Erformed the experiments: TS TK. Analyzed the information: TS. Contributed reagents
Erformed the experiments: TS TK. Analyzed the data: TS. Contributed reagents materialsanalysis tools: M. Shimoda HDR ST NI. Wrote the paper: TS M. Shimoda. Guided the experiments: KI SH HA KK TY NG M. Setou ST. Supplied substantial input in to the writing from the manuscript: ST NI.Ambroxol ameliorates neurodevelopmental defects and decreases ER tension induced by mutant hGBA expression in Drosophila eyeAmbroxol is called a pharmacological chaperone for mutant glucocerebrosidase including the L444P point mutation [30]. Our
VOLUMENUMBERJUNEJOURNAL OF CLINICAL ONCOLOGYONCOLOGY GRAND ROUNDSStrategies for Relapsed Peripheral T-Cell Lymphoma: The Tail That Wags the CurveMatthew A. Lunning, Alison J. Moskowitz, and 15-LOX drug Steven Horwitz, Memorial Sloan-Kettering Cancer Center, New York, NY See accompanying write-up on page 1970 The Oncology Grand Rounds series is created to spot original reports published within the Journal into clinical context. A case presentation is followed by a description of diagnostic and management challenges, a critique of the relevant literature, and a summary in the authors’ suggested management Estrogen receptor Molecular Weight approaches. The aim of this series would be to aid readers far better realize the way to apply the outcomes of important studies, such as these published in Journal of Clinical Oncology, to patients observed in their very own clinical practice.A 69-year-old lady was referred for further evaluation and management of relapsed angioimmunoblastic T-cell lymphoma.Atdiagnosis,shereceivedsixcyclesofdose-adjustedEPOCH(etoposide,prednisone,vincristine,cyclophosphamide, and doxorubicin) and achieved a full response (CR). Her initially surveillance computed tomography scan three months later demonstrated enlarging cervical lymphadenopathy. A lymph node excision confirmed relapsed angioimmunoblasticT-celllymphomawithatypicallymphocytesexpressingCD3,CD4,CD10,PD-1,andEBER,withlossof CD5(Fig1).AclonalT-cellreceptorbetaandgammarearrangementbypolymerasechainreactionwasidenticaltothat inherinitialdiagnosticbiopsy.Atourinitialconsultation,optionsforstandardaswellasinvestigationaltherapieswere discussed, and HLA typing was initiated. The patient was enrolled onto an investigational phase II study; nevertheless, she developed progressive disease after two cycles. She was then treated with romidepsin 14 mgm2 administered intravenouslyfor3consecutiveweekswith1weekoff.Aftertwocycles,sheachievedapartialresponse,andafterfouradditional cycles, she maintained her response with out additional improvement. We discussed additional treatment possibilities.CHALLENGES IN DIAGNOSIS AND MANAGEMENTNearly two decades ago, the Revised European-American Lymphoma classification formally differentiated B- and T-cell lymphomas.1 Peripheral T-cell lymphomas (PTCLs) are malignancies arising from mature or post-thymic T lymphocytes. PTCL represents approximately 10 of all new diagnoses of non-Hodgkin lymphoma.2 Despite the infrequency, PTCLs are heterogeneous malignancies with 22 described clinicopathologic subtypes.3 The subtypes PTCL ot otherwise specified (NOS), angioimmunoblastic T-cell lymphoma (AITL), and anaplastic large-cell lymphoma (ALCL) represent the three most typical entities, accounting for almost 75 of patient circumstances in North America and Europe.four Based on the International Peripheral T-Cell Lymphoma Project (the largest retrospective series), 5-year general survival (OS) for PTCL-NOS, AITL, ALK-negative ALCL, and ALK-positive ALCL are 32 , 32 , 49 , and 70 , respectively. There is no universally agreed-o.