Hemophilic arthropathy: Current knowledge and future perspectives

Gualtierotti, Roberta; Solimeno, Luigi Piero; Peyvandi, Flora

doi:10.1111/jth.15444

Cited by 153 publications

(149 citation statements)

References 97 publications

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“…For example, uncontrolled hypertension more than doubles the risk of intracranial hemorrhage. 5 Because information on these risk factors/comorbidities were not shared in the paper, 3 we do not know whether any contributing factors were present that might have played a role in the bleeding events observed in these four patients.…”

Section: • Should We Avoid Caplacizumab In Actively Bleeding Patients?mentioning

confidence: 99%

“…It has been postulated that, in absence of evident joint bleeds, subclinical joint bleeds occur and may initiate the process of inflammation leading to synovitis and osteochondral damage. 5 A few studies have explored the burden of arthropathy in patients with non-severe hemophilia. One of these studies was a register-based study in Sweden that compared arthropathy between patients with mild hemophilia and control group from the general population, matched for age and sex.…”

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confidence: 99%

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The factor VIII treatment history of non‐severe hemophilia A—Response from original authors Abdi et al

Kloosterman

Abdi

Gouw

et al. 2021

Journal of Thrombosis and Haemostasis

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We read with great interest the letter of Álvarez Román et al., 1 reporting an alarming incidence of joint damage in 28 adults with nonsevere hemophilia A (14 moderate and 14 mild hemophilia) in Spain as measured by the HEAD-US protocol. We thank Álvarez Román et al. for sharing these previously unpublished data.The study group found joint abnormalities in the majority of patients, with only 29% (8 of 28 patients) reporting zero scores for all joints as measured by HEAD-US. In the total cohort, varying degrees of synovial or articular changes were seen in the joints. Percentage of patients who reported synovial hypertrophy ranged from 17.9% to 39.3%. In general, the ankles were the most affected joint, with HEAD-US scores ≥3 reported in 25% and 39.3% of patients in the left and right ankle, respectively. Patients with moderate hemophilia had a total score of 0 less frequently than patients with mild hemophilia (21% vs. 35%). Moreover, in nearly all joints higher proportions of positive scores were seen in the moderate patient group.

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Section: • Should We Avoid Caplacizumab In Actively Bleeding Patients?mentioning

confidence: 99%

mentioning

confidence: 99%

The factor VIII treatment history of non‐severe hemophilia A—Response from original authors Abdi et al

Kloosterman

Abdi

Gouw

et al. 2021

Journal of Thrombosis and Haemostasis

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“…Recurrent joint bleeding (hemarthrosis) causes synovial hypertrophy and hemosiderin deposition in macrophage-like synovial cells, as a consequence of the intra-articular shedding of blood and iron. An inflammatory process develops and angiogenesis leads to the formation of fragile vessels, thus exposing the synovial membrane to further bleeding in a vicious circle [ 7 ]. Subsequently, fibrosis of the subsynovial layer occurs and the production of proteolytic enzymes has a destructive effect on both cartilage and bone.…”

Section: Introductionmentioning

confidence: 99%

Current and Emerging Approaches for Pain Management in Hemophilic Arthropathy

et al. 2022

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Introduction: Hemophilia is an inherited bleeding hematological disorder characterized by the partial or complete deficiency of clotting factor VIII or IX. Hemophilic arthropathy is the consequence of repeated joint bleeding (hemarthrosis) and its management is based on the prevention of acute bleeding through the administration of the deficient clotting factor concentrate or non-factor therapies. In addition, the management of acute and chronic pain is pivotal in hemophilic arthropathy in order to restore function and allow rehabilitation of the joint. Methods:We conducted a qualitative review of the literature regarding current and emerging strategies for pain treatment in hemophilic arthropathy. This review considers systemic and local pharmacological and non-pharmacological interventions for acute and chronic pain management.Results: In hemophilic arthropathy, pain management is based on analgesics such as paracetamol, which represents the first choice for acute and chronic pain in adults and children, in association with opioids for adults. Non-steroidal anti-inflammatory drugs inhibit platelet function, so that the currently preferred drugs are short courses of cyclooxygenase 2 inhibitors. Local treatment with intra-articular injections of corticosteroids is an option for refractory cases and physiotherapy has an important role after hemarthrosis and for the long-term management of chronic pain for both pediatric and adult patients. Conclusions: The management of pain in hemophilia requires more standardization. Meanwhile, the safest drugs should be used at the lowest effective dosage and for periods as short as possible. For the non-pharmacological management of pain in these patients, a multidisciplinary team including hematologists, orthopedic surgeons, rheumatologists, and physiotherapists is warranted.

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“…Recurrent hemarthroses consequent to bleedings contributed to cartilage degeneration named hemophilic arthritis. [ 2 ]. Inappropriate treatment of intramuscular bleeding may cause soft tissue hematoma called hemophilic pseudotumor [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

Risk Factors for Amputation in the Surgical Treatment of Hemophilic Osteoarthropathy: A 20-Year Single-Center Report

Feng

Zhu

et al. 2022

Pain Research and Management

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Purpose. Hemophilic osteoarthropathy (HO) is a common cause of spontaneous bleeding in hemophiliacs. Surgical procedures are necessary for patients with severe HO. As a last resort, amputation is sometimes needed to treat complex HO cases. This study aimed to review the existing records of patients who underwent amputations in HO surgical treatment, summarize the risk factors, and provide relevant references for surgeons. Methods. We retrospectively reviewed the records of hemophilic patients received surgeries at Peking Union Medical College Hospital between 2000 and 2020. The amputation patients without neoplasm or acute trauma were screened out. Patient information was extracted from medical records. A literature retrieval of hemophilic amputation cases was conducted via PubMed. The risk factors of amputation were summarized and analyzed via descriptive statistics and Fisher’s precision probability test. Results. Four male hemophilia A patients out of 201 patients underwent lower limb amputation. The reasons of amputation contained severe pseudotumor with factor inhibitor and large bone defects, recurrent pathological fracture with pseudotumor, skin ulcer with chronic osteomyelitis, and pseudotumor with systematic infection. In cases reported in retrieved literature, severe pseudotumor with complications, bacterial infection and factor inhibitor were common factors. Discussion. As the first study on amputations in HO patients, we found that severe hemophilic pseudotumor, chronic bacterial infection, and coagulation factor inhibitor was potential risk factors for amputation. Sufficient factor replacement therapy is fundamental in the prevention of amputation. The early diagnosis and specially designed surgical techniques could improve the rate of limb salvage.

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Hemophilic arthropathy: Current knowledge and future perspectives

Abstract: This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

Cited by 153 publications

References 97 publications

The factor VIII treatment history of non‐severe hemophilia A—Response from original authors Abdi et al

The factor VIII treatment history of non‐severe hemophilia A—Response from original authors Abdi et al

Current and Emerging Approaches for Pain Management in Hemophilic Arthropathy

Risk Factors for Amputation in the Surgical Treatment of Hemophilic Osteoarthropathy: A 20-Year Single-Center Report

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