Our paper describes 5 patients with a
vascular malformation -
angiomatosis. In the first patient, a young man,
angiomatosis affected the stomach, intestine, the area of mesenterium and retroperitoneum as well as mediastinum. Angiomatous mass had invaded pelvic bones and vertebrae. Treatment was initiated with
interferon alpha in a maximum tolerated dose of 3 million units 3 times a week. Because of low efficacy of
interferon alpha,
thalidomide was added at a dose of 100 mg per day. Bone
pain disappeared following a few applications of
zoledronate administered in regular monthly intervals. After 3 years of concomitant administration of
interferon alpha and
thalidomide, we changed the regimen due to adverse effects and are administering
thalidomide and
interferon alternatively in 4-monthly intervals. Treatment has resulted in 50% reduction, according to imaging, of angiomatous mass, reduced intensity of
disseminated intravascular coagulation and disappearance of clinical signs. The second was a case of multiple
angiomatosis affecting the intestine only (multiple intestinal
angiodysplasias) where we used
thalidomide monotherapy. This treatment reduced blood losses and haemoglobin concentrations rose to normal levels. This male patient had consumed 120 transfusion units per year before the initiation of
thalidomide. The third case was a slowly progressing
vascular malformation of the face. This
vascular malformation troubled its sufferer by spontaneous shortening that could not be resolved surgically because of its fragility. Two years of combined treatment with
interferon a 6 million unites 3 times a week and
thalidomide 100 mg daily led to a reduction and flattening of the malformation, paling of its colour and ceasing of spontaneous
bleeding. This development enabled
minor surgery--partial excision of this large
vascular malformation. Histology examination confirmed that there was no evidence of new capillary formation. Histological examination thus confirmed efficacy of the treatment. The fourth case involved a patient with large
vascular malformations affecting supraclavicular region of the neck and nape in whom
radiotherapy was applied (54 Gy) leading to a reduction of the malformation mass by a at least 50%. The fifth is a case of an extensive periorbital
lymphangioma that diminished following treatment with
interferon alpha. These cases illustrate the benefits of combined treatment including
thalidomide and
interferon alpha in patients with multiple
angiomatosis or large proliferating
hemangioma (
vascular malformation). If combined treatment with
thalidomide and
interferon a is not possible, it is beneficial to use
thalidomide monotherapy.
Radiotherapy is another alternative, although it is necessary to apply doses exceeding 50 Gy which may not be always possible.