Participation of Athletes with Hemophilia

With the injury risk associated with sports, one may ask, why should those with hemophilia participate in athletics? Supporting participation are the psychological and physiologic benefits of being involved in sports and exercise. The legal statute, the Americans with Disabilities Act, also supports the rights of hemophiliac individuals to participate.

Benefits of Exercise and Psychological Benefits. The benefits of physical activity are numerous for the general population. Among these benefits are improved cardiorespiratory efficiency, control of body fat, improved strength and flexibility, and improved psychological and emotional well-being.¹⁷ Hemophilic patients receive the same benefits from exercise and some additional benefits.^18,19 Through exercise, muscle power and range of motion can be maintained, reducing the number and severity of bleeding episodes caused by abnormal stresses.^20,21 Exercise has been proven to increase the level of circulating factor VIII.^20,22 All these benefits of exercise support the importance of providing athletic opportunities to those with hemophilia.

Americans with Disabilities Act. On July 26, 1990, the ADA was signed into law. This act protects against athletic disqualification based on disability. According to the law, schools and colleges may not discriminate against an otherwise qualified individual because of that individual's disability. In sport, an athlete who otherwise meets all criteria for participation, including having the skill level and strength and conditioning required to make the team, must be allowed to play.²³

An objective of the team physician is to avoid the unnecessary restriction of athletic participation. However, his or her principal responsibility is to protect the health of athletes.^24,25 It is important to note that court decisions have upheld the right of team physicians to medically disqualify athletes.^25,26 Team physicians must make individual participation decisions after considering the demands of a particular sport, the potential harmful effects on an athlete's health, and other participants' safety.^24,25 With modifications to procedures, some hemophilic athletes can be accommodated in an efficient manner and, thus, should be permitted to play.

CONCLUSIONS

Individuals with hemophilia have special needs. Prevention and management plans must be devised to expedite the care of these athletes¹⁸; however, many people who care for these athletes are not aware of the necessary prevention and intervention strategies. The physical, psychological, and social benefits of being involved in a team sport have been established,¹⁸ and the ADA has called attention to the rights of athletes with disabilities. Therefore, hemophilic athletes must not be denied the right to participate in sports if reasonable measures can be taken to accommodate these athletes. Team physicians and athletic trainers need to be prepared to treat hemophiliac athletes; however, it may not be in the best interests of certain athletes with hemophilia to participate, as the risks outweigh the benefits. In these cases, it is appropriate for the team physician to disqualify the athlete.