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  • Correspondence

    To the Editor: Hairy-cell leukemia (HCL) is a mature B-cell lymphoid cancer that is commonly treated with purine analogues. Virtually all patients with HCL carry the BRAF V600E mutation, which constitutively activates the MEK–ERK pathway and which can be inhibited in vitro by the mutation…

    • May 24, 2012
    • N Engl J Med 2012; 366:2038-2040
    • Free Full Text

    The authors report a dramatic response to vemurafenib in a patient with hairy-cell leukemia refractory to nucleosides and rituximab.

  • Clinical Therapeutics

    Foreword. This Journal feature begins with a case vignette that includes a therapeutic recommendation. A discussion of the clinical problem and the mechanism of benefit of this form of therapy follows. Major clinical studies, the clinical use of this therapy, and potential adverse effects are…

    • May 24, 2012
    • Maloney D.G.
    • N Engl J Med 2012; 366:2008-2016

      A 62-year-old man receives a diagnosis of diffuse large B-cell lymphoma; treatment with a regimen including the anti-CD20 antibody rituximab is recommended. Rituximab has been shown to improve progression-free survival when added to standard chemotherapy regimens for lymphoma.

    • Case Records of the Massachusetts General Hospital

      Presentation of Case. Dr. Steven Jay Isakoff (Hematology–Oncology): A 32-year-old woman was seen in the outpatient cancer center at this hospital because of infiltrating ductal carcinoma of the right breast. The patient had been well until 2 months before admission, when she became aware of a…

      • May 24, 2012
      • Baselga J., Smith B.L., Rafferty E.A., Bombonati A.
      • N Engl J Med 2012; 366:2018-2026
      • CME

      A 32-year-old woman noted a crease in her right breast. Imaging studies showed a suspicious mass, and a biopsy specimen revealed infiltrating ductal carcinoma, positive for HER2. Staging showed liver lesions consistent with metastases. Management decisions were made.

    • Original Article

      A goal of therapy for multiple myeloma, to induce complete remission and prolong survival, is usually accomplished with combination therapy. Autologous hematopoietic stem-cell transplantation is often used after induction chemotherapy to improve the response or to consolidate complete remission.…

      • May 10, 2012
      • McCarthy P.L., Owzar K., Hofmeister C.C., et al.
      • N Engl J Med 2012; 366:1770-1781

        In patients who had undergone stem-cell transplantation, lenalidomide maintenance therapy improved progression-free and overall survival, though at the expense of some increased hematologic toxicity and second malignant tumors.

      • Original Article

        During the past decade, high-dose chemotherapy with autologous stem-cell transplantation has become the standard treatment for newly diagnosed myeloma in patients younger than 65 years of age. However, the median duration of response after this procedure does not exceed 3 years, and few patients…

        • May 10, 2012
        • Attal M., Lauwers-Cances V., Marit G., et al.
        • N Engl J Med 2012; 366:1782-1791

          Lenalidomide maintenance after stem-cell transplantation significantly prolonged progression-free and event-free survival in patients with multiple myeloma. At 4 years, overall survival was similar in the lenalidomide-treated and placebo-treated groups.

        • Editorial

          With the introduction of high-dose therapy and new agents, outcomes of treatment have improved considerably for patients with myeloma. As therapy has become more effective, the goals of therapy have wavered between fixed-duration therapy aimed at eradication of the malignant clone and maintenance…

          • May 10, 2012
          • Badros A.Z.
          • N Engl J Med 2012; 366:1836-1838

            With the introduction of high-dose therapy and new agents, outcomes of treatment have improved considerably for patients with myeloma.1 As therapy has become more effective, the goals of therapy have wavered between fixed-duration therapy aimed at ...

          • Original Article

            Melphalan–prednisone (MP) has long been the treatment of choice for patients with multiple myeloma who are older than 65 years of age. The introduction of new agents in the past few years has substantially changed the treatment of multiple myeloma. MP plus either thalidomide or bortezomib is…

            • May 10, 2012
            • Palumbo A., Hajek R., Delforge M., et al.
            • N Engl J Med 2012; 366:1759-1769
            • CME

            Patients treated with an induction regimen of melphalan, prednisone, and lenalidomide followed by lenalidomide maintenance therapy had longer progression-free survival than those who did not receive maintenance therapy.

