Primary deep venous thrombosis of the upper extremity (UEDVT) is an unusual disorder. Limited data are available on the contribution of hypercoagulable status in the pathogenesis of this disease. This study aims to report the prevalence of inherited and acquired thrombophilic risk factors (TF) in patients with primary (effort-related and spontaneous) UEDVT. From 1993 to 2002, 31 patients (17 females, median age 38.8 years, range 16-60 years; and 14 males, median age 31.4 years, range 20-56 years) with primary UEDVT (n = 15 effort-related and n = 16 spontaneous) were referred for screening of hypercoagulable status. Nineteen (61.3%) patients had at least one coagulation abnormality. The most common acquired TF were antiphospholipid antibodies (31% lupus anticoagulant and 12.9% anticardiolipin antibodies). Factor V Leiden (12.9%) and prothrombin G20210A mutation (20%) were the most prevalent genetic risk factors. Five patients (16.1%) had high plasma homocysteine levels, and one patient (4.7%) had protein S deficiency. Effortrelated UEDVT was associated with male gender (P = 0.04) and younger age (P = 0.02). There was no significant difference in the prevalence of acquired or inherited TF between patients with effort-related or spontaneous UEDVT. A local anatomic abnormality was detected in seven patients (22.5%), and the prevalence of TF was significantly lower within this group (P = 0.006). The incidence of TF in patients without an anatomic abnormality was 75% (RR 5.25). This study found a high prevalence of an underlying thrombophilic status in spontaneous and effort-related UEDVT. Hypercoagulable status may play a significant role in both groups. Screening for local anatomical abnormalities and thrombophilia should be included in the evaluation of primary UEDVT. Am.
Antibodies directed against protein S (anti-ProtS) may be involved in the development of thrombosis in patients with the antiphospholipid syndrome. We assessed the prevalence and clinical significance of anti-ProtS and evaluated their immunological characteristics in 184 patients with SLE and 99 healthy donors. All patients were tested for IgG anti-ProtS by an in-house ELISA. Plasma levels and functional activity of protein S were also tested. Anti-ProtS were found in 57 patients (31%) and 4 healthy controls (4%). Patients with thrombosis had anti-ProtS more frequently than controls (29% vs 4%, OR 9.5 [95% CI 3.07-29.3], p<0.0001). Anti-ProtS were more frequent in patients with venous thrombosis and in those with arterial thrombosis, than in controls (41% vs. 4%, OR 16.5 [95% CI 5-54], p<0.0001 and 23% vs. 4%, OR 7 [95%CI 2.1-23.5], p=0.0008, respectively). Patients with prematurity, preeclampsia and intrauterine growth restriction had anti-ProtS more frequently than the control group (36%, 47% and 44% vs. 4%; OR 13.6 [95% CI 2.8-66], p=0.003, OR 21 [95% CI 5-86], p<0.0001 and OR 19 [95% CI 4-99], p=0.0014, respectively). Plasma levels of free protein S were not statistically different between patients with and without anti-ProtS and controls (77.9% [20.7-100] vs. 83.7% [52.7-100] vs. 89% [62-101], respectively). Free protein S functional activity was no different between subgroups (105% [48-230] in anti-ProtS positive vs. 123% [95-283] in anti-ProtS negative vs. 136% [60-174] in controls). Anti-ProtS are frequent in SLE patients with thrombosis and pregnancy morbidity. These antibodies do not interfere with free protein S in plasma since its level and/or functional activity are not impaired.
We read with interest the multicentre study on the use of alpha-interferon (IFN-a) in systemic mastocytosis (SM) (Casassus et al, 2002). We would like to make several comments.Although there was a good response to some symptoms in the group, this was at the expense of significant sideeffects. Bone pain appeared to be the most frequent symptom and showed improvement. However, we have found very good symptomatic relief of bone pain with bisphosphonate treatment in three patients with SM. One patient, a 42-year-old man, presented with long-standing and severe, refractory bone pain. Magnetic resonance imaging (MRI) demonstrated highly atypical multifocal marrow abnormalities that were suggestive of widespread metastatic disease. The diagnosis of SM was made after extensive malignancy screening, a repeat MRI and a bone marrow trephine, which was grossly fibrotic with mild myelodysplasia and abnormal mast cells. Monthly pamidronate infusions not only produced significant pain relief, but a repeat MRI after 19 months of treatment also demonstrated the improvement of bone marrow appearance. This was suggestive of a direct effect on mast cell production by the bisphosphonates, analogous to the effect of nitrogen-containing bisphosphonates in myeloma. The stronger bisphosphonate zoledronic acid may be even better in both respects and we are currently carrying out a trial to compare the two. A second patient with SM, a 48-year-old man who presented with pelvic pain, also had widespread MRI abnormalities of both the axial and appendicular skeleton. He has received pamidronate infusions monthly for 4 months, which has led to a good resolution of bone pain. A third patient with bone pain also demonstrated significant pain relief from pamidronate, but unfortunately suffered a delayed allergic reaction (by 12 h), which required admission and steroids to settle. As with all drugs, the possibility of allergic reactions in SM has to be carefully monitored, although the delayed nature of this reaction was unusual. In general, bisphosphonates would appear to be a better option for control of bone pain in SM rather than a-interferon, as the latter has many side-effects that are often similar to the patient's symptoms.
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