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Flashcards in Glossary Deck (135):
1

ABC type

activated B cell type. A subset of diffuse large B cell lymphomas that have a non germinal center B cell pattern of gene expression. These lesions do worse than the GCB type of diffuse large B cell lymphoma with current therapies

2

acute

a hematopoietic (myeloid, erthyroid, megakaryocytic) or lymphoid precursor blocked at a very early (blast or promyelocyte) stage of differentiation.

3

ALCL

anaplastic large cell lymphoma

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ALK1

anaplastic lymphoma kinase

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allogenic stem cell transplantation

transfer of hematopoietic stem cells from donor after conditioning the recipient with chemotherapy or radiation. Donors are typically HLA matched. Stem cells collected by bone marrow aspiration or mobilization, which requires the administration of GF to cause spillage of markedly left shifted cells, including CD34 stem cells, into the peripheral blood

6

amyloid

aggregates of proteins or protein fragments of various types. Despite their unrelated nature, these tend to settle out in deposits with a beta pleated structure. Identified histologically via Congo red stain. produce apple green birefringence under polarized light.

7

amyloidosis

the clinical disorder produced by systemic amyloid deposition. Prominent are the effects of amyloid deposition on the glomerular filtration apparatus

8

Ann Arbor staging system: stage 1

neoplasm limited to single site, +/-B (constitutional) symptoms (night sweats, weight loss, fevers)

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Ann Arbor staging system

stage 1-4. used clinically to gauge extent of neoplastic spread. initially conceived for hodgkins lymphoma but also used in non hodgkin lymphoma. corresponds to the TNM staging system used for solid tumors.

10

ATLL

adult T cell leukemia/lymphoma

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atypia

cytologic or histologic abnormalities that suggest a cell is or is becoming neoplastic. Ex: the features of plasma cell atypic include bizarre enlarged nuclei, prominent nucleoli, numerous mitotic figures. typically associated with an aggressive course

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autologous stem cell transplantation

process by which stem cells are collected from a patient (often with multiple myeloma or lymphoma) after mobilization and stored frozen. After patient is subjected to chemotherapy and radiation, rescued with their own stem cells

13

B symptoms

cytokine mediated constitutional symptoms (fever, night sweats, weight loss) associated with some lymphomas, classically Hodgkin lymphoma

14

basophilic

acidic substances like DNA that bind basic dyes like Wright's stain and hematoxylin. Blue under the microscope

15

BCL2

anti-apoptotic protein found in mitochondria, located on chromosome 18, inappropriately expressed in follicular lymphoma, though not in physiologic germinal centers.

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BCL6

a marker, like CD10, of germinal center B cell origin. functions as a transcriptional repressor.

17

BCR-ABL

constitutively activated in frame tyrosine kinase fusion produced by translocation t(9;22), the Philadelphia chromosome. expressed by neoplastic cells of CML

18

Bence Jones Protein

light chains filtered by the glomerulus into the urine

19

cast nephropathy

myeloma kidney caused by obstructive distal tubular aggregates of light chain casts

20

CD

cluster of differentiation. antigens crucial to the classification of hematopoietic neoplasms.

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CD1a

a marker of T lymphoblasts

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CD2

a pan-T antigen (expressed by all mature T cells)

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CD3

another pan-T antigen (expressed on the surface of all mature T cells). immature T cells express CD3 in their cytoplasm

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CD4

a marker of helper T cells. the receptor for HIV in T cells

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CD5

another pan-T antigen. Aberrantly expressed by some mature B cell neoplasms (CLL/SLL)

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CD7

pan-T antigen expressed by all mature T cells

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CD8

marker of cytotoxic T cells

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CD10

expressed by mature B cells of germinal center origin and neoplasms that arise from these cells (follicular/Burkitt lymphoma).

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CD19

a broad spectrum pan-B cell antigen expressed by both immature and mature B cells late into B cell life (expressed by plasma cells)

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CD20

a pan-B cell antigen with a more restricted range of expression during B cell life than CD19. expressed by mature B cells, but not B lymphoblasts or plasma cells

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CD34

marker of immature myeloid and lymphoid cells (blasts). included within the pool of CD34+ cells are stem cells that allow hematopoietic transplantation to succeed

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CD21

a marker expressed by follicular dendritic cells, which form the microarchitechtural support structures (follicular dendritic meshwork) that allows B cell follicles to aggregate. Also expressed by B cells and serves as a receptor by which EBV infects B cells.

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CD56 (NCAM)

adhesion molecule expressed by neoplastic plasma cells which allows them to take up residence in bone marrow. why multiple myeloma is a marrow based disorder.

