Non-Hodgkin lymphomas (NHLs) are a heterogeneous group of malignancies of the lymphoid system.
Clinical history depends on the type of lymphoma and stage at presentation.
Diagnosis is confirmed by tissue sampling (e.g., lymph node, bone marrow, blood, skin).
Diffuse large B-cell lymphoma is the most common type of lymphoma, based on annual incidence.
Treatment is based on the histologic subtype and stage of the lymphoma; symptom severity informs decisions about when to start therapy.
R-CHOP-21 (rituximab plus cyclophosphamide, doxorubicin, vincristine, prednisone given on day 1 of a 21-day cycle) is the most commonly used chemotherapy regimen.
NHLs are a heterogeneous group of malignancies of the lymphoid system. This heterogeneity (>30 entities) stems from the functions of the lymphoid arm of the immune system, which employs various types of lymphocytes to defend against external (infectious) and internal (neoplastic) threats. Neoplasms derive from lymphocyte B-cell progenitors, mature B-cells, T-cell progenitors, or mature T cells.
Malignant lymphoid cells retain many qualities of their normal counterparts (B cells to produce immunoglobulins, T cells to travel to extranodal sites such as skin and central nervous system).
This topic does not cover mycosis fungoides/Sézary syndrome. For more information on these subtypes, see Cutaneous T-cell lymphoma.
History and exam
Key diagnostic factors
- night sweats
- weight loss
Other diagnostic factors
- massive splenomegaly in marginal zone lymphoma
- shortness of breath
- abdominal discomfort
- change in mental status
- focal neurologic deficits
- chest pain
- bone pain, back pain (bone involvement)
- skin lesions
- neurologic abnormalities on exam
- age >50 years
- male sex
- Epstein-Barr virus (EBV)
- human T-lymphocytotrophic virus-1 (HTLV-1)
- human herpesvirus-8
- Helicobacter pylori
- celiac disease
- hepatitis C virus (HCV)
- Sjogren syndrome
- Wiskott-Aldrich syndrome
- use of immunomodulatory drugs
- organ transplant
- Borrelia burgdorferi
- Coxiella burnetii
- Chlamydia psittaci
- rheumatoid arthritis
- systemic lupus erythematosus (SLE)
- common variable immunodeficiency
- Chediak-Higashi syndrome
- Klinefelter syndrome
- breast implants
1st investigations to order
- lymph node biopsy
- PET/CT scan
- PET scan
- CBC with differential
- blood smear
- skin biopsy
- bone marrow biopsy
- basic metabolic panel
- liver function tests
- lactate dehydrogenase (LDH)
Investigations to consider
- flow cytometry
- 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
- serum protein electrophoresis with immunofixation
- multiple-gated acquisition scan
- gene expression profiling
aggressive B-cell lymphomas
aggressive T-cell lymphomas
indolent B-cell lymphomas
indolent T-cell lymphomas
Julie E. Chang, MD
Department of Medicine, Section of Hematology & Oncology
University of Wisconsin School of Medicine and Public Health
JEC has been paid for time served on advisory boards for MorphoSys, BeiGene, and Genentech. JEC has research funding from Genentech and Celgene.
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.
EC, CHN, MO, BK, and KBH declare that they have no competing interests. MP has received sponsorship from Sanofi and Janssen to attend conferences.
Leonidas G. Koniaris, MD
Associate Professor Surgery
Department of Cell Biology and Anatomy
University of Miami School of Medicine
LGK declares that he has no competing interests.
Shankaranarayana Paneesha, MD, MRCP, FRCPath
Department of Haematology and Stem Cell Transplantation
SP declares that he has no competing interests.
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