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Hematologic Arthritis
Introduction
This chapter reviews the elbow pathology associated with some hematologic conditions. The symptoms and radiographic changes of hematologic arthritis resemble inflammatory conditions such as rheumatoid arthritis. The medical treatment of the underlying process plays an important role in the management of elbow symptoms. Hemophilia and sickle cell disease represent the most common examples of hematologic arthritis. The elbow may also be affected in other conditions such as leukemia and other myeloproliferative disorders. The management of elbow problems in patients with underlying hematologic conditions requires a multidisciplinary approach.
Hemophilic Arthropathy
Hemophilia is an X-linked hereditary bleeding disorder characterized by deficiency of coagulation factors VIII (hemophilia A) or IX (hemophilia B). Patients with basal factor VIII or IX levels of less than 1% (severe hemophilia) are at particular risk of developing spontaneous intraarticular bleeding (hemarthrosis). Spontaneous hemarthrosis in hemophilia can occur as early as the second year of life. Rarely, spontaneous hemarthrosis may develop in patients with von Willebrand disease.
Historically, the knee has been the most frequent site for hemarthrosis in hemophilia. However, modern coagulation factor replacement therapy has significantly impacted the frequency and location of hemarthroses. Recent data have shown that the ankle and elbow are now the most frequently affected joints.
The main goal of management of articular manifestations of hemophilia is to identify and control the acute hemarthrosis through a multidisciplinary approach in an effort to prevent chronic, irreversible changes to joint function and structure. Early factor replacement therapy as well as rehabilitation can in many instances lead to full recovery of an isolated acute hemarthrosis. However, as repetitive bleeding occurs, synovitic hypertrophy may increase the risk of spontaneous bleeding, and it is frequently accompanied by chronic joint effusion and joint contractures. Finally chronic hemophilic arthropathy may result in terminal joint destruction.
Pathogenesis
The mechanism leading to hemophilic arthropathy is a complex multifactorial process involving increased intraarticular pressure, synovial inflammation, and cartilage degradation secondary to the deleterious effects of intraarticular blood. As a consequence of repetitive bleeding, chronic hypertrophic synovitis ensues, along with soft tissue contractures, cartilage loss and bone destruction ( Box 79.1 ). In children, the growth plates may be affected; this can lead to radial head hypertrophy and angular deformity.
Box 79.1
Pathogenesis of Hemophilic Arthropathy
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Direct deleterious effect of joint hematoma on
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Articular cartilage
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Bone
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Capsule and ligaments
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Secondary hypertrophic synovitis
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Cartilage, bone and soft tissue damage
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Contracture
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-
Growth plate abnormalities
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Growth deformities (angular, others)
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HIV infection
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Arthropathy
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Opportunistic infections
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Extensive localized bleeding leading to pseudotumor
Clinical Presentation
Patients with hemophilic arthropathy of the elbow may present in various stages of the disease. The first episodes of acute elbow hemarthrosis present with marked joint effusion (acute hemarthrosis). Due to distension of the joint capsule, the elbow usually assumes a flexed position, with significant limitation of motion. Patients who have had multiple episodes of bleeding develop chronic joint effusion (subacute hemarthrosis). Frequently synovitic hypertrophy can be palpated. In the more advanced stages of hemophilic arthropathy, muscle atrophy can be seen, along with angular deformity and occasionally increased laxity (chronic arthropathy). Ulnar nerve symptoms from hypertrophic osteophytes have been described.
As hemophilic arthropathy progresses, patients experience increasing loss of range of motion (ROM). Gamble et al. measured elbow motion in 48 patients with hemophilia followed for an average of 10.8 years. Patients older than 25 years had decreased motion in all planes compared with patients younger than 15 years. Pronation was the first motion to be restricted, and extension was the motion most affected at the end of follow-up. Interestingly, many patients with hemophilic arthropathy are more limited by their lack of pronation and supination than by limited flexion and extension.
Hemophilic arthropathy commonly affects other joints including the knees and ankles. In the upper extremity, the elbow is more commonly affected than the shoulder or the wrist; in a series of 23 moderate and severe hemophiliacs, the elbow joints were the site of recognizable arthropathy in 87% of the cases, whereas the shoulder and wrist were affected in a small proportion of patients. When surgery is contemplated, attention should be paid to the order in which the different joints need to be addressed.
