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1、NCCN org Version 3 2019 01 31 19 2019 National Comprehensive Cancer Network NCCN All rights reserved NCCN Guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN NCCN Clinical Practice Guidelines in Oncology NCCN Guidelines Hairy Cell Leukemi
2、a Version 3 2019 January 31 2019 Continue NCCN Mary Dwyer MS Hema Sundar PhD Continue NCCN Guidelines Panel Disclosures Medical oncology Hematology Hematology oncology Radiotherapy Radiation oncology Bone marrow transplantation Pathology Internal medicine Dermatology Patient advocacy Discussion Writ
3、ing Committee Member Randall S Davis MD University of Alabama at Birmingham Comprehensive Cancer Center Christopher D Fletcher MD University of Wisconsin Carbone Cancer Center Brian Hill MD PhD Case Comprehensive Cancer Center University Hospitals Seidman Cancer Center and Cleveland Clinic Taussig C
4、ancer Institute Brad S Kahl MD Siteman Cancer Center at Barnes Jewish Hospital and Washington University School of Medicine Manali Kamdar MD University of Colorado Cancer Center Lawrence D Kaplan MD UCSF Helen Diller Family Comprehensive Cancer Center Nadia Khan MD Fox Chase Cancer Center Thomas J K
5、ipps MD PhD UC San Diego Moores Cancer Center Shuo Ma MD PhD Robert H Lurie Comprehensive Cancer Center of Northwestern University Sami Malek MD University of Michigan Rogel Cancer Center Anthony Mato MD Memorial Sloan Kettering Cancer Center Claudio Mosse MD PhD Vanderbilt Ingram Cancer Center Vish
6、ala T Neppalli MD Roswell Park Comprehensive Cancer Center Mazyar Shadman MD MPH Fred Hutchinson Cancer Research Center Seattle Cancer Care Alliance Tanya Siddiqi MD City of Hope Comprehensive Cancer Center Deborah Stephens DO Huntsman Cancer Institute at the University of Utah Nina Wagner MD The Si
7、dney Kimmel Comprehensive Cancer Center at Johns Hopkins William G Wierda MD PhD Chair The University of Texas MD Anderson Cancer Center John C Byrd MD Vice Chair The Ohio State University Comprehensive Cancer Center James Cancer Hospital and Solove Research Institute Jeremy S Abramson MD Massachuse
8、tts General Hospital Cancer Center Syed F Bilgrami MD Yale Cancer Center Smilow Cancer Hospital Greg Bociek MD MSc Fred presence of peripheral lymphadenopathy uncommon Performance status Peripheral blood smear examination CBC with differential Comprehensive metabolic panel with particular attention
9、to renal function Lactate dehydrogenase LDH Bone marrow biopsy aspirate Hepatitis B testingf if treatment contemplated Pregnancy testing in women of child bearing age if systemic therapy planned USEFUL UNDER CERTAIN CIRCUMSTANCES Chest abdominal pelvic CT with contrast of diagnostic quality Discussi
10、on of fertility issues and sperm banking See Initial Treatment HCL 2 eHCL with IGHV4 34 rearrangement behaves more like HCLv although it has a morphology and immunophenotype like cHCL IGHV4 34 HCL typically lacks BRAF V600E mutations does not respond well to purine analog therapy and has a relativel
11、y poorer prognosis compared to cHCL There is evidence that HCLv and IGHV4 34 HCL often show mutations in MAPK1 fHepatitis B testing is indicated because of the risk of reactivation during treatment eg immunotherapy chemoimmunotherapy chemotherapy targeted therapy Tests include hepatitis B surface an
12、tigen and core antibody for a patient with no risk factors For patients with risk factors or previous history of hepatitis B add e antigen If positive check viral load and consult with gastroenterologist NCCN Guidelines Version 3 2019 Hairy Cell Leukemia Version 3 2019 01 31 19 2019 National Compreh
13、ensive Cancer Network NCCN All rights reserved NCCN Guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN Note All recommendations are category 2A unless otherwise indicated Clinical Trials NCCN believes that the best management of any pati
14、ent with cancer is in a clinical trial Participation in clinical trials is especially encouraged NCCN Guidelines Index Table of Contents Discussion Printed by Maria Chen on 1 31 2019 8 49 06 PM For personal use only Not approved for distribution Copyright 2019 National Comprehensive Cancer Network I
15、nc All Rights Reserved HCL 2 gGrever MR Abdel Wahab O Andritsos LA et al Consensus guidelines for the diagnosis and management of patients with classical hairy cell leukemia Blood 2017 129 553 560 hStandard dose purine analogs should not be administered to patients with active life threatening or ch
16、ronic infection Treat active infection prior to initiating treatment with standard dose purine analogs If it is not possible to control infection consider initiating treatment with low dose pentostatin before using standard dose purine analogs to secure a durable response iSee HCL Response Criteria HCL A jSee Treatment References HCL B kStudied for primary refractory disease and early relapse 1 2 y after first course of purine analog lSee NCCN Guidelines for CLL SLL Special Considerations for th
