Summary
Definition
History and exam
Key diagnostic factors
- B symptoms (fever, night sweats, weight loss)
- fatigue/malaise
Other diagnostic factors
- lymphadenopathy
- splenomegaly
- hepatomegaly
- shortness of breath
- cough
- abdominal discomfort
- headache
- change in mental status
- focal neurological deficits
- chest pain
- bone pain, back pain
- jaundice
- pallor
- purpura
- skin lesions
- neurological abnormalities on examination
- breast pain
- swelling or mass in the breast
Risk factors
- age >50 years
- male sex
- Epstein-Barr virus (EBV)
- human T-lymphocytotrophic virus-1 (HTLV-1)
- human herpesvirus-8 (Kaposi's sarcoma-associated herpesvirus)
- Helicobacter pylori
- coeliac disease
- HIV
- hepatitis C virus (HCV)
- Sjogren syndrome
- Wiskott-Aldrich syndrome
- ataxia-telangiectasia
- use of immunomodulatory drugs
- organ transplant
- Borrelia burgdorferi
- Coxiella burnetii
- Chlamydia psittaci
- Campylobacter jejuni
- rheumatoid arthritis
- systemic lupus erythematosus (SLE)
- common variable immunodeficiency
- Chediak-Higashi syndrome
- Klinefelter syndrome
- pesticides
- phenoxyherbicides
- breast implants
Diagnostic investigations
1st investigations to order
- FBC with differential
- peripheral blood smear
- comprehensive metabolic panel (including liver function tests [LFTs])
- serum lactate dehydrogenase (LDH)
- uric acid
- lymph node biopsy
- biopsy (extranodal sites e.g., brain, skin)
- bone marrow biopsy and aspirate
- immunohistochemistry
- flow cytometry
- fluorodeoxyglucose (FDG)-PET/CT scan
Investigations to consider
- core needle biopsy
- fine-needle aspiration (FNA) biopsy
- genetic testing
- hepatitis B and C serology
- HIV testing
- Epstein-Barr virus (EBV) testing
- human T-cell lymphotropic virus (HTLV) testing
- MRI (brain, spine)
- ultrasound (breast, axilla)
- lumbar puncture
- endoscopy and colonoscopy
- serum protein electrophoresis with immunofixation
- quantitative immunoglobulins
- serum beta-2 microglobulin
- Helicobacter pylori testing
- multigated acquisition (MUGA) scan
- echocardiography
Treatment algorithm
aggressive B-cell lymphomas
aggressive T-cell lymphomas
indolent B-cell lymphomas
indolent T-cell lymphomas
Contributors
Authors
Julie E. Chang, MD
Associate Professor
Department of Medicine, Section of Hematology & Oncology
University of Wisconsin School of Medicine and Public Health
Madison
WI
Disclosures
JEC has been paid for time served on advisory boards for MorphoSys, BeiGene, and Genentech. JEC has research funding from Genentech and Celgene.
Acknowledgements
Dr Julie Chang would like to gratefully acknowledge Dr Esther Chan, Dr Chin Hin Ng, Dr Melissa Ooi, Dr Michelle Poon, Dr Boris Kobrinsky, and Dr Kenneth B. Hymes, previous contributors to this topic.
Disclosures
EC, CHN, MO, BK, and KBH declare that they have no competing interests. MP has received sponsorship from Sanofi and Janssen to attend conferences.
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.
Shankaranarayana 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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