34

CD138 (syndecan)

adhesion molecule expressed by plasma cells, both benign and neoplastic

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centroblast

proliferative B cell making up the dark zone of a reactive germinal center. in follicular lymphoma, these are the transformed component. an increase predicts a more aggressive phenotype.

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centroblastic

term used to describe a common variant of diffuse large B cell lymphoma, in which the neoplastic cells share a superficial morphology similarity with a benign centroblast, with an oval nucleus and multiple small nucleoli bosun to the nuclear membrane

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centrocyte

quiescent B cell predominating in the light zone of the reactive germinal center. cytologic appearance in the peripheral blood is distinctive, with prominent nuclear clefts. formally called small cleaved cells

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CFU

colony forming unit. nomenclature based on properties of progenitor cells grown on solid medium.

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CHOP

cyclophosphamide, doxorubicin, oncovin, prednisone. common multi agent regimen for aggressive non hodgkin lymphoma. CHOP plus Rituxan is R-CHOP

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chronic

neoplasm of hematopoeitic (myeloid, erthyroid, megakaryocytic) or lymphoid cells allowed to progress through their differentiation sequence to maturity (neutrophils, lymphocytes rather than blasts)

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CLL/SLL

chronic lymphocytic leukemia/small lymphocytic leukemia. one neoplasm with two different clinical manifestations. predominantly involving peripheral blood (leukemia) or forming solid masses (lymphoma). indolent CD5+ mature B cell neoplasm with a variable clinical course that reflects the underlying stat of the B cell from which it arose

42

clockface

nuclear chromatin condensed in a regular pattern at the nuclear periphery (as in the clock face nuclear chromatin of a plasma cell)

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CML

chronic myellogenous/myeloid leukemia, aka chronic granulocytic leukemia (CGL). form of leukemia characterized by the increased and unregulated proliferation of maturing granulocytes in the bone marrow with spillage into the peripheral blood. t(9;22) that fuses BCR and ABL to generate constitutively active tyrosine kinase

44

cryoglobulinemia

presence of large amounts of cryoglobulins in the blood. can be associated with various diseases that result in the expansion of plasmacytic cells, which can be reactive and neoplastic

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cryoglobulins

proteins (typically Igs) that can settle out (become insoluble) at low temperatures

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cytogenetics

study of karyotypic anomalies in metaphase spreads of primary or cultured cells. cells are arrested in metaphase with mitotic inhibitors and stained with giesma so the DNA G banding pattern can be identified.

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cytology

microscopic appearance of a single cell

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dark zone

the part of the reactive germinal center containing mostly activated B cells (centroblasts) which proliferate as part of the process of antibody refinement through somatic hypermutation and class switching.

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de novo

diffuse large B cell lymphomas that arise without transformation of a preceding indolent lesion

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DLBCL

diffuse large B cell lymphoma

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DN

double negative- lacking both CD4 & CD8. feature of T lymphoblasts

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DP

double positive- expressing both CD4 & CD8. feature of somewhat more mature T lymphoblasts

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EBV

epstein barr virus. infects B cells via CD21. always found in endemic Burkitt lymphoma.

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extranodal

growth or origin of a lymphoma in a non nodal site (as in MALT lymphoma)

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FL

follicular lymphoma, an indolent lymphoma arising from germinal center B cells inappropriately expressing BCL2 as a result of t(14;18)

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flame cell

IgA secreting plasma cell. has a pink flame cytoplasmic border by wright stain

57

follicle

b cell aggregate in a lymph node or other lymphoid organ, or at a state of chronic inflammation. can be either primary, consisting entirely of IgD naive mantle type b cells which have not yet encountered antigen or secondary, consisting of mantle cells surrounding the germinal center, in which they are undergoing affinity maturation

58

GCB type

germinal center B cell type. a subset of diffuse large B cell lymphomas with a germinal center B cell pattern of gene expression. does better than ABC type of diffuse large B cell lymphomas with current therapies

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germinal center

the part of the secondary follicle in which affinity maturation takes place

60

HTLV1

the first known human tumor causing retrovirus. the etiologic ager in ATLL. endemic to japan, caribbean, tropical africa. spread vertical and blood bourne. long latency before the onset of an aggressive mature t cell neoplasm

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humoral immune paresis

the repression of normal Ig production in the setting of a prominent monoclonal paraproteinemia. correlates with the significant immunosuppression and consequent susceptibility to infections

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hypercalcemia

elevated blood calcium. associated with bone lysis, causes by direct destruction of bone by neoplastic cells and by the elaboration of osteoclast activating factors that stimulate bone resorption

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IgH

Ig heavy locus, a region on human chromosome 14 that contains the gene segments and regulatory machinery for production go the Ig heavy chain

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immunoblastic

morphologically similar to a benign immunoblast with an oval nucleus, a prominent central nucleolus and ample plasmacytoid cytoplasm. term to describe the morphologic appearance of a type of diffuse large B cell lymphoma particularly common in primary EBV+CNS lymphomas

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immunofixation

identifies electrophoretically separated proteins.

