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Small Lymphocytic Lymphoma:
How does it differ from Chronic Lymphocytic Leukemia?
Aggressive or Indolent?
CD markers?
SLL = CLL
CLL has blood involvement w/lymphocytes
SLL has nodal involvement with lymphocytes

Indolent lymphoma, presenting around 60

CD 23+, CD5+
Histologic hallmark of SLL.
Proliferation centers (areas of mitotically active cells); pathognomonic for CLL/SLL
Proliferation centers are pathognomonic for ______.
CLL/SLL
SLL:
Transformative properties
May transform to Diffuse Large Cell (B Cell) Lymphoma

Just like CLL!
SLL:
Treatment
Same as CLL:
Fludarabine
Rituximab
Cyclophosphamide
This lymphoma comprises 50% of primary gastric lymphomas.
Marginal Zone Lymphoma (MALT)
Extra Nodal Marginal Zone Lymphoma:
AKA
Where does it occur?
What tissue states increases chance of developing this lymphoma? Examples.
MALT: Muscosal-Assocd Lymphoid Tissue

Anywhere there's mucosa, GI tract most commonly

Often within tissues with chronic inflammn (H pylori infection)
Salivary Gland (Sjogren's dz)
Thydois (Hashimoto's thyroiditis)
Extranodal Marginal Zone Lmphoma:
CD Markers
Hallmark Lesion
CD 20, Surface Ig

CD5-, CD10-

Hallmark lzn: Lymphoepithelial lesion (results from tissue invasion by lymphocytes)
Lymphoepithelial lesions are pathognomonic of ________.
MALT Lymphomas
Primary Gastric Lymphoma:
Treatment
Triple antibx to eradicate H pylori-->complete regression of lymphoma in most patients!
Mantle Cell Lymphoma:
Associated translocaiton, gene product
CD Markers
MALT = good prognosis (malt is good :) )
Mantle = Bad

Assocd w/ t(11;14)

AND CYCLIN 2 (c-myc)

CD5+
t(11;14):
Associated lymphoma
Pathophys
Assocd w/mantle cell lymphoma

Places bcl-1 gene under control of IgH-->overexpression of cyclin D1 (accelerates cell cycle)
Burkitt's Lymphoma:
Tumor Type
CD Markers
High grade (highly aggressive) B cell lymphoma

CD Markers = mature B Cell:
Ig Kappa or lambda

CD 19+ CD20+ CD10+
This specific lymphoma is assocaited with EBV infection.
African (Endemic) Burkitt's.
African (Endemic) Burkitt's Lymphoma:
Median age
Gene product
Presentation
African Burkitt's:
assocd w/EBV infection, translocation causing overexpression of c-myc.

Children!

Presentation:
Jaw/facial bone tumor-->spreads to BM and meninges (RAPID GROWTH RATE)
This lymphoma presents as a starry sky pattern.
Burkitt's Lymphoma
t(14;18):
Associated Disease
Gene Product
Follicular Lymphoma
bcl-2
t(8;14):
Associated Disease
Gene Product
Burkitt's Lymphoma
c-myc
Sporadic (Non-Endemic) Burkitt's Lymphoma:
Median age
Presentation
Children

Rapidly growing abdominal mass, ascites, renal, testis, ovarian involvement

But can spread to BM/CNS like endemic form.

Only 15-20% involve EBV

THIS IS FOUND IN THE US
Burkitt's Lymphoma:
Treatment
Intensive multiple agent chemo w/CNS prophylaxis

Risk: tumor lysis syndrome
HIV-Associated Lymphomas:
Examples
What do they have in common?
HIV-assocd lymphomas:
Diffuse Large B Cell Lymphoma (EBV present ~80%)

Burkitt's lymphoma (EBV ~30-50%)

Aggressive!!
Peripheral T-Cell Lymphomas:
Aggressive/Indolent
CD Marker
Aggressive

CD4+
Mycosis Fungoides:
CD Markers
Body Sites Affected
T-CELL LYMPHOMA

CD4+

Affects epidermis
Sezary Syndrome:
Advanced mycosis fungoides in which malignant lymphocytes circulate in peripheral blood.

Still has skin manifestations
Cerebriform cells are pathognomonic of _______.
Sezary Syndrome
Adult T-Cell Lymphoma:
Associated Virus
Risk of developing
Presentation
Manifestation of infection with HTLV-1, but only 2.5% chance of developing adult t-cell lymphoma/leukemia.

Latency period of 30+ years!

Presentation:
Skin lesions
LAD
Organomeg
HYPERCALCEMIA
ELEVATED LDH
Flower cells are pathognomonic of _________.
Adult T Cell Lymphoma
Anaplastic Large Cell Lymphoma:
CD Markers
Prognosis
CD 30+ only (not CD15+!!!_

Excellent prognosis w/chemotx

Typically seen w/young adults.