hematology - non-hodgkin lymphoma

lmyph nodes

located under arms, groin, neck, chest, and abdomen; secondary lymphoid organ that is the site where lymphocytes interact with antigens, enlarges during infection

non-hodgkin lymphoma

focused in secondary lymphoid organs and possible extramedullary areas and can have a leukemic phase

age, serum LDH, stage, extranodal involvement, ECOG performance

what is the scoring of prognosis based on for non-hodgkin lymphoma?

EBV

lymphomas are often associated with _____ infection

human T cell lymphotrophic virus type 1 (HTLV-1)

infectious agent: associated with development of ATLL, acquired through placenta, sex, blood transfusion, breast feeding; osteolytic lesions, hypercalcemia, immunosuppression

human T cell lymphotrophic virus type 1 (HTLV-1)

infectious agent: cells have flower cell appearance; irregular nucleus, clumped chromatin, basophilic cytoplasm, may loos like sezary cells

IL-2

what can be used as a tumor marker for human T cell lymphotrophic virus type 1 (HTLV-1)?

epstein barr virus (EBV)

infectious agent: associated with burkitt lymphoma, happens in post transplant, AIDS, and congenital immunodeficiency patients

burkitt lymphoma

cells have fine chromatin, basophilic cytoplasm, and vacuoles; BM and PB show starry sky appearance via the macrophages

blood and bone marrow

what are the main sites of burkitt lymphoma?

burkitt lymphoma

curable in over 90% of patients when found early, flow cytometry needs to be done quickly to maintain viability of sample

endemic burkitt lymphoma

mostly seen in children in equatorial africa, extranodal involvement is common in orbits and mandible with EBV

immunodeficiency associated burkitt lymphoma

seen with HIV, post transplantation, congenital ID; blood and bone marrow are main sites

sporadic burkitt lymphoma

mostly an abdominal disease with infiltration of BM and CNS

human herpes virus 8 (HHV-8)

infectious agent: coinfection with EBV; occurs in AIDS patients and patients with castleman disease

simian virus 40 (SV40)

infectious agent: seen with vaccine containment

agriculture

there is increased risk of NHL in _____ occupations as well as dark hairdye before 1980 and nitrite in drinking water

follicular lymphoma

neoplasm of germinal center B cells, second most common NHL that occurs in middle to old age adults, painless lymphadenopathy

follicular lymphoma

cells have deep nuclear clefts in circulating lymphoma cells, no cure and repeated relapse and remission until death

mantle cell lymphoma

low amount of NHL cases, median age is 68; aggressive and patients present with extensive lymphadenopathy; BM, blood, GI all involved

mantle cell lymphoma

cell appearance varies so diagnosis is difficult, requires demonstration of t(11, 14) seen by FISH or overexpression of cyclin D1 seen by IHC

diffuse large B cell lymphoma

most common NHL and usually disease of elderly, aggressive but curable; can arise spontaneously or as result of SLL, FL, or MZL

diffuse large B cell lymphoma

rapidly expanding lymphadenopathy, extranodal involvement in GI, testes, and bone

marginal zone lymphoma

indolent B cell lymphoma associated with chronic antigen stimulation with infection or autoimmunity

MALT lymphoma

MZL: associated with organs that do not have lymphoid tissue, like stomach, thyroid, salivary glands, skin, and small intestine

splenic marginal zone lymphoma

MZL: in white pulp of spleen, splenic lymph nodes, BM, and PB; massive splenomegaly and lymphocytosis, cytoplasmic projections

nodal marginal zone lymphoma

MZL: primarily in lymph nodes and associated with hepatitis C

mycosis fungoides

cutaneous T cell lymphoma with CD4+ cells, confined to skin until later stages, psoriasis like skin lesions

sezary syndrome

cutaneous T cell lymphoma with CD4+ cells, aggressive systemic disorder with PB involvement and worse prognosis, redness of skin, lymphadenopathy, sezary cells in skin, severe pruritis

anaplastic large cell lymphoma

T cell lymphoma that has large pleomorphic cells that express CD30; present with B symptoms, extranodal involvement in skin, bone, liver, and lung

ALK negative anaplastic lymphoma

associated with breast implants

peripheral T cell lymphomas

have absence of CD7 or CD5, catch all