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immunohistochemistry

exploits the principle that antibodies bind specifically to antigens expressed by cells in tissues. requires evaluation of tissue section

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immunophenotypic profile

particular pattern of protein expression of a given neoplasm

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immunophenotyping

performed by flow cytometry and/or immunohistochemistry.

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indolent

waxing/waning course, used to describe the behavior of a group of B cell non hodgkin lymphomas which are largely incurable since their slow rate if division makes them less susceptible to the DNA damage caused by chemotherapy or radiation

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Ki67

nuclear marker of proliferation. absent during the resting phase of the cell cycle. the fraction of Ki67 positive tumor cells in correlated with the clinical course of some neoplasms.

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leukemia

when neoplastic cells spill from the marrow into the peripheral blood in large numbers, a leukocytosis results. A neoplasm of circulating cells rather than a solid mass of neoplastic lymphocytes. Start in the marrow so presenting sign may be pancytopenia rather than leukocytosis

72

leukemoid reaction

a non clonal reaction to a stimulus that drives a granulocytic expansion in the marrow, with spillage of variably mature neutrophils into the periphery. can be mistaken for chronic myelogenous leukemia since it is due to t(9;22)

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leukocytosis

an elevated peripheral blood white count. can be due to expansion of immature cells

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acute myeloid leukemia

leukocytosis of immature clonal, myeloid origin cells

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acute lymphoblastic leukemia

leukocytosis of immature clonal, lymphoid origin cells

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granulocytic left shift

leukocytosis of immature reactive, myeloid origin cells

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myeloproliferative disorder

leukocytosis due to expansion of mature clonal, myeloid origin cells

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chronic lymphocytic leukemia

leukocytosis due to mature, clonal, lymphoid origin cells

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granulocytosis

leukocytosis due to mature, reactive, myeloid origin cells

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lymphocytosis

leukocytosis due to mature reactive lymphoid origin cells

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light chain restriction

evidence of B cell clonality (neoplasia). either only kappa or only lambda whereas it's usually 60/40

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light zone

the part of a reactive germinal center containing mostly quiescent B cells (centrocytes). Bc these cells have smaller and irregular nuclei, they don't stain as darkly

83

lymphadenitis

inflammation of a lymph node or multiple lymph nodes. A benign condition that sometimes can clinically be mistaken for a neoplasm

84

lymphadenopathy

enlargement of lymph nodes. Can be regional or widespread

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lymphoblast

immature lymphocytes found physiologically in the bone barrow and thymus.

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lymphoepithelial lesion

population of neoplastic B cells destroying mucosal structures. A classic high power feature of a MALT lymphoma

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lymphoma

a tumor forming lesion of lymphocytes

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M spike

a monoclonal paraprotein in serum or urine results in a dense homogenous band by serum electrophoresis. Associated with a single narrow church spire like peak

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MALT lymphoma

an extranodal marginal zone lymphoma of mucosa associated lymphoid tissues. start as chronic inflammatory lesions, often secondary to an infectious agent.

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mantle zone

area immediately surrounding the germinal center in a secondary follicle, which contains resting IgD B cells that have not yet been exposed to antigen

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marginal zone

a somewhat poorly define inter follicular area in the lymph node containing mature B cells that have traversed the germinal center

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MDS

myelodysplastic syndromes, a group of neoplastic marrow disorders associated clinically with low blood counts (cytopenia) and a propensity to evolve into acute myelogenous leukemia.

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MGUS

monoclonal gammopathy of undetermined significance. a laboratory finding (detection of low level serum monoclonal paraprotein) w/o other features to suggest multiple myeloma.

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monoclonal

a population of cells that share the same cell of origin

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monoclonal gammopathy

the laboratory finding of a monoclonal paraprotein in the serum or urine. May or may not meet criteria for myeloma.

96

Mott cell

a plasma cell containing prominent intracytoplasmic globular accumulations of immunoglobulin. one of a variety of appearances plasma cells (benign and malignant) can assume.

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MPD

myeloproliferative disorders. chronic hematologic malignancies that lead to an expansion in the marrow and peripheral blood of maturing cells of the hematopoietic series, usually with a dominance of one form.

