LYMPHOMA

-Indolent (follicular, small cell) or Aggressive (diffuse, large cell)

-Most lymphoid neoplasms resemble some recognizable stage of B or T-cell differe
ntiation
-Solid tumors of immune system (At lymph nodes, mediastinal region)
-Majority are B-cell lymphomas, can have T-cell, and NK-cell lymphomas (rare)
-Diffuse Large Cell Lymphoma, Burkitt Lymphoma, Follicular Lymphoma, and Hodgkin
's Lymphoma are common
-Causes: genetic alterations, infection, antigen stimulation, immunosuppression
(T-cells can't kill the bad B-cells)
-FoxO and Wnt genes are implicated
-Complicaitons: bone marrow failure, CNS infiltration, hemolysis, thrombocytopen
ia, compression of structures, pleural/pericardial effusion, SVC syndrome, ascit
es.
-EBV-->Burkitt's lymphoma, Hodgkin's disease; HTLV-1-->Adult T cell leukemia/lym
phoma; HIV-->Diffuse Large Cell Lymphoma, Burkitt's lymphoma
-Mantle Cell Lymphoma-->BCL-1 = cyclin D-1 which phosphorylates the RB, releases
E2F-1, which activates genes in DNA.
-Follicular Cell Lymphoma-->BCL-2
-Diffuse Large Cell Lymphoma-->p53, BCL-56
-Burkitt's lymphoma-->C-MYC
-Non-Hodgkin's Lymphomas (62.4%)= Diffuse large B cell is majority of this
-Hodgkins lymphoma= localized single gorup of nodes, better prognosis, Reed-Ster
nburg Cells, bimodal distribution, strongly associated with EBV, constitutional
symptoms
-NHL= multiple nodes, extranodal involvement, majority involve B-cells, peak inc
idence for certain subtypes is 20-40 year olds, associated with HIV, fewer const
itutional symptoms
-Indolent: incurable, long natural history, slow cellular progression
-Aggressive: curable, short natural history, disease of rapid cellular progressi
on; responds well to chemo.
-Follicular lymphoma: most common type of indolent lymphoma, usually widespread
at presentation, often asymptomatic, incurable, arises from germinal center B ce
lls
-Indolent NHL treatment: early treatment does not prolong survival, watchful wai
ting, radiation to involved fields, single agent chemo or combination, Rituxumab
for
CD20+, interferion, bone marrow transplantation.
-Aggressive NHL: B symptoms= night sweats, fever, chills; average age 64. Diffus
e Large B-cell Lymphoma: accounts for 13% of lymphomas, is most aggressive, usua
lly asymptomatic
-Burkitt's Lymphoma:"Starry-Sky" appearance, C-MYC (transcription factor); assoc
iated with immunodeficiency-->HIV; endemic and sporadic in African children-->na
sopharyngeal carinoma, mandibular mass
-Aggressive NHL treatment: cyclophosphamide, doxorubicin, vincristine, prednison
e, and Rituxumab
-Hodgkin's Lymphoma: Reed-Sternberg cell; Subtypes: nodular sclerosis,...; Male>
Female, strongly correlated with EBV, HIV also associated
Lymphadenopathy, mediastinal mass, B-symptoms, hepatosplenomegaly, alcoh
ol-induced pain
Adverse prognostic features: >3 nodal sites, bulky disease, ESR>50, B-sy
mptoms, male, age>40,...
Treatment: 85% curable, depends on stage, prognostic factors and co-morb
idities, Stage1-2: radiation, chemo, or combo; Bulky 1-2: combo; Stage2-4: adria
mycin, bleomycin, vinblastine, dacarbazine
Complications: infertility, cardiac disease, lung disease (bleomycin), s
econdary malignancy: AML, skin, lung, thyroid
-Bleomycin-->pulmonary fibrosis (restrictive lung disease)
-Doxorubicin-->dilated cardiomyopathy