შეჯამება
განსაზღვრება
ანამნეზი და გასინჯვა
ძირითადი დიაგნოსტიკური ფაქტორები
- abdominal fullness or discomfort
- splenomegaly
სხვა დიაგნოსტიკური ფაქტორები
- weakness and fatigue
- unexplained weight loss
- bruising and bleeding
- pallor
- recurrent infections
- hepatomegaly
- lymphadenopathy
- neurologic findings
- associated systemic immunologic disorders
რისკფაქტორები
- middle age
- male sex
- white ancestry
- western hemisphere location
- environmental exposures
- genetic predisposition
- Epstein-Barr virus
- infectious mononucleosis
დიაგნოსტიკური კვლევები
1-ად შესაკვეთი გამოკვლევები
- CBC with differential
- peripheral blood smear
- bone marrow trephine biopsy and aspiration (morphology assessment)
- immunophenotyping (immunohistochemistry or flow cytometry)
- comprehensive metabolic panel
- serum lactate dehydrogenase (LDH)
- viral serology for hepatitis B and C
გასათვალისწინებელი კვლევები
- molecular analysis (for BRAF V600E mutation or IGHV4-34 rearrangement)
- CT chest, abdomen, and pelvis
მკურნალობის ალგორითმი
no indication(s) for treatment
indication(s) for treatment present: without splenic rupture or massive splenomegaly or marked thrombocytopenia precluding chemotherapy
indication(s) for treatment present: with massive symptomatic splenomegaly or splenic rupture or marked thrombocytopenia precluding chemotherapy
early relapse (<2 years) or refractory disease
late relapse (≥2 years)
კონტრიბუტორები
ავტორები
Ambuj Kumar, MD, MPH
Professor
USF Health Office of Research
Department of Internal Medicine, College of Medicine
Moffitt Cancer Center & Research Institute, Department of Health Outcomes & Behavior
University of South Florida
Tampa
FL
გაფრთხილება:
AK declares that he has no competing interests.
Mohamed A. Kharfan-Dabaja, MD, MBA, FACP
Professor
Division of Hematology-Oncology
Blood and Marrow Transplantation Program
Mayo Clinic
Jacksonville
FL
利益声明
MKD declares that he has no competing interests.
鸣谢
Dr Ambuj Kumar and Dr Mohamed Kharfan-Dabaja would like to gratefully acknowledge Dr Benjamin Djulbegovic, a previous contributor to this topic.
利益声明
BD declares that he has no competing interests.
同行评议者
Daniel Catovsky, MD, FRCP, FRCPath, DSc, FMedSc
Consultant Haemato-Oncologist
Section of Haemato-Oncology
Brookes Lawley Institute of Cancer
Sutton
UK
利益声明
DC declares that he has no competing interests.
Roger Lyons, MD
Clinical Professor of Medicine
University of Texas Health Science Center San Antonio
San Antonio
TX
利益声明
RL declares that he has no competing interests.
Rebecca Connor, MD
Chief Fellow
Section of Hematology and Oncology
Department of Internal Medicine
Wake Forest University Baptist Medical Center
Winston-Salem
NC
利益声明
RC declares that she has no competing interests.
Claire Dearden, BSc, MD, FRCP, FRCPath
Consultant Haematologist
The Royal Marsden Hospital
Sutton
Surrey
UK
利益声明
CD declares that she has no competing interests.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
参考文献
关键文献
Parry-Jones N, Joshi A, Forconi F, et al. Guideline for diagnosis and management of hairy cell leukaemia (HCL) and hairy cell variant (HCL-V). Br J Haematol. 2020 Dec;191(5):730-7.全文 摘要
Troussard X, Maître E, Paillassa J. Hairy cell leukemia 2024: update on diagnosis, risk-stratification, and treatment - annual updates in hematological malignancies. Am J Hematol. 2024 Apr;99(4):679-96.全文 摘要
Grever MR, Abdel-Wahab O, Andritsos LA, et al. Consensus guidelines for the diagnosis and management of patients with classic hairy cell leukemia. Blood. 2017 Feb 2;129(5):553-60.全文 摘要
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: hairy cell leukemia [internet publication].全文
Robak T, Matutes E, Catovsky D, et al. Hairy cell leukaemia: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2015 Sep;26(suppl 5):v100-7.全文 摘要
参考文献
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

鉴别诊断
- Chronic lymphocytic leukemia (CLL)
- Mantle cell lymphoma
- Prolymphocytic leukemia
更多 鉴别诊断指南
- NCCN clinical practice guidelines in oncology: hairy cell leukemia
- Guideline for diagnosis and management of hairy cell leukaemia (HCL) and hairy cell variant (HCL-V)
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