- Affects elderly M>F, few symptoms, long survival w/o Tx

- diffuse, mature small lymphocytes + smudge cells --> tumors of LN's, marrow and/or spleen

- CD19 (B-cell), CD20, CD5, CD23 positive

- Stages 0-IV, B Cell (90%), Hypo-B cell

- Rarely leads to Aggressive lymphoma (Richter's)

- Worse survival: 17p- or 11q-, nonmutated Ig VH, CD38, ZAP-70 (tyrosine kinase)

- monoclonal B-cell lymphocytosis may be precursor

Small/Chronic Lymphocytic Lymphoma (SLL/CLL)

 0 = peripheral/marrow lymphocytosis (12 yr)

I = 0 + lymph node enlargement (8 yr)

II = 0 + splenomegaly (6 yr)

III = I + II (2 yr)

IV = III + anemia (Hgb<10g) + thrombocytopenia (platelets <100x10^9/L)

Staging- Binet simplification of Rai method

- MUST distinguish from SLL/CLL b/c worse prognosis if given SLL Tx

- Massive splenomegaly, marrow infiltration, pancytopenia

- Malignant B-cells w/ ragged edges (+) for acid phosphatase (tartrate resistent [TRAP])

- Tx w/ splenectomy, alpha interferon, chemotherapy

Hairy cell leukemia (HCL)

- CD19,20,5+ but CD23-

- Very aggressive (~survival = 3 yrs)

- Cannot usually control w/ chemotherapy

- t(11;14) --> bcl-1 or PRAD-1 oncogene --> cyclin D1

Mantle cell lymphoma

- small cleaved follicular center cells (low proliferation)

- Always B-cell, CD19/20+ & CD10+, CALLAntigen+

- t(14;18) of bcl-2 gene --> immortal w/o apoptosis (normal do not express bcl-2)

- 75% involve marrow, widely disseminated

- Survival~ 7-9 yrs, but progresses to higher grade w/ >50% larger cell type & diffuse pattern

Follicular lymphomas

- from marginal zone B-cells outside mantle layer

- Nodal, Splenic, Extra-nodal (MALT) locations of origin

- prefers to stay at site of origin (disseminate late)

- better prognosis w/o LN involvement

- H. pylori - Stomach MALToma

- Chlamydia psittaci - Ocular adnexal MALToma

- Borrelia burgdoferi - Cutaneous MALToma

- Campylobacter jejuni- IPSID (small intestine)

HCV - lympoplasmacytic lymphoma

- Usually indolent

Marginal Zone lymphoma

- B-cell (CD19/20+) w/ spectrum of aggressiveness

- immunoblastic lymphoma or sarcoma --> diffuse (50% from immunologic disorder)

- Activated B cell, Germinal center, Type 3

- 50-60% can be cured

- ABC type/Bcl-2 expression --> poorer prognosis

Diffuse large B-cell lymphoma

- high grade B-cell lymphoma of children

- CD19/20/10+, CALLA+

- "Starry sky" = scattered, benign histiocytes w/ clear cytoplasm on darker malignant blasts

- t (8;14) [c-myc gene] - follows Ig promoter --> c-myc product after each immunoglobulin chain

- low LN involvement, can be leukemic

- rapidly fatal w/o Tx, chemotherapy effective

- African- = EBV + jaw bone/abd. viscera (ovaries in F)

- US/Europe = less EBV, more GI tumors

Burkitt (small non-cleaved cell) lymphoma

- precursor T-cell neoplasm

- 40% of childhood lymphomas

- very aggressive but now w/ good therapy

- mediastinal involvement (thymus) + CNS/skin

- CD3+ & TdT+

Lymphoblastic lymphoma/leukemia (T-cell)

- peripheral T-cell neoplasm

- T cell malignancy (CD4+) in skin

- non-specific skin lesions --> tumors

- clusters of malignant cells in epidermis (Patrier abscesses) --> to the viscera late

- children

Mycosis fungoides

- peripheral T-cell neoplasm

- T cell malignancy (CD4+) in skin

- diffusely infiltrate skin causing erythroderma

- found in peripheral blood

- children

Sezary's syndrome

- peripheral T-cell neoplasm

- malignant T-cells w/ CD3/CD7+, TdT-

- intermediate-large cells

- children

Peripheral T-cell lymphoma

(not otherwise specified)

- peripheral T-cell neoplasm

- results from HTLV-1 viral infection

- endemic in Japan/Caribbean

- CD4+, large cells w/ skin lesions & hypercalcemia

- high grade

Adult T-cell leukemia/lymphoma

- Classical Hodgkin's Lymphoma

- Low RS cells w/ many small lymphocytes, eosinophils

- CD30/CD15+, CD20/CD45-

- Male w/ peripheral LN's involved

Present w/ early stage (I & II)

- Best prognosis of classic Hodgkin's Lymphoma

Lymphocyte rich HL

- Classical Hodgkin's Lymphoma

- Lots of RS cells, few small lymphocytes

- elderly male w/ retroperitoneal/visceral dx

profound failure of cellular immunity (like AIDS)

- cure rate <40%

Lymphocyte Depletion HL

- Classical Hodgkin's Lymphoma

- many small lymphocytes and RS cells

- middle age pt w/ cervical and chest/abd. dx

- cure rate 75%

Mixed cellularity HL

- Classical Hodgkin's Lymphoma

- thick bands of collagen

- "lacunar cells" = RS within clear spaces

- young adults w/ cervical/mediastinal dx

- 85% cure rate

- most common HL

Nodular sclerosis HL

- Non-Classical Hodgkin's Lymphoma

- many small lymphocytes, few RS cells

- lymphocytic-histiocytic (L&H type)

- B-cell lymphoma due to CD20/CD45+

- cervical or inguinal nodes in young adult females

- greater then 90% cure rate

- small chance to progress to large B-cell lymphoma

Nodular Lymphocyte predominance HL

(NLPHL)