Intraspinal bleeding in haemophilia: successful treatment with factor VIII concentrate.

Abstract

SUMMARY A severely affected haemophilic boy became tetraparetic as a result of a spontaneously occurring intraspinal haematoma. Myelography defined the extent of the lesion and showed it to be extradural in site. Infusion of large doses of factor VIII concentrate led to dramatic improvement and avoided the need to operate.Intraspinal bleeding in haemophilia is extremely rare and recovery is even rarer. To date 14 cases have been recorded, and 11 of these have died. We report here a severely affected haemophilic patient who developed acute tetraparesis from spontaneous intraspinal bleeding; prompt treatment with large doses of factor VIII (antihaemophilic factor) contributed to a remarkable clinical recovery, accompanied by radiological resolution of the haematoma. Case reportThe patient was a 20 year old, severely affected haemophilic male with a baseline factor VIII level of less than 1%. He was previously described when he sustained a ruptured left ureter in the Ibrox football disaster of 1970of (Forbes et al., 1971. Four days before his referral he had again been a spectator on the terraces at a football match during which he 'strained' his neck but had no direct trauma. After this he had experienced mild neck and shoulder pain which had been treated as a suspected muscular bleed with a single infusion of factor VIII concentrate (about 750 units), a dose which was sufficient to raise his plasma level to about 20% of normal. This level would normally be considered adequate for such a trivial bleed. At that time no neurological signs were apparent. However, next day he was ad-

Spinal subdural hematoma revealing hemophilia A in a child: A case report

“…In the sagittal T2-weighted cervical MRI, the hematoma from C5 to midthoracic level, was hyperintense and mainly posteriorly located in the thoracic and thoracolumbar level. This is compatible with the timing of the imaging (6 th day after trauma and hemorrhage)[9,10] and is postulated to occur because of methemoglobin formation [11]. In the T1-weighted axial MRI scan of the lower thoracic region, areas of hyperintensity in the periphery of the hematoma were seen, suggesting methemoglobin formation.…”

“…The limited available literature mainly addresses the management of spinal epidural hematoma in the hemophiliacs [13-15,9]. Schmitz et al [16] has reported his survey of treatment and outcome in hemophilic patients with spinal epidural hematoma from 1977 to 1998.…”

Spinal epidural haematoma in haemophilia A with inhibitors – efficacy of recombinant factor VIIa concentrate

“…Such bleeds have been described in patients of all ages [ 1], but predominately in infants and children when due to inherited bleeding disorders [ 4]. As expected, patients with severe haemophilia A or B are most often affected, and neurological sequelae [ 5] or death [ 2] often ensue unless effective replacement therapy can be administered.…”

Spinal epidural heamatoma in Haemophilia A

“…However, more and more cases are reported where patients with Haemophilia could be treated successfully with conservative measures ( Table 1). Even a spastic tetraparesis could be improved with replacement therapy [19]. Table 1 gives a survey of treatment and outcome in Haemophilia in the last 20 years.…”