Imaging Studies
Imaging in hemophilia plays an important role in establishing the diagnosis of joint involvement. In pediatric patients with severe hemophilia, radiographs should be taken every 6 months, while yearly intervals are recommended for adult individuals.
Radiographs
Plain radiographs are helpful in performing a broad assessment of hemophilic arthropathy. Findings may include joint line narrowing, osteophytes, subperiosteal new bone formation, and bone loss or deformity. Furthermore, osteonecrosis, subchondral cysts, and epiphyseal overgrowth may be seen.
Two main classification systems are used to rate the severity of the radiographic changes in hemophilic arthropathy: the Arnold and Hilgartner and the Pettersson classifications. The Arnold and Hilgartner scale includes five stages, as detailed in Table 79.1 ( Figs. 79.1–79.4 ). It is more simple to use but less detailed. The Pettersson classification has been adopted by the World Federation of Hemophilia ( Table 79.2 ). The score ranges from 0 (no radiographic abnormalities) to 13 (severe arthropathy). Radiographs are limited to detecting changes mainly at the bony level, thereby providing little input in the earlier stages of hemophilic arthropathy.
TABLE 79.1
Radiographic Classification of Hemophilic Arthropathy
| Stage | Features |
|---|---|
| I | No skeletal abnormalities |
| Soft issue swelling present | |
| II | Osteoporosis, epiphyseal overgrowth |
| Normal joint line | |
| No bone cysts | |
| III | Subchondral joint changes |
| Subchondral cysts visible | |
| Trochlear notch widened | |
| IV | Joint space narrowing |
| V | Loss of joint space |
| Joint contracture | |
| Enlargement of epiphyses or architectural changes |
TABLE 79.2
Pettersson Classification of Hemophilic Arthropathy (World Federation of Hemophilia)
| Variable | Finding | Score |
|---|---|---|
| Osteoporosis | Absent | 0 |
| Present | 1 | |
| Epiphyseal enlargement | Absent | 0 |
| Present | 1 | |
| Subchondral bone irregularity | Absent | 0 |
| Partial | 1 | |
| Complete | 2 | |
| Joint space narrowing | Absent | 0 |
| Joint space >1 mm | 1 | |
| Joint space <1 mm | 2 | |
| Subchondral cyst formation | Absent | 0 |
| 1 cyst | 1 | |
| 2+ cysts | 2 | |
| Joint margin erosion | Absent | 0 |
| Present | 1 | |
| Joint surface incongruency | Absent | 0 |
| Slight | 1 | |
| Pronounced | 2 | |
| Articular deformity | Absent | 0 |
| Slight | 1 | |
| Pronounced | 2 |
In the elbow, three main patterns of joint involvement have been identified: medial, lateral, and global involvement. Patients with involvement of predominantly the medial side of the joint present with ulnohumeral joint narrowing and medial spurring, which clinically may correlate with ulnar neuropathy. Lateral joint involvement is associated with radial head enlargement, posterolateral elbow pain, and restriction of pronation and supination. Finally, global involvement of the joint is associated usually with more severe stiffness affecting all planes of motion.
Ultrasound
Ultrasound is increasingly being used in the diagnosis of early stages of hemophilic arthropathy because it can reliably identify joint effusion and synovial hypertrophy. Ultrasound has the advantage of allowing dynamic joint assessment while being less expensive than magnetic resonance imaging (MRI) and not requiring sedation in pediatric or claustrophobic patients. However, the reliability of ultrasound is operator dependent, which has made standardization challenging.
MRI
MRI has become the main imaging modality for the assessment of hemophilic arthropathy. It allows evaluation of all structures involved in hemophilic arthropathy and has been shown to be more sensitive than clinical examination and simple radiography in detecting early changes. The main drawbacks of MRI are cost, duration of image acquisition, and the requirement for sedation in children. As with other inflammatory conditions, MRI has been used to more precisely evaluate abnormalities of the articular cartilage, soft tissues, and synovium and to monitor the progression of the arthropathy. Several scoring systems based on magnetic resonance findings have been devised to identify patients at risk for the development of severe arthropathy and to prevent articular changes by the use of more intensive medical treatment.
Laboratory Studies
The evaluation of patients with hemophilic arthropathy should be completed with coagulation studies and laboratory tests to detect HIV or hepatitis C infections, when indicated.
Management
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