Last reviewed: 24 Aug 2021
Last updated: 15 Mar 2021

Summary

Definition

History and exam

Key diagnostic factors

  • night sweats
  • weight loss
  • fatigue/malaise
  • fever

Other diagnostic factors

  • lymphadenopathy
  • massive splenomegaly in marginal zone lymphoma
  • hepatomegaly
  • shortness of breath
  • cough
  • abdominal discomfort
  • headache
  • change in mental status
  • dizziness, ataxia
  • chest pain
  • bone pain, back pain (bone involvement)
  • jaundice
  • pallor
  • purpura
  • skin lesions
  • neurologic abnormalities on exam

Risk factors

  • age >50 years
  • male sex
  • Epstein-Barr virus (EBV)
  • human T-lymphocytotrophic virus-1 (HTLV-1)
  • human herpesvirus-8
  • Helicobacter pylori
  • celiac disease
  • HIV
  • hepatitis C virus (HCV)
  • Sjogren syndrome
  • Wiskott-Aldrich syndrome
  • ataxia-telangiectasia
  • organ transplant
  • Borrelia burgdorferi
  • rheumatoid arthritis
  • systemic lupus erythematosus (SLE)
  • common variable immunodeficiency
  • Chediak-Higashi syndrome
  • Klinefelter syndrome
  • pesticides
  • phenoxyherbicides
  • breast implants

Diagnostic investigations

1st investigations to order

  • CBC with differential
  • blood smear
  • lymph node biopsy
  • skin biopsy
  • bone marrow biopsy
  • basic metabolic panel
  • liver function tests
  • lactate dehydrogenase (LDH)

Investigations to consider

  • flow cytometry
  • immunohistochemistry
  • polymerase chain reaction for tumor markers
  • immunoglobulin gene rearrangement studies
  • cytogenetics studies with or without fluorescence in situ hybridization (FISH)
  • hepatitis B and C serology
  • HIV antibody
  • lumbar puncture
  • colonoscopy
  • CT scan
  • multiple-gated acquisition scan
  • echocardiography
  • PET scan

Emerging tests

  • gene expression profiling

Treatment algorithm

Contributors

Authors

Esther Chan, MD

Consultant

Department of Haematology-Oncology

National University Cancer Institute

Singapore

Disclosures

EC declares that she has no competing interests.

Chin Hin Ng, MD, MRCP, FRCPath

Consultant

Department of Haematology-Oncology

National University Cancer Institute

Singapore

Disclosures

CHN declares that he has no competing interests.

Melissa Ooi, MB BCh, BAO, MRCP(I), FRCPath, PhD

Consultant

Department of Haematology-Oncology

National University Cancer Institute

Singapore

Disclosures

MO declares that she has no competing interests.

Michelle Poon, MBBS, MRCP, FRCPath

Senior Consultant

Department of Haematology-Oncology

National University Cancer Institute

Singapore

Disclosures

MP has received sponsorship from Sanofi and Janssen to attend conferences.

Acknowledgements

Dr Esther Chan, Dr Chin Hin Ng, Dr Melissa Ooi, and Dr Michelle Poon would like to gratefully acknowledge Dr Boris Kobrinsky and Dr Kenneth B. Hymes, previous contributors to this topic.

Disclosures

BK and KBH declare that they have no competing interests.

Peer reviewers

Leonidas G. Koniaris, MD

Associate Professor Surgery

Department of Cell Biology and Anatomy

University of Miami School of Medicine

Coral Gables

FL

Disclosures

LGK declares that he has no competing interests.

Shankaranarayanan Paneesha, MD, MRCP, FRCPath

Consultant Haematologist

Department of Haematology and Stem Cell Transplantation

Heartlands Hospital

Birmingham

UK

Disclosures

SP declares that he has no competing interests.

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