Management of muscle haematomas in patients with severe haemophilia in an evidence‐poor world

Sørensen, Benny; Benson, Gary; Bladen, Melanie; Classey, S.; Keeling, David; McLaughlin, Paul; Yee, T. T.; Makris, Michael

doi:10.1111/j.1365-2516.2011.02720.x

Cited by 32 publications

(27 citation statements)

References 48 publications

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“…Associated conditions in elderly patients are solid and lymphoproliferative tumors [2–4], autoimmune disease [5] and drugs [6], in up to 50% of cases the etiology remains unexplained, as in our patient. To better exclude the presence of solid tumors, as indicated in the international recommendations [7], we performed a mammogram and a toraco-abdominal CT scan, resulting normal, in adjunction to the ultrasound that is considered an important diagnostic tool in revealing deep muscle hematomas [8]. …”

Section: Discussionmentioning

confidence: 99%

“…The diagnosis of AHA should be clinically suspected in every patient with spontaneous bleeding, without familial or personal history, and is confirmed by laboratory investigations revealing the typical prolonged aPTT with normal PT-INR, due to a deficiency of one of the intrinsic coagulation factors or the presence of an inhibitor (most commonly against FVIII). Also LAC positivity and heparin therapy, that are other causes of prolonged aPTT with normal INR, should be excluded [8]. …”

Section: Discussionmentioning

confidence: 99%

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Efficacy of Corticosteroids Alone in the Eradication of Factor VIII Inhibitor in an Old Female with Idiopathic Acquired Haemophilia A: Description of a Case

Girelli¹,

Biasoli

Bassi³

et al. 2012

Case Reports in Rheumatology

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Acquired haemophilia A (AHA) is a rare and serious disorder mainly affecting elderly patients. It is caused by the production of autoantibodies directed against coagulation factors; patients present with spontaneous bleeding, potentially fatal, in the absence of familial or personal history. Autoimmune disorders, infections, solid and hematologic tumors, and drugs are predisposing factors, but up to 50 percent of cases remain unexplained. The diagnosis of AHA is confirmed by specific laboratory tests; and the therapy is a clinical challenge, due to the fact that older patients are often affected by comorbidities. By passing agents may be used when persistent bleeding or haemodynamic instability is observed; corticosteroids, alone or with immunosuppressive therapy, are necessary to inhibit the production of the autoantibodies. We describe a case in which steroids in monotherapy successfully, safely, and persistently inhibited the production of anti-Factor VIII antibodies, in an old patient admitted after rheumatologic consult.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Efficacy of Corticosteroids Alone in the Eradication of Factor VIII Inhibitor in an Old Female with Idiopathic Acquired Haemophilia A: Description of a Case

Girelli¹,

Biasoli

Bassi³

et al. 2012

Case Reports in Rheumatology

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“…The standard treatment in such case would be weight-based FVIII concentrate 1. However, this treatment is very costly and in a resource-limited country, it is usually not feasible or realistic in many cases.…”

Section: Descriptionmentioning

confidence: 99%

Haemophilia-A-related haematoma: management in resource constraint settings

et al. 2016

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DESCRIPTIONHaemophilia is a X-linked recessive benign haematological disorder characterised by bleeding manifestations and coagulation abnormalities. Haemophilia-A (HA) is more common form of the disease and is associated with deficiency of factor VIII (FVIII). Here we present an interesting case of a 16-year-old boy from the low socioeconomic strata, who was diagnosed to have severe HA (FVIII activity <1%) in early childhood.By early pubertal age, he developed osteoarthropathy of left elbow joint secondary to recurrent episodes of haemarthrosis and due to nonaffordability of FVIII therapy. This time, he presented with acute onset, unprovoked, painful, gradually progressive swelling over left side of the back. On examination, there was a large, subcutaneous, mildly tender, non-pitting, non-pulsatile, diffuse swelling over the left side of the back extending inferiorly up to the inferior angle of left scapula of ∼16 cm×12 cm (figure 1).The standard treatment in such case would be weight-based FVIII concentrate.1 However, this treatment is very costly and in a resource-limited country, it is usually not feasible or realistic in many cases. Cryoprecipitate and fresh frozen plasma (FFP) are acceptable ways of supplementing FVIII. However, the use of FFP is limited by the volume overload, and it can increase FVIII to a maximum concentration of 15-20% only. As the patient could not afford FVIII concentrate and bleeding was located in a non-vital region, patient was managed with FFP as a cheap, easily available alternative therapy. He was transfused FFP at the rate of 15 mL/kg/day for 7 days. Excellent response was noted in the form of significant reduction in the swelling and pain, along with improvement in joint mobility in 7 days (figures 1 and 2). Learning points▸ In a resource-constrained situation, management of a non-fatal bleed in haemophilia should be promptly started with alternative therapy, in order to decrease the debility of haemophiliacs. ▸ Fresh frozen plasma (FFP) is an effective alternative therapy for non-fatal haematoma in haemophilia-A. ▸ Fluid overload and minimal recovery of factor VIII level are major limitations for FFP transfusion.Figure 2 Chest X-ray (lateral view) showing soft tissue haematoma in the back, followed by resolution of soft tissue collection after fresh frozen plasma transfusion. Figure 1 Clinical examination showing large subcutaneous haematoma in the back in the initial period followed by complete resolution of the swelling after fresh frozen plasma transfusion.

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“…Expectations for time to full recovery in PWH should be realistic and based on known timelines from non-hemophilic populations [16]. The decision to conclude the rehabilitation can be based on the ability to stretch the injured muscle to prebleed levels and the pain-free use of the injured muscle.…”

Section: Sébastien Lobet 1 and David Stephensenmentioning

confidence: 99%

“…The goal of physiotherapy in the initial acute phase of hematoma is to decrease pain and swelling, support wound healing, and maintain hemostasis [16]. The RICE regime is recommended with supplementary restrictions.…”

Section: Acute Hematomamentioning

confidence: 99%