Non-Hodgkin’s Lymphoma

Pathophysiology

1) a group of 20 conditions all marked by clonal neoplastic transformation of lymphoid cells 2) initial phase involves infiltration of lymph nodes with a variable secondary leukemic phase in which malignant cells circulate in the bloodstream 3) can be either of B cell (90% of patients) or T cell origin 4) classified as low-grade or indolent (small cell or mixed cell), intermediate grade, or high grade (large cell or lymphoblastic)

Signs and Symptoms

1) fever and night sweats 2) painless adenopathy 3) enlarged spleen 4) enlarged liver

Histology/Gross Pathology

1) lymph nodes have a pale color on cut surface 2) obliteration of normal lymph node architecture with white pulp enlargement 3) adults – 40% of patients have a follicular/nodular pattern of lymph node involvement (better prognosis) and 60% have a diffuse pattern 4) children – virtually all patients have the diffuse pattern 5) infiltration of spleen, liver (discrete nodules occur in intermediate and high-grade neoplasms), and bone marrow occurs 6) some children have a “starry sky pattern” of lymph node infiltrate.

Associated Conditions

increased incidence with – 1) organ transplantation 2) HIV/AIDS 3) rheumatoid arthritis 4) sicca syndrome 5) Sjogren’s syndrome 6) Chediak-Higashi disease 7) Wiskott-Aldrich disease 8) ataxia telangiectasia 9) IgA deficiency 10) celiac disease 11) post-treatment for Hodgkin’s disease 12) lupus 13) Klinefelter’s syndrome 14) dioxin exposure 15) treatment with alkylating agents 16) herbicides 17) benzene

Biochemistry

1) Burkitt’s lymphoma – associated with Ebstein-Barr virus 2) follicular small cell cleaved variant – associated wtih bcl-2 gene and translocation of chromosomes 14 and 18 3) mature T cell lymphoma – associated with hypergammaglobulinemia 4) low-grade B cell variant – associated with monoclonal immunoglobulin

Inheritance/Epidemiology

1) increased in frequency in USA in past 50 years 2) slightly more common in men 3) median age of onset is 50 years

Treatment

1) chemotherapy 2) radiation

Tips for USMLE

if question mentions a 68 year-old man with a 2 month history of night sweats, fever, and painless adenopathy in the left axilla, think of a non-Hodgkin’s lymphoma


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Non-Hodgkin’s Lymphoma
Burkitt’s Lymphoma
Ataxia-telangiectasia