Fifty-one patients with lymphoblastic lymphoma (LBL) treated with one of five successive intensive chemotherapy protocols for acute lymphoblastic leukemia (ALL) since 1971 were reviewed. The patients were divided into leukemic and nonleukemic groups, and their clinical and laboratory parameters compared. The projected 5-year survival rate for all patients treated with the L10/17 protocols was 45% for both leukemic and nonleukemic LBL. The response to treatment was compared with that of 111 patients with ALL and was nearly identical. Poor prognostic factors were age beyond 30, WBC greater than 50,000/microL, failure to achieve a complete response (CR), and a late CR during induction. Leukemia at presentation, T cell surface markers, and the presence of a mediastinal mass did not adversely affect survival. The use of intensive chemotherapy protocols has proven to be a significant advance in the treatment of LBL.
Word countAbstract : 246 Main text: 2836 4 Abstract Background and Objectives: The ATTRACT trial reported that pharmacomechanical catheterdirected thrombolysis (PCDT) did not reduce postthrombotic syndrome (PTS), but reduced moderate-to-severe PTS and the severity of PTS symptoms. In this analysis, we examine the effect of PCDT in patients with femoral-popliteal DVT (without involvement of more proximal veins). Patients/Methods: Within the ATTRACT trial, 300 patients had deep vein thrombosis (DVT) involving the femoral vein without involvement of the common femoral or iliac veins and were randomized to receive PCDT with anticoagulation or anticoagulation alone (No PCDT). Patients were followed for 24 months. Results: From 6 to 24 months, between the PCDT vs. No PCDT arms, there was: no difference in any PTS (Villalta scale ≥5: Risk Ratio [RR]=0.97; 95% confidence interval [CI], 0.75 to 1.24); moderate-or-severe PTS (Villalta scale ≥10: RR=0.93; 95% CI, 0.57 to 1.52; severity of PTS scores; or general or disease-specific quality of life (p >0.5 for all comparisons). From baseline to both 10 days and 30 days, there was no difference in improvement of leg pain or swelling between treatment arms. From baseline to 10 days, major bleeding occurred in three vs. none (p=0.06) and any bleeding occurred in eight vs. two (p=0.032) PCDT vs. No PCDT patients. Over 24 months, recurrent venous thromboembolism occurred in 16 PCDT and 12 No PCDT patients (p=0.24).Conclusion: In patients with femoral-popliteal DVT, PCDT did not improve short-or long-term efficacy outcomes, but it increased bleeding. Therefore, PCDT should not be used as initial treatment of femoral-popliteal DVT. (NCT00790335).
Doege-Potter syndrome is a rare paraneoplastic syndrome that is often diagnosed incidentally during the workup of hypoglycemia of unclear etiology. It is characterized by a non-islet cell tumor hypoglycemia secondary to excessive production of partially processed IGF-II hormone from a solitary fibrous tumor (SFT). Often these tumors are intrathoracic, benign, and asymptomatic. Occasionally they present as a paraneoplastic event; hypertrophic osteoarthropathy in Pierre-Marie-Bamberger syndrome and hypoglycemia in Doege-Potter syndrome. The NAB2-STAT6 gene fusion is the hallmark of the SFT. Complete surgical resection of the tumor often results in resolution of symptoms and cure in most cases. Here we present the case of an 83-year-old non-diabetic female with recurrent syncopal events who was diagnosed with the Doege-Potter syndrome secondary to a SFT of pleura. Her tumor was positive for NAB2-STAT6 gene fusion on RT-PCR. Following the resection of the giant tumor mass, she became symptom-free within 24 h, and has remained asymptomatic at 4 months follow-up.
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