lymphoma (lymphosarcoma)
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Classification
- malignant lymphomas are a heterogeneous group of lymphoid cell neoplasms
- malignant transformation can occur at any stage of B-cell differentiation or T-cell differentiation
- lymphomas are solid tumors, usually involving lymphoid tissue but may involve other organs & may develop a leukemic phase
- lymphomas are divided into non-Hodgkin's lymphomas & Hodgkin's lymphomas
- of non-Hodgkin's lymphomas, ~90% are of B cell origin.
- lymphomas are further classifies as indolent, aggressive & highly aggressive[3]
Comparison of Hodgkin's & non-Hodgkin's lymphoma
| Feature | non-Hodgkin's | Hodgkin's |
|---|---|---|
| Cell of Origin | 90% B-cell | ? |
| - | 10% T-cell | - |
| Sites of disease | - | - |
| localized | uncommon | common |
| nodal spread | discontiguous | contiguous |
| extranodal | common | uncommon |
| mediastinal | uncommon | common |
| abdominal | common | uncommon |
| bone marrow | common | uncommon |
| B symptoms* | uncommon | common |
| Chromosomal translocation | common | ? |
| cure rate | 30-40% | > 75% |
* B symptoms: weight loss, fever, night sweats
Etiology
increased risk of secondary lymphoma
- infections:
- post renal transplantation & cardiac transplantation
- congenital immune deficiency syndromes
- alpha heavy-chain disease
- Hodgkin's disease (post-treatment)
- Sjogren syndrome
- celiac disease
- inflammatory disorders:[1]
- Chemical/drug exposure
- herbicides
- chlorinated organic compounds
- ferilizing materials used in farming[3]
- phenytoin, radiation, chemotherapy
Clinical manifestations
- enlarging lymphadenopathy
- systemic symptoms
Laboratory
- complete blood count (CBC)
- chemistry panel[3],
- including serum urate, serum lactate dehydrogenase
- erythrocyte sedimentation rate[3]
- beta-2 microglobulin in serum[3]
- immunoglobulins in serum[3]
- serology for infectious agents
- flow cytometry can identify monoclonal cells & T-cell & B-cell biomarkers[3]
- see ARUP consult[2]
Diagnostic procedures
- excisional biopsy (preferred) or core biopsy for deep lymphadenopathy
- do not use fine-needle aspiration[2]
- bone marrow biopsy for staging*[3]
- lumbar puncture for CSF analysis in patients with aggressive lymphoma involving testes, bone marrow, paranasal sinuses, or eye[3] (following diagnosis & staging)
* bone marrow biopsy not needed for Hodgkin's lymphoma or large cell lymphoma if PET scan & complete blood count are normal[3]
Radiology
- chest X-ray (first diagnostic imaging)
- CT scan (chest, abdomen & pelvis) & PET scan for staging[3]
- repeat PET scan after 2-3 cycles of chemotherapy for prognosis[3]
- a negative PET scan may obviate need for bone marrow biopsy in Hodgkin's lymphoma & large cell lymphoma
- PET scans are insensitive to very indolent lymphomas
Staging
Management
- patients with soft, mobile lymph nodes limited to 1 or 2 adjacent sites may be followed for 6-8 weeks without further workup[3]
- perisistent or enlarging lymphadenopathy, especially if associated with B symptoms needs further evaluation[3]
- indolent B-cell lyphomas
- follicular lymphoma, CLL, hairy cell leukemia are observed without therapy until symptomatic
- rituximab + multiagent chemotherapy when symptomayic vs allogeneic hematopoietic stem cell transplantation
- gastric MALT lymphomas are treated with eradication of H pylori
- follicular lymphoma, CLL, hairy cell leukemia are observed without therapy until symptomatic
- aggressive B-cell lymphomas
More general terms
More specific terms
Additional terms
References
- ↑ 1.0 1.1 FDA MedWatch: April 14, 2011 & Nov 4, 2011 Tumor Necrosis Factor (TNF) blockers, Azathioprine and/or Mercaptopurine: Update on Reports of Hepatosplenic T-Cell Lymphoma in Adolescents and Young Adults http://www.fda.gov/Safety/MedWatch/SafetyInformation/SafetyAlertsforHumanMedicalProducts/ucm251443.htm
- ↑ 2.0 2.1 2.2 2.3 ARUP Consult: Lymphoma Phenotyping The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/lymphoma-phenotyping
ARUP Consult: Leukemia/Lymphoma Phenotyping Evaluation by Flow Cytometry https://arupconsult.com/ati/leukemia-lymphoma-phenotyping-evaluation - ↑ 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 3.12 3.13 3.14 3.15 3.16 3.17 Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18, 19. American College of Physicians, Philadelphia 2012, 2015, 2018, 2021.
Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022 - ↑ Amador-Ortiz C, Chen L, Hassan A et al Combined core needle biopsy and fine-needle aspiration with ancillary studies correlate highly with traditional techniques in the diagnosis of nodal-based lymphoma. Am J Clin Pathol. 2011 Apr;135(4):516-24 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21411774
- ↑ Lenz G, Staudt LM. Aggressive lymphomas. N Engl J Med. 2010 Apr 15;362(15):1417-29 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20393178
- ↑ Frederiksen JK, Sharma M, Casulo C, Burack WR. Systematic review of the effectiveness of fine-needle aspiration and/or core needle biopsy for subclassifying lymphoma. Arch Pathol Lab Med. 2015 Feb;139(2):245-51. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/25611108
- ↑ Thanarajasingam G, Bennani-Baiti N, Thompson CA. PET-CT in Staging, Response Evaluation, and Surveillance of Lymphoma. Curr Treat Options Oncol. 2016 May;17(5):24. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/27032646