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lymphoma (lymphosarcoma)

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 1) infections: a) HIV1 infection b) Helicobacter pylori c) Epstein-Barr virus d) HTLV1 e) hepatitis B infection f) hepatitis C infection 2) post renal transplantation & cardiac transplantation 3) congenital immune deficiency syndromes a) severe combined immunodeficiency disease (SCID) b) Chediak-Higashi syndrome c) Wiskott-Aldrich syndrome d) ataxia-telangiectasia e) IgA deficiency 4) alpha heavy-chain disease 5) Hodgkin's disease (post-treatment) 6) Sjogren syndrome 7) celiac disease 8) inflammatory disorders: [1] a) rheumatoid arthritis b) Crohn's disease c) ankylosing spondylitis d) psoriatic arthritis e) plaque psoriasis 9) 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 - serology for viral infections [2,3] - hepatitis B serology - hepatitis C serology - HIV1 testing - HTLV1 serology - herpesvirus 8 serology - Epstein-Barr virus serology [3] - H pylori serology - flow cytometry can identify monoclonal cells & T-cell & B-cell biomarkers [3] - see ARUP consult [2] Special laboratory: - 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: - see staging of lymphoma 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 - aggressive B-cell lymphomas - diffuse large B-cell lymphoma, mantle cell lymphomas, Burkitt lymphoma - combined chemotherapy - prophylaxis for tumor lysis syndrome for Burkitt lymphoma [3]

Interactions

disease interactions

Related

lymphoid leukemia staging of lymphoma

Specific

Hodgkin's disease (Hodgkin's lymphoma) non-Hodgkin's lymphoma

General

hematologic malignancy (hematopoietic malignancy, hematologic cancer) lymphoid neoplasm (lymphoid leukemia/lymphoma)

Figures/Diagrams

Figures/diagrams/slides/tables related to lymphoma Malignant Lymphomas

References

  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. 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. 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
  4. 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: 21411774
  5. Lenz G, Staudt LM. Aggressive lymphomas. N Engl J Med. 2010 Apr 15;362(15):1417-29 PMID: 20393178
  6. 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: 25611108
  7. 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: 27032646