Severe postthrombotic syndrome is associated with characteristic sonographic pattern of the residual thrombosis.

Postthrombotic syndrome (PTS) may affect 50% of patients with deep venous thrombosis, 5-10% of them may present severe manifestations. The causes for PTS development and severity have not been well established. This study evaluated whether PTS may be associated with the presence, and echogenicity, of the residual vein thrombosis (RVT). We included patients with a history of deep venous thrombosis in the past 58 months. These patients were further evaluated for PTS diagnosis, clinical comorbidities, plasma levels of D-dimer, serum levels of C-reactive protein and for the presence of RVT. Particularly, RVT was detected by ultrasound examination and the residual thrombi echogenicity was determined by grayscale median (GSM). Fifty-six patients were included, of which 41 presented PTS. Mild PTS was detected in 23 patients, moderate PTS in 11 and severe PTS in seven patients. Patients with severe PTS showed higher body mass index, higher abdominal circumference and higher C-reactive protein levels when compared with the other patients (P = 0.007, P = 0.002, P = 0.02, respectively). The ultrasound-generated GSM was significantly lower in patients with severe PTS compared with patients with mild-moderate PTS or no PTS (median = 24, 35 and 41, respectively; P = 0.04). A GSM value less than 25, which was consistent with a hypoechoic RVT, was the best cut-off value to discriminate patients with severe PTS from those with mild or moderate PTS and those without PTS. RVT is a common finding among patients with PTS and the echogenicity of the RVT may impact the severity of PTS.

Inflammatory, immune and lipid transportation proteins are differentially expressed in spontaneous and proximal deep vein thrombosis patients.

INTRODUCTION: Deep vein thrombosis (DVT) is a multi-causal disease associated with high morbidity and mortality due to complications, and 25% of patients present recurrence within 5 years. The identification of factors involved with DVT can help in the management of patients, prevention of recurrence and in the development of new therapies. The evaluation of plasma components using proteomics potentially provides a window into the individual's state of health. We analyzed the protein profile of plasma samples from 3 DVT patients and compared results to those obtained from 1 sibling and 1 neighbor of each patient. These patients were selected as they presented a personal and family history of spontaneous and recurrent episodes of proximal DVT. MATERIAL AND METHODS: Albumin was removed using Affi-Gel Blue Gel, and the proteins were alkylated, reduced, precipitated and hydrolyzed. The peptides were fractionated by SCX chromatography, the 7 fractions obtained were directed to the ESI Q-TOF Premier mass spectrometer. Protein search was performed using the Mascot engine against the IPI human database. RESULTS: Proteins that were statistically overexpressed in DVT patients included C4-A plasma protease, C1 inter-alpha-trypsin inhibitor, heavy chain H inhibitor and serum amyloid A. Proteins that were statistically reduced in DVT patients included alpha-2-HS-glycoprotein, isoform 2 of inter-alpha-trypsin inhibitor heavy chain H4 and apolipoprotein A-IV. CONCLUSIONS: The evaluation of plasma from patients with spontaneous DVT allows the identification of differently expressed proteins when compared to controls; this expression may be of pathological importance for immune and inflammatory processes in DVT.

Increased ADAMTS13 activity in patients with venous thromboembolism.

Increased levels of inflammatory markers and clotting factors have been related to the pathogenesis and prognosis of venous thromboembolism. In particular, the imbalance between VWF and ADAMTS13 has been described in patients with arterial thrombosis. In this study, 77 patients with previous VTE and 77 matched controls were selected for the evaluation of the inflammatory markers, FVW, ADAMTS 13 and D-dimer. The presences of post-thrombotic syndrome and residual vein obstruction were also assessed in patients. Serum levels of TNF-α and IL-6 were significantly increased in patients compared to controls (median=2.25 vs 1.59 pg/mL, P ≤ 0.001; 1.16 vs 0.98 pg/ml, P=0.013, respectively). Plasma levels and activity of VWF (median=150.25 vs 95.39 U/dL, P ≤ 0.001; 145.26% vs 92.39%, P ≤ 0.001) and ADAMTS 13 (median=1088.84 vs 950.80 ng/mL, P ≤ 0.001; 96.03 vs 83.64%, P ≤ 0.001) were also higher in patients. We further analysed the subgroups of patients with higher risk for VTE recurrence or VTE sequelae, defined as the presence of high D-dimer levels, RVO or PTS. All inflammatory markers were significantly higher in patients with increased D-dimer. The presence of PTS or RVO was not associated with higher inflammatory or coagulation parameters. The increased levels of inflammatory markers and VWF may suggest that there is a persistence of inflammatory activity in patients even at long periods after the VTE episode. In this context, it may be postulated that increased levels of ADAMTS13 could represent a compensatory mechanism against persistently increased levels of VWF. Moreover, increased inflammatory activity was associated with increased D-dimer levels, thus it is possible that this inflammatory activity may also be related to the risk of VTE recurrence.

Diagnosis of Scott syndrome in patient with bleeding disorder of unknown cause.

Scott syndrome is a rare bleeding disorder due to an impaired exposure of phosphatidilserine on the platelet membrane, compromising the platelet procoagulant activity, thrombin generation and, thus, the clot formation. We report a case of a 17-year-old female adolescent with bleeding episodes of unknown cause. She had normal coagulation, but altered platelet aggregation under arteriolar flow, indicating platelet dysfunction. Furthermore, the expression of Annexin V was markedly reduced and the diagnosis of Scott syndrome was established. She was treated with platelet transfusions and demonstrated a clinical improvement. Scott syndrome may be investigated in cases with bleeding history and normal coagulation tests.