          • Editorial

            In this issue of the Journal, Schlumberger et al. and Mallick et al. describe the administration of radioiodine (iodine-131) after total thyroidectomy in patients with low-risk thyroid cancer. Postsurgical treatment has long played an important role in the management of this increasingly common…

            • May 3, 2012
            • Alexander E.K. and Larsen P.R.
            • N Engl J Med 2012; 366:1732-1733

              In this issue of the Journal, Schlumberger et al.1 and Mallick et al.2 describe the administration of radioiodine (iodine-131) after total thyroidectomy in patients with low-risk thyroid cancer. Postsurgical treatment has long played an important role in ...

            • Original Article

              Thyroid cancer is the most frequently occurring endocrine cancer, with more than 2100 new cases each year in the United Kingdom and more than 48,000 in the United States. Most cases are differentiated thyroid cancer, which is associated with a high 10-year survival rate (90 to 95%). Many patients…

              • May 3, 2012
              • Mallick U., Harmer C., Yap B., et al.
              • N Engl J Med 2012; 366:1674-1685

                In this trial, low-dose radioiodine was as effective as high-dose radioiodine in patients with differentiated thyroid tumors, and recombinant human thyrotropin (thyrotropin alfa) was as effective as thyroid hormone withdrawal.

              • Images in Clinical Medicine

                Figure 1.

                • May 3, 2012
                • Tan S.-K. and Tay Y.-K.
                • N Engl J Med 2012; 366:1724
                • Free Full Text

                A 52-year-old man presented with a 1-year history of a vesicular rash on his scrotum. The lesions were painless, but they occasionally ruptured and drained clear fluid.

              • Original Article

                Radioiodine (131I) is administered to patients with thyroid cancer after total thyroidectomy for three reasons–: first, to eradicate normal-thyroid remnants (ablation) in order to achieve an undetectable serum thyroglobulin level; second, to irradiate any neoplastic focus in order to decrease the…

                • May 3, 2012
                • Schlumberger M., Catargi B., Borget I., et al.
                • N Engl J Med 2012; 366:1663-1673
                • CME

                This trial compared two thyrotropin-stimulation methods and two 131I doses for postoperative ablation in patients with low-risk thyroid cancer. Rates of ablation were similar in all treatment groups. Doses lower than those currently recommended may be adequate for this condition.

              • Original ArticleOnline First

                Adrenocortical carcinoma is a rare cancer (estimated incidence, 0.7 to 2.0 cases per 1 million population per year), with a poor prognosis; the 5-year survival rate is less than 15% among patients with metastatic disease.– Mitotane is the only drug approved for the treatment of adrenocortical…

                • May 2, 2012
                • Fassnacht M., Terzolo M., Allolio B., et al.
                • 10.1056/NEJMoa1200966
                • Free Full Text

                Adrenocortical carcinoma is a rare cancer (estimated incidence, 0.7 to 2.0 cases per 1 million population per year)1,2 with a poor prognosis; the 5-year survival rate is less than 15% among patients with metastatic disease.37 Mitotane is the only drug ...

              • Original Article

                Bladder cancer, with more than 385,000 new cases worldwide in 2008, is a major cause of cancer complications. The median age at diagnosis is over 70 years, and since the tumor often is related to smoking, many patients have a substantial number of coexisting illnesses that pose risks for radical…

                • April 19, 2012
                • James N.D., Hussain S.A., Hall E., et al.
                • N Engl J Med 2012; 366:1477-1488

                  Management of muscle-invasive bladder cancer with radiation is associated with a 45% local relapse rate at 2 years. Concurrent treatment with mitomycin C and fluorouracil reduced recurrence to 33% and was not associated with increased late treatment-related toxicity.

                • Editorial

                  In comparison with prostate cancer (a prevalent, noisy, and newsworthy neighbor), bladder cancer is less widely discussed, is evaluated in far fewer randomized trials, and is less frequently the subject of articles in prominent medical journals. This is why the results from the British randomized…

                  • April 19, 2012
                  • Shipley W.U. and Zietman A.L.
                  • N Engl J Med 2012; 366:1540-1541

                    In comparison with prostate cancer (a prevalent, noisy, and newsworthy neighbor), bladder cancer is less widely discussed, is evaluated in far fewer randomized trials, and is less frequently the subject of articles in prominent medical journals. This is ...

