Objective. Previous studies have confirmed the poor correlation of symptoms, signs, and levels of acute-phase reactants with disease activity in ϳ50% of all patients with Takayasu arteritis (TA). Invasive angiographic studies demonstrate vessel lumen anatomy, but do not provide qualitative information about the vessel wall. Moreover, sequential invasive angiographic studies expose patients to high-dose ionizing radiation and catheter/procedure-related morbidity. The aim of the present study was to determine the utility of new developments in vascular magnetic resonance (MR) technology in patients with TA.Methods. Electrocardiogram-gated "edemaweighted" MR was used to evaluate the aorta and its primary branches with regard to the vascular lumen, vessel wall anatomy, and vessel wall edema in 24 TA patients (77 studies). Inclusion criteria were age <50 years and features of TA on both clinical examination and invasive angiographic studies. Patients were stratified based on clinical and laboratory indications of having either unequivocally active disease, inactive disease, or uncertain disease status.Results. MR revealed vessel wall edema in 94% (17 of 18), 81% (13 of 16), and 56% (24 of 43) of studies obtained during periods of unequivocally active disease, uncertain disease activity, and apparent clinical remission, respectively. Westergren erythrocyte sedimentation rate and C-reactive protein values did not correlate with either the clinical assessment of disease activity or MR evidence of vascular edema. The frequency of presumed vascular inflammation (edema), as assessed by MR, in patients who appeared to be in remission was similar to the reported frequency of new angiographic lesions and histopathologic evidence of active disease in surgical specimens from patients thought to be in remission. However, the presence of edema within vessel walls did not consistently correlate with the occurrence of new anatomic changes found on subsequent studies.Conclusion. Inconsistencies in the presence or absence of vessel edema and subsequent anatomic changes have cast doubt on the utility of edemaweighted MR imaging as a sole guide to disease activity and treatment in TA. In this study, the greatest utility of MR was in providing a safe, noninvasive means of assessing changes in vascular anatomy.

Classical Hodgkin lymphoma (cHL) and mediastinal (thymic) large B-cell lymphoma (MLBL) have clinical, histopathologic, and molecular genetic similarities. MAL, a gene that encodes a protein associated with lipid rafts in T and epithelial cells, is overexpressed in a majority of MLBLs and has been reported in a minority of cHLs. To study the clinical significance of MAL in cHL, we immunostained 86 cases; 16 cHLs (19%) expressed MAL. Expression correlated with nodular sclerosis subtype, and within this subtype, with grade 2 histology. Univariable analysis revealed association of age of 45 years or older, MAL expression, and an International Prognostic Score of more than 2 with worse failure-free survival. Age of 45 years or older, MAL expression, and stage III or IV were associated with worse overall survival (OS). Cox proportional hazards modeling showed age (P = .04 and P = .03, respectively) and MAL expression (P = .03 and P = .01, respectively) as independent predictors of time to failure-free survival and OS. Stage showed borderline significance in OS (P = .08). MAL expression seems to identify a subset of cHL with an adverse outcome and provides additional evidence for a link between cHL and MLBL.

Classical Hodgkin lymphoma (cHL) and mediastinal (thymic) large B-cell lymphoma (MLBL) have clinical, histopathologic, and molecular genetic similarities. MAL, a gene that encodes a protein associated with lipid rafts in T and epithelial cells, is overexpressed in a majority of MLBLs and has been reported in a minority of cHLs. To study the clinical significance of MAL in cHL, we immunostained 86 cases; 16 cHLs (19%) expressed MAL. Expression correlated with nodular sclerosis subtype, and within this subtype, with grade 2 histology. Univariable analysis revealed association of age of 45 years or older, MAL expression, and an International Prognostic Score of more than 2 with worse failure-free survival. Age of 45 years or older, MAL expression, and stage III or IV were associated with worse overall survival (OS). Cox proportional hazards modeling showed age (P = .04 and P = .03, respectively) and MAL expression (P = .03 and P = .01, respectively) as independent predictors of time to failure-free survival and OS. Stage showed borderline significance in OS (P = .08). MAL expression seems to identify a subset of cHL with an adverse outcome and provides additional evidence for a link between cHL and MLBL.

No abstract

Patients with plasma cell dyscrasia are at risk to develop DVT (prevalence in MGUS was 8%, in MM 10%) (Cancer101(3):558–566, 2004). The risk has been reported to be higher in newly diagnosed (ND) patients treated with Thalidomide and anthracyclines (Clinical Lymphoma. 4(1):32–5, 2003). DVd-T is effective in the treatment of MM patients but carries an increased risk of DVT. Our incidence of DVT with Thalidomide and Thalidomide/Dexamethasone with slow dose escalation and less frequent Dexamethasone was not different from non-Thalidomide containing regimens. We treated 105 patients with DVd-T from 8/2001to 4/2004. We included an algorithm to evaluate patients with symptoms of DVT aggressively and assayed different coagulation parameters (including Platelet Aggregation studies (PA) with Collagen, Epinephrine, ADP, Arachidonic Acid and different concentration of Ristocetin, factor V Leiden (FVL), Activated protein C resistance (APCR), and Von Willebrand factor (vWF)) at baseline and before each cycle of DVd-T. Because the first 35 patients had a high rate of DVT, the protocol was amended to add Aspirin (ASA) 81 mg daily based on preliminary laboratory data preformed on the first group of patients. Two patients on Warfarin for other indications were excluded from the analysis. Of the 103 patients remaining 54 were ND MM and 49 had (Relapsed/Refractory RR) MM. Mean age was 58.9 years, 56% were males and 26%, 42%, 19% and 13% had SWOG stage I, II, III and IV respectively. Four patients were heterozygous for FVL and did not develop DVT. Fifty-eight patients received ASA at the start of treatment while 26 received ASA after the start of treatment and 19 did not receive ASA. Twenty six post treatment (pRx) DVT occurred at a mean 109 days pRx with 15/84 (17.8%) occurring after the start of ASA and 11/19 (57.8%) occurring off ASA (p=0.0002). No significant bleeding complications were noted in patients on ASA. Two patients receiving ASA discontinued therapy; another patient during his travel was immobile for 5–6 hours at a time and inconsistent with ASA therapy, all three developed DVT. One patient on ASA developed below the knee DVT not requiring anticoagulation. Thus, pRx DVT on ASA that required anticoagulation was 11/82 (13%) versus 13/21 (61.9% p&lt;0.001). When compared to pretreatment levels, PA 30 days after initiation of therapy to Arachidonic acid (P&lt;0.001), ADP (P=0.025), Collagen (P&lt;0.001), Epinephrine (P&lt;0.001), Ristocetin 1500 mcg/ml (p=0.06) and 1200 mcg/ml (p=0.05) and vWF (p=0.01) were exaggerated. Following ASA therapy, 30 days pRx PA to ristocetin and vWF levels were not statistically different to pretreatment level suggesting that ASA has blunted the increase in PA. Baseline PA to Epinephrine was higher in patients who did not develop DVT and pRx PA to epinephrine was higher in patients who developed DVT (p&lt;0.001 and p=0.08 respectively). ASA decreases the incidence of DVT following the DVd-T regimen without increasing the risk of bleeding. The mechanism of thrombosis prevention of ASA may involve attenuating the increase of vWF and PA that occurs after DVd-T.