98

MUM1

a relatively recent introduction to the prognostic armamentarium. expression of MUM1 by a diffuse large B cell lymphoma suggests that the B cell progenitor of this clonal lesion has traversed the germinal center and that the neoplasm is therefore of ABC type

99

MYC

helix-loop-helix leucine zipper containing TF that plays an important role in cell cycle progression, apoptosis, and cellular transformation.

100

neoplastic

a clonal population

101

NFkB

nuclear factor kappa light chain enhancer of activated B cells. plays a key role in regulation the immune response to infection.

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NHL

non hodgkin lymphoma

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nLP-HL

nodular lymphocyte predominant hodgkin lymphoma

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pancytopenia

a depression in all three major peripheral blood parameters: the white count (leukopenia), hemoglobin (anemia), and platelets (thrombocytopenia)

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paraimmunoblast

one of the transformed cells that can be seen in CLL/SLL. an intermediate to large neoplastic B cell.

106

paraprotein

in contrast to the normal heterogenous protein species, normally present in the peripheral blood and/or urine, the predominance of a single Ig species secreted by a plasmacytic neoplasm

107

paratrabecular

involving that part of the bone marrow that is directly adjacent to trabecular bone. Paratrabecular growth is typical of bone marrow involvement by follicular lymphoma

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plasmacytoma

a solid mass of neoplastic plasma cells

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plasmacytoid

demonstrates some of the morphologic features of a plasma cell. generally used to describe neoplastic B cells with particularly abundant cytoplasm

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podocyte

visceral epithelial cells that form the glomerular filtration apparatus. small molecules slip through slits between the feet of these cells

111

polychromasia

term used to describe the microscopic appearance of peripheral blood in the setting of an expansion of reticulocytes.

112

prolymphocyte

one of the transformed cells that can be seen in CLL/SLL. a somewhat immature lymphocyte that has large nucleus, fine chromatin, multiple nucleoli.

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prolymphocytic leukemia

the progression of CLL/SLL to a more aggressive lesion

114

Raynaud's phenomenon

the obstruction to blood flow in distal extremities caused by the aggregation of cryoglobins secreted by some lymphoproliferative disorders

115

reactive

a polyclonal expansion. expansion as an appropriate response to a stimulus

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red pulp

filtration compartment of the spleen

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richters transformation

transformation of CLL/SLL into diffuse large B cell lymphoma.

118

Rituximab (Rituxan)

anti-CD20 antibody. kills CD20 cells by a variety of mechanisms.

119

smudge cell

term used to describe the disrupted neoplastic cells common in the peripheral blood smears of patients with CLL

120

somatic hypermutation

the process whereby germinal center B cells develop highly avid antibodies through rapid mutation and selective apoptosis

121

SPEP

serum protein electrophoresis

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stage

clinical term used to describe the extent of neoplastic spread.

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grade

the microscopic features of a lesion that suggest behavior

124

starry sky

the combination of sheets of neoplastic cells and tangible body macrophages characteristic of Burkitt lymphoma

125

t(8;14)

translocation for Burkitts lymphoma. juxtaposes the IgH regulatory machinery with the coding sequence for Myc and results in over expression of this powerful oncogene

126

t(14;18)

the defining translocation for follicular lymphoma. Juxtaposes the IgH regulatory machinery with the coding sequence for Bcl2 and results in the inappropriate germinal center B cell expression of this anti-apoptotic protein

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t(11;18)

a recurrent translocation in gastric MALT lymphomas. causes the fusion of cIAP2 and MALT1 genes, and leads to dysregulation of the NFkB pathway

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t(9;22)

the philadelphia chromosome. defining translocation for CML. causes the fusion of BCR and ABL, resulting in a constitutively active tyrosine kinase

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t(15;17)

defining translocation for acute promyelocytic leukemia. fuses APL and RARa to generate a dominant negative that inhibits normal RARa signaling and leads to a block in the myeloid maturation at the promyelocyte stage

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TdT

terminal deoxynucleotidyl transferase, a specialized DNA polymerase expressed in immature B and T lymphocytes. Adds N nucleotides to the variable, diversity and joining eons during immunoglobin gene recombination

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tangible body macrophages

scavengers of apoptotic lymphocytes, found in the dark zone of germinal centers and in any aggressive lymphoma. most prominent in Burkitts lymphoma

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tumor lysis syndrome

a constellation of metabolic complications and their downstream effects due to a sudden release of breakdown products from dying cells.

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uremia

an excessive serum level of urea. consequence of renal failure

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white pulp

lymphoid compartment of the spleen, has a periarteriolar distribution

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ZAP70

cytoplasmic T cell signaling molecule. Aberrantly expressed by CLL/SLL and correlates with absence of somatic hypermutation, suggesting origin in a naive B cell and a propensity for more aggressive behavior