ICL 11.2: Lymphoma II Flashcards

Question

what pattern is Burkitt lymphoma and why?

Answer 1

Burkitt lymphoma has a short doubling time (shortest in all human cancers) = excessive tumor debris it's cleaned up by macrophages (tingible bodies). they are light in background of dark blue – starry sky pattern slide 17

Answer 2

t(8;14) 80% of cases or t(2;8) 15% or t(8;22) 5% C-myc oncogene on 8, kappa light chain on 2, lambda light chain on 22, heavy chain on 14. c-myc is anti-apoptotic transcription factor that induces proliferation

Answer 3

``` CD19+ CD20+ CD22+ CD10+ BCL6+ IgM+ TdT- ```

Answer 4

ALL and BL look similar morphologically TdT (terminal deoxynucleotidyl transferase is found in lymphoblasts ALL is positive and BL is negative for TdT

Answer 5

blast-like cells with multiple cytoplasmic vacuoles vacuoles make the cells look like there's white holes in them slide 18

Answer 6

1. endemic African type 2. sporadic American type 3. HIV-associated

Answer 7

large mass under jaw all infected with EBV age 4-7 50% of pediatric cancers in equatorial Africa

Answer 8

abdominal location esp. ileocecum low EBV association <35 years old M>>F 1-2% of all lymphomas

Answer 9

low EBV young, immunodeficient adults 2nd most common HIV+ NHL

Answer 10

EBV unclear EBV role in pathogenesis but EBV may induce B cell proliferation and expansion during which chromosomal translocations are likely to arise given the equatorial location of endemic BL, the same location for malaria, there is some thought that malaria may prime B cells and make them more susceptible to EBV infections

Answer 11

high dose chemotherapy and should be started within 48 hours of diagnosis often includes intrathecal treatment (methotrexate or cytarabine) as well as rituximab (anti-CD20)

Answer 12

Anaplastic large cell lymphoma

Answer 13

t(2;5) gene abnormality produces constitutively active tyrosine kinase ALK gene rearrangement is diagnostic!

Answer 14

children/young adults

Answer 15

anaplastic, bizarre large cells CD30+

Answer 16

majority associated with textured implant usually ALK negative, but has good prognosis treatment is total capulectomy, removal of the implant consider removing the contralateral implant

Answer 17

1. horseshoe nucleus with lots of cytoplasm 2. embryo nucleus - looks like a kidney bean slide 23

Answer 18

cutaneous T-helper CD4 lymphoma with slow, indolent course skin resident effector memory T cells are the cells of origin of MF cerebriform nucleus in sezary cells (slide 29) skin changes-inflammatory (patch), plaque, tumor collections of these cells in the epidermis = Pautrier microabscess (slide 27/28) leukemic phase = Sezary syndrome, associated with generalized exfoliative erythroderma and usually only seen in older individuals

Answer 19

associated with generalized exfoliative erythroderma and usually only seen in older individuals characterized by the presence of atypical T cells in skin, lymph nodes and peripheral blood thought to arise from thymus memory T cells while skin resident effector memory T cells are the cells of origin of MF

Answer 20

nasal type, is a rare, fast-growing, highly aggressive type of NHL it can start in T cells, but it develops most often in natural killer (NK) cells this type of NHL is called extranodal because it develops in organs or tissues other than the lymph nodes blood vessel wall destruction leads to extensive ischemic necrosis = angioinvasive and angiodestructive

Answer 21

high EBV association

Answer 22

+ EBV and usually CD56+, CD2+, CD3-

Answer 23

combined chemo and radiation

Answer 24

no in the US they also have a worse prognosis than B-cell lymphomas

Answer 25

not classified into small cell or large cell skin involvement is common normal T-cells are +CD2,3,5,7, and either CD4 or 8 but lymphomas commonly have antigen dropout and/or coexpression of CD4 and CD8 or loss of both CD4 and CD8 all have diffuse pattern

Answer 26

all are peripheral (mature) T-cells except precursor T-ALL/lymphoblastic lymphoma Tdt positive ONLY in lymphoblastic lymphoma

Answer 27

precuror-T lymphoblastic leukemia/lymphoma = T-cell ALL

Answer 28

caused by HTLV-1 (human T-cell leukemia/lymphoma virus 1) endemic in Japan, Caribbean basin

Answer 29

caused by HTLV-1 leukemic variant is usually rapidly progressive and fatal - skin lesions - lymphadenopathy - lymphocytosis with cloverleaf or floret nucleus - hypercalcemia

Answer 30

lymphocytes with cloverleaf or floret nucleus slide 37 nucleus literally looks like a 3 leaf clover

Answer 31

there's T-cell (CD3+) or NK-cell (CD56+) variants STAT3 mutation common = transcription factor

Answer 32

usually indolent course lymphocytosis and splenomegaly neutropenia and anemia sometimes pure red cell aplasia in BM some cases of Felty syndrome have underlying LGL

Answer 33

common in large granular lymphocytic leukemia (LGL) Pure red cell aplasia (PRCA) is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow

Answer 34

