Lymphomas

Question 1

Lymphoma classification

Answer 1

Hodgkins’s (classical, non-classical) and Non-Hodgkin’s (B cell (85%),T cell (15%) precursor or mature)

Question 2

Classical Hogkins’s lymphoma

Answer 2

1% of all new cancers in US, bimodal distribution, highly curable; affects men except for nodular sclerosing type; strongly associated with EBV; characteristic Reed Sternberg cells (CD15+, CD30- B cell origin) that only makes up 1-5% of the tumor

Question 3

Non-classical Hogkins’s lymphoma (nodular lymphocyte predominant Hogkins Disease, NLPHD)

Answer 3

5% of Hogkin’s lymphoma, characterized by nodular and polymorphous proliferation of L & H cells (Reed-Sternberg variants); CD15-,CD30-, CD20+, CD79a+, Bcl-6+, CD45+

Question 4

HIV associated lymphoproliferative disorders

Answer 4

100x greater risk of lymphoma, B cell lineage -> Large cell lymphoma, Burkitt-like lymphoma, primary effusion lymphoma (usually do not express B cell antigens like CD19-, CD20-, but CD45+), associated with HHV-8 and EBV)

Question 5

Non-Hogkin’s lymphoma

Answer 5

muliple, peripheral nodes, incidence usually between 20-40 yrs old

Question 6

Precursor B-lymphoblastic lymphoma

Answer 6

Precursor B cell Non-Hogkin’s lymphoma; predominantly seen in children and young adults; cells show high nuclear cytoplasmic ratio; CD10+, CD19+, CD20+, CD34+, Tdt+, CD20+

Question 7

Precursor B-lymphoblastic lymphoma cytogenetics

Answer 7

Hyperdiploidy (> 50 chromosomes), t(12;21) -> good prognosis; T(4;11), t(9;22) -> poor prognosis

Question 8

CLL/ SLL (small lymphocytic lymphoma)

Answer 8

most common adult leukemia; smudge cells (broken down nucleus of CLL cells), CD5+, CD20+

Question 9

CLL/ SLL (small lymphocytic lymphoma) cytogenetics

Answer 9

Del 13q12-14 -> favorable prognosis; Del 11q, 12+, del 17p -> unfavorable prognosis

Question 10

Hairy cell leukemia

Answer 10

mature B cell Non-Hogkin’s lymphoma; cell have hair-like projections, cause marrow fibrosis -> massive splenomegaly, pancytopenia, monocytopenia; CD19+, CD20+, CD25+, CD103+

Question 11

Hairy cell leukemia treatment

Answer 11

Interferon, cladribine (2-CDA), pentostatin (deoxycoformycin)

Question 12

Extra nodal Marginal Zone Lymphoma/MALToma

Answer 12

often related to H. pylori; presence of (11;18) and IgH gene rearrangement

Question 13

Follicular Lymphoma

Answer 13

mature small B cell Non-Hogkin’s lymphoma, indolent; Characteristic cleaved/twisted nuclei, resembling raisins; classic paratrabecular aggregates are seen in involved bone marrows; CD10+, Bcl-2+, Bcl-6+

Question 14

Follicular Lymphoma cytogenetics

Answer 14

T(14;18)

Question 15

Mantle cell lymphoma

Answer 15

mature small B cell Non-Hogkin’s lymphoma, most aggressive; typically affects elderly men; CD5+, cyclin D1+

Question 16

Mantle cell lymphoma cytogenetics

Answer 16

T(11;14) (bcl-1;IgH)

Question 17

Diffuse Large B-Cell Lymphoma (DLBCL)

Answer 17

mature large B cell Non-Hogkin’s lymphoma; common, 40% of all lymphomas, most in > 50 yrs; large cells usually have nuclei twice that of a small lymphocyte, and are typically bigger than tissue macrophage nuclei; c-Myc gene rearrangement in DLBCL; Bcl-2+, Bc1-6+, CD10+

Question 18

Burkitt Lymphomas

Answer 18

mature medium B cell Non-Hogkin’s lymphoma, aggressive; associated with EBV, lesions; “starry sky” (sheets of lymphocytes with interspersed macrophages); SmIg+, CD10+, c-Myc rearrangement or overexpression

Question 19

Precursor T-lymphoblastic lymphoma

Answer 19

Precursor T cell Non-Hogkin’s lymphoma; affects young adults; stage II thymocyte express CD3, CD4, CD8, CD1a, Tdt

Question 20

Anaplastic Large Cell Lymphoma (ALCL)

Answer 20

mature T cell Non-Hogkin’s lymphoma, curable; involves lymph nodes and extranodal sites, large eccentric nucleoi mimic Reed Sternberg cells; ALK-1+, t(2;5)

Question 21

Adult T cell lymphoma

Answer 21

mature T cell Non-Hogkin’s lymphoma, incurable affecting Japanese, Carribean, and West Africans; skin lesion, lytic bone lesions, hypercalcemia; associated with HTLV-1 infection; CD4+, CD25+, CD7-

Question 22

Mycosis Fungoides/Sezary Syndrome

Answer 22

mature T cell Non-Hogkin’s lymphoma, incurable; epidermotrophic cutaneous T cell lymphoma (skin plaques) and Pautrier’s micro-abscess; CD3+, CD4+, CD7-, CD8-

Question 23

Peripheral T cell lymphoma

Answer 23

mature T cell Non-Hogkin’s lymphoma, incurable; DNA analysis reveals rearrangement of at least one T cell receptor locus