                  • Original Article

                    Ovarian cancer is the leading cause of death from gynecologic tumors in the Western world. Approximately 80% of patients with newly diagnosed ovarian cancer have a response to platinum-based chemotherapy. However, most patients have relapses, and responses to subsequent therapies are generally…

                    • April 12, 2012
                    • Ledermann J., Harter P., Gourley C., et al.
                    • N Engl J Med 2012; 366:1382-1392

                      In a placebo-controlled, phase 2 study, olaparib, an oral PARP inhibitor that should be effective in tumor cells with BRCA1/2-related base excision repair defects, increased progression-free survival from 4 months to 8 months in patients with platinum-sensitive relapsed ovarian cancer.

                    • Editorial

                      Approximately 80% of children with acute lymphoblastic leukemia (ALL) are now cured, with estimates that contemporary treatment may further increase the cure rate to near 90%. Yet this figure is deceptive; for those children with unfavorable features, defined either by disease biology or by…

                      • April 12, 2012
                      • Rabin K.R.
                      • N Engl J Med 2012; 366:1445-1446

                        Approximately 80% of children with acute lymphoblastic leukemia (ALL) are now cured, with estimates that contemporary treatment may further increase the cure rate to near 90%.1 Yet this figure is deceptive; for those children with unfavorable features, ...

                      • Original Article

                        Current treatment for acute lymphoblastic leukemia (ALL) can effect a cure in approximately 80% of children with the disease.– The leading cause of treatment failure is relapse, for which a number of risk factors have been identified, with inadequate therapy being one of the most important.– A…

                        • April 12, 2012
                        • Schrappe M., Hunger S.P., Pui C.-H., et al.
                        • N Engl J Med 2012; 366:1371-1381

                          Induction chemotherapy fails to induce a complete remission in only about 2 to 3% of children with ALL. In an analysis of more than 1000 such patients, the authors defined subgroups with a favorable prognosis and those with an unfavorable prognosis.

                        • Editorial

                          For years, scientists have postulated the evolution of a cancer as a series of acquired mutations and epigenetic alterations that accumulate in a progressive way, beginning with a single transformed cell. Within this process, subclones of cells develop that acquire new properties, giving cells…

                          • March 22, 2012
                          • Godley L.A.
                          • N Engl J Med 2012; 366:1152-1153

                            For years, scientists have postulated the evolution of a cancer as a series of acquired mutations and epigenetic alterations that accumulate in a progressive way, beginning with a single transformed cell. Within this process, subclones of cells develop ...

                          • Clinical Implications of Basic Research

                            Effective, curative chemotherapy has been a goal of modern cancer medicine for half a century. Many newly developed agents have led to modest improvements in survival. However, few new curative treatments of advanced cancers have been developed during the past quarter century — perhaps because of…

                            • March 22, 2012
                            • Weiner L.M. and Lotze M.T.
                            • N Engl J Med 2012; 366:1156-1158

                              Autophagy, a programmed cell-survival strategy that allows cells to sustain themselves by digesting their own contents, turns out to be a critical link between chemotherapy-induced cell death and the triggering of an antitumor immune response.

                            • Original Article

                              Previous studies have highlighted the clinical and biologic heterogeneity of acute myeloid leukemia (AML).– However, a relatively small number of cytogenetic and molecular lesions have sufficient relevance to influence clinical practice. The prognostic relevance of cytogenetic abnormalities has…

                              • March 22, 2012
                              • Patel J.P., Gönen M., Figueroa M.E., et al.
                              • N Engl J Med 2012; 366:1079-1089

                                Malignant cells in most patients with acute myeloid leukemia have mutations. Cytogenetic classification yields some prognostic information, but the intermediate-risk group is heterogeneous. Mutational analysis identifies intermediate-risk patients who may benefit from high-dose therapy.

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                              Medical Meetings Pediatrics Conferences and Meetings

                              2012 Certifying Examinations of the American Board of Pediatrics

                              The general pediatrics examination will be held in various cities, Oct. 16-18. Registration for first-time applicants is ongoing through May 3. Registration for re-registrants is ongoing through May 24. The following subspecialty examinations will be held in various cities: "Hospice and Palliative Medicine" (Oct. 4); "Pediatric Transplant Hepatology" (Oct. 11); "Pediatric Cardiology" (Nov. 7); "Pediatric Pulmonology" (Nov. 8); "Medical Toxicology" (Nov. 12); and "Pediatric Critical Care Medicine" (Nov. 14). Registration for first-time applicants is ongoing through April 30. Registration for re-registrants is ongoing through June 15.

                              Contact the American Board of Pediatrics, 111 Silver Cedar Court, Chapel Hill, NC 27514-1513; or call (919) 929-0461; or fax (919) 918-7114 or (919) 929-9255; or see http://www.abp.org .

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