some cases of Felty syndrome have underlying LGL Felty syndrome (FS), also known as ''super rheumatoid'' disease, is a severe form of rheumatoid arthritis (RA), characterized by a triad of RA, splenomegaly and neutropenia, resulting in susceptibility to bacterial infections

Answer 35

t(11;14) BCL1 on chromosome 11, translocation to 14 increases expression BCL1 codes overexpression of Cyclin D1

Answer 36

t(14;18) BCL2 – inhibits pro-apoptotic protiens, located on chromosome 18 and moves to heavy chain on 14 BCL2 over expression allows for lymphoma proliferation

Answer 37

t(8;14) c-myc (chromosome 8) over expression by translocation to heavy chain on 14 makes up 80% of BL translocation, other less common translocations include t(2;8) 15%; or t(8;22) 5%

Answer 38

t(2;5) ALK gene rearrangements

Answer 39

hematopoiesis dysfunction that results in the bone marrow filled with dysplastic cells that can’t leave the marrow, resulting in systemic cytopenias patients present with symptoms of bone marrow failure like weakness, fatigue, bleeding

Answer 40

high risk for transformation into AML blast count must be from 0 – 19% >20% is AML blast count can be prognostic

Answer 41

treatment includes observation, chemotherapy, high dose combination chemotherapy and/or stem cell transplant

Answer 42

malignant proliferation of plasma cells in bone marrow malignant plasma cells inhibit formation of normal polyclonal immunoglobulin = increase risk for infection monoclonal IgG or IgA creates an M spike in SPEP - monoclonal immunoglobulin is closely correlated to amount of tumor mass present

Answer 43

Serum Protein Electrophoresis (SPEP) Urine Protein Electrophoresis (UPEP) Bone Marrow Biopsy (at least 10% plasma cells) Blood Smear Free Ligh Chain Assay (FLC) Bone survey Urine Immunofixation Electrophoresis (UIFE) Serum immunofixation Electrophoresis (SIFE)

Answer 44

Bence-Jones proteins they're light chain only proteins that are small enough to be filtered into the urine

Answer 45

1. Russel bodies – large, eosinophilic, cytoplasmic inclusions thought to represent distended endoplasmic reticulum (slide 45) 2. Dutcher bodies – Excess cytoplasmic immunoglobulin invaginating into the nucleus give the appearance of intranuclear inclusions (slide 47) 3. Rouleaux Formation – increased immunoglobulin in serum interferes with normal repellant force that pushes red cells apart, allowing them to stack upon each other (slide 46)

Answer 46

plasma cell disorder >10% plasma cells M-spike, but no other end organ damage or biopsy proven amyloidosis to confirm MM MGUS --> smoldering myeloma --> MM

Answer 47

plasma cell disorder < 10% plasma cells; small M-spike no lytic bone lesions, anemia, kidney dysfunction, at risk of developing MM, since MGUS can lead to multiple myeloma at the rate of about 1.5% a year, yearly monitoring is recommended MGUS --> smoldering myeloma --> MM

Answer 48

Light chain deposition disease (LCDD) – MM but not amyloidosis in kidney Heavy chain deposition disease (HCDD – MM, produce both light and heavy immunoglobulin chains; unable to form amyloid fibrils so they don’t have hallmark histologic features of amyloidosis –identified via immunofluorescence

Answer 49

light chain deposition disease it's the deposition of monoclonal light chains in multiple organs. It is a rare disease characterized by deposition of non-amyloid immunoglobulin light chains, which do not stain with Congo red LCDD is categorized as a monoclonal immunoglobulin deposition disease a single clone of plasma cells is responsible for overproduction of kappa chains and, very rarely, lambda chains a monoclonal population of plasma cells can be detected in the bone marrow, and an altered serum-free light chain ratio is present in 25% of patients, an abnormal serum free light chain ratio is noted, even without an abnormal finding with serum and or urine electrophoresis with immunofixation incidence of LCDD is unknown. The median age at diagnosis is 58 years, and it is more common in M than F

Answer 50

Heavy-chain deposition disease is the least common non-organized monoclonal immunoglobulin deposition disease Unlike the more common LCDD an association of multiple myeloma or plasma cell dyscrasias with HCDD is less common, with only 7 of 23 (30%) reported cases being associated with development of demonstrable monoclonal plasmacytosis. nodular glomerulosclerosis is the classic glomerular pattern of injury of all monoclonal immunoglobulin disorders, and the disease (though suspected on light microscopy) can be conclusively diagnosed only by an extended panel of immunofluorescence that includes antibodies against heavy-chain isotypes

Answer 51

characterized by decreased number of monoclonal plasma cells in the BM compared to MM amyloid has an affinity for visceral organs and causes end organ damages Congo Red stain is used to identify amoyloid deposits

Answer 52

target CD20 used for treatment of CLL, NHL

Answer 53

target CD30 used for HL, primary cutaneous large cell lymphoma, CD30+ MF

Answer 54

proteasome inhibitor used for tx of MM, amyloidosis

Answer 55

nitrogen mustard chemotherapy used for tx of CLL, MM, NHL

ICL 11.2: Lymphoma II Flashcards

(80 cards)