Glossary Flashcards
1
Q
ABC type
A
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
Q
acute
A
a hematopoietic (myeloid, erthyroid, megakaryocytic) or lymphoid precursor blocked at a very early (blast or promyelocyte) stage of differentiation.
3
Q
ALCL
A
anaplastic large cell lymphoma
4
Q
ALK1
A
anaplastic lymphoma kinase
5
Q
allogenic stem cell transplantation
A
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
Q
amyloid
A
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
Q
amyloidosis
A
the clinical disorder produced by systemic amyloid deposition. Prominent are the effects of amyloid deposition on the glomerular filtration apparatus
8
Q
Ann Arbor staging system: stage 1
A
neoplasm limited to single site, +/-B (constitutional) symptoms (night sweats, weight loss, fevers)
9
Q
Ann Arbor staging system
A
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
Q
ATLL
A
adult T cell leukemia/lymphoma
11
Q
atypia
A
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
12
Q
autologous stem cell transplantation
A
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
Q
B symptoms
A
cytokine mediated constitutional symptoms (fever, night sweats, weight loss) associated with some lymphomas, classically Hodgkin lymphoma
14
Q
basophilic
A
acidic substances like DNA that bind basic dyes like Wright’s stain and hematoxylin. Blue under the microscope
15
Q
BCL2
A
anti-apoptotic protein found in mitochondria, located on chromosome 18, inappropriately expressed in follicular lymphoma, though not in physiologic germinal centers.
16
Q
BCL6
A
a marker, like CD10, of germinal center B cell origin. functions as a transcriptional repressor.
17
Q
BCR-ABL
A
constitutively activated in frame tyrosine kinase fusion produced by translocation t(9;22), the Philadelphia chromosome. expressed by neoplastic cells of CML
18
Q
Bence Jones Protein
A
light chains filtered by the glomerulus into the urine
19
Q
cast nephropathy
A
myeloma kidney caused by obstructive distal tubular aggregates of light chain casts
20
Q
CD
A
cluster of differentiation. antigens crucial to the classification of hematopoietic neoplasms.
21
Q
CD1a
A
a marker of T lymphoblasts
22
Q
CD2
A
a pan-T antigen (expressed by all mature T cells)
23
Q
CD3
A
another pan-T antigen (expressed on the surface of all mature T cells). immature T cells express CD3 in their cytoplasm
24
Q
CD4
A
a marker of helper T cells. the receptor for HIV in T cells
25
CD5
another pan-T antigen. Aberrantly expressed by some mature B cell neoplasms (CLL/SLL)
26
CD7
pan-T antigen expressed by all mature T cells
27
CD8
marker of cytotoxic T cells
28
CD10
expressed by mature B cells of germinal center origin and neoplasms that arise from these cells (follicular/Burkitt lymphoma).
29
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)
30
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
31
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
32
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.
33
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
35
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.
36
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
37
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
38
CFU
colony forming unit. nomenclature based on properties of progenitor cells grown on solid medium.
39
CHOP
cyclophosphamide, doxorubicin, oncovin, prednisone. common multi agent regimen for aggressive non hodgkin lymphoma. CHOP plus Rituxan is R-CHOP
40
chronic
neoplasm of hematopoeitic (myeloid, erthyroid, megakaryocytic) or lymphoid cells allowed to progress through their differentiation sequence to maturity (neutrophils, lymphocytes rather than blasts)
41
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)
43
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
45
cryoglobulins
proteins (typically Igs) that can settle out (become insoluble) at low temperatures
46
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.
47
cytology
microscopic appearance of a single cell
48
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.
49
de novo
diffuse large B cell lymphomas that arise without transformation of a preceding indolent lesion
50
DLBCL
diffuse large B cell lymphoma
51
DN
double negative- lacking both CD4 & CD8. feature of T lymphoblasts
52
DP
double positive- expressing both CD4 & CD8. feature of somewhat more mature T lymphoblasts
53
EBV
epstein barr virus. infects B cells via CD21. always found in endemic Burkitt lymphoma.
54
extranodal
growth or origin of a lymphoma in a non nodal site (as in MALT lymphoma)
55
FL
follicular lymphoma, an indolent lymphoma arising from germinal center B cells inappropriately expressing BCL2 as a result of t(14;18)
56
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
59
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
61
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
62
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
63
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
64
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
65
immunofixation
identifies electrophoretically separated proteins.
66
immunohistochemistry
exploits the principle that antibodies bind specifically to antigens expressed by cells in tissues. requires evaluation of tissue section
67
immunophenotypic profile
particular pattern of protein expression of a given neoplasm
68
immunophenotyping
performed by flow cytometry and/or immunohistochemistry.
69
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
70
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.
71
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)
73
leukocytosis
an elevated peripheral blood white count. can be due to expansion of immature cells
74
acute myeloid leukemia
leukocytosis of immature clonal, myeloid origin cells
75
acute lymphoblastic leukemia
leukocytosis of immature clonal, lymphoid origin cells
76
granulocytic left shift
leukocytosis of immature reactive, myeloid origin cells
77
myeloproliferative disorder
leukocytosis due to expansion of mature clonal, myeloid origin cells
78
chronic lymphocytic leukemia
leukocytosis due to mature, clonal, lymphoid origin cells
79
granulocytosis
leukocytosis due to mature, reactive, myeloid origin cells
80
lymphocytosis
leukocytosis due to mature reactive lymphoid origin cells
81
light chain restriction
evidence of B cell clonality (neoplasia). either only kappa or only lambda whereas it's usually 60/40
82
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
85
lymphoblast
immature lymphocytes found physiologically in the bone barrow and thymus.
86
lymphoepithelial lesion
population of neoplastic B cells destroying mucosal structures. A classic high power feature of a MALT lymphoma
87
lymphoma
a tumor forming lesion of lymphocytes
88
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
89
MALT lymphoma
an extranodal marginal zone lymphoma of mucosa associated lymphoid tissues. start as chronic inflammatory lesions, often secondary to an infectious agent.
90
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
91
marginal zone
a somewhat poorly define inter follicular area in the lymph node containing mature B cells that have traversed the germinal center
92
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.
93
MGUS
monoclonal gammopathy of undetermined significance. a laboratory finding (detection of low level serum monoclonal paraprotein) w/o other features to suggest multiple myeloma.
94
monoclonal
a population of cells that share the same cell of origin
95
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.
97
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.
102
NHL
non hodgkin lymphoma
103
nLP-HL
nodular lymphocyte predominant hodgkin lymphoma
104
pancytopenia
a depression in all three major peripheral blood parameters: the white count (leukopenia), hemoglobin (anemia), and platelets (thrombocytopenia)
105
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
108
plasmacytoma
a solid mass of neoplastic plasma cells
109
plasmacytoid
demonstrates some of the morphologic features of a plasma cell. generally used to describe neoplastic B cells with particularly abundant cytoplasm
110
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.
113
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
116
red pulp
filtration compartment of the spleen
117
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
122
stage
clinical term used to describe the extent of neoplastic spread.
123
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
127
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
128
t(9;22)
the philadelphia chromosome. defining translocation for CML. causes the fusion of BCR and ABL, resulting in a constitutively active tyrosine kinase
129
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
130
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
131
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
132
tumor lysis syndrome
a constellation of metabolic complications and their downstream effects due to a sudden release of breakdown products from dying cells.
133
uremia
an excessive serum level of urea. consequence of renal failure
134
white pulp
lymphoid compartment of the spleen, has a periarteriolar distribution
135
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
