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82 Cards in this Set

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what is a lymphoma?
proliferation of lymphocytes as discrete tissue masses
what is precursor B or T cell lymphoblastic lympohoma aka
ALL
how does lympohoma present
painless lymphadenopathy (swollen nodes)

constitutional or B symptoms:fever, loss of appetit, weight loss

immunologic disturbances: autoantibodies, monoclonal protiens, recurrent infection

extranodal masses: skin, gonads, testes, eye, brain
what is walderyers ring?
a circle of lymphnodes that act as oral survalence, can be a sight of lymphoma
how is lymphoma diagnosed
must have biopsy of lymph node
preferably whole lymphnode
what type of cell type is in most malignant lymphomas
b cells
what is hodgkin lymphoma
presence of hodgkin and reed-sternberg cells

nonneoplastic inflammartory cells migrate into nodes bc of RS cells

RS cells are responsive to treatment but but the nodes dont shrink bc most of the cells in the swelling are inflammartory

there are very effective treatment protocols which is why its important to get the diagnosis for it.
what are the subytpes of hodgkin lympohoma?
nodular sclerosis
mixed cellularity
lymphocyt rich
lymphocyte depleted
- the four above represent classical HL with RS cells

lymphocyte predominant
what are the two most common types of HL
nodular sclerosis
mixed cellularity
what are the classificatino of lymphoid neoplasms
I precursor B cell neoplasms
II periperal B cell neoplasms
III precursor T cell neoplasms
IV peripheral T cell and NK cell neoplasms
V Hodgkin lymphoma
what is the difference between precursor and perhipheral neoplasms when refering to lymphoma?
precursor is mainly blasts
peripheral is more mature cells
What are common lyphoid precurors(CLP)
the first cell in the develpment process of the lymphocyte found in the bone marrow
what can the Naive B Cell (NBC) give rise to
small lymphoctic lymphoma/leukemia
CLL
What can the germinal center give rise to?
follicular lymphoma
Burkitt lymphoma
diffuse large B cell lymphoma
HODGKIN LYMPHOMA
What can the marginal zone give rise to?
Diffuse Large B Cell lymphoma
marginal zone lympohoma
small lymphocytic lymphoma
CLL
Where do T cell lymphomas arise
precursor T cell lymphoma arises in the thymus from DN cells

peripheral T cell lymphoma arise from the lymph node
what defines a b cell lymphoid malignancies
monoclonal: that is a population all derived from one cell and produce all the same type of heavy or light immunoglobulin chains

so all look the same under a microscope
what defines a T cell lymphoid malignanciy
also monoclonal but does not make immunoglobulins, so abnomal loss or gain of surface antigens, T cell receportor arrangement studies helpful.
what is the most common B cell lymphoma
diffuse large B cell lymphoma
what is the most common T cell lymphomas
periperal T cell lymphoma, NOS
What is the characterization of diffuse large B cell lymphoma?
a proliferation of large neoplastic B lympohid cells
- nuclear size equal to or exceding
normal macrophage nuclei or more than twice the size o a normal lymphocyte

have a diffuse not nodular growth pattern.
immuno-histo chemistry of a B cell lymphoma would show what?
brown staining of all cells
what is the marker used to identify B cell lymphoma
CD20 main one

CD19, CD22, CD79a
what gene translocation can be present in B cell lymphoma and what is the difference btw them
bcl-2,t(14;18) occurs in 20-30% and usually indicates that this arose from a follicular lymphoma

bcl-6 gene, (3q27 rearrangment) occurs in 30% and usually indicates de novo developement caused by dysregulation of germinal centers.
what stage is a lymphoma if there is bonemarrow involvment
stage 4
what are the two speacial subsets of diffuse B cell lymphoma
immunodeficiency associated

body cavity
what is the cause in immunodeficiency associated diffuse B cell lymphoma?
sever T cell depletion allows emergence of malignant B cells

OFTEN assiated with EBV
what is the cause of Body cavity associated diffuse B cell lymphoma
neoplasm arises in the body fluids,(plural, pericardial, abdominal, )

ASSOCIATED WITH KSH/HHV8/EBV viruses

deadly

only in AIDS patients with kaposi sarcoma.

jellyfish like cells
what is the second most common type of lymphoma in the western world
B Cell follicular Lymphoma characterized as being nodular rather than diffuse.
what is the prognosis of folicular lymphoma
not bad
the folicular lymphoma is indolent which means the cells are more mature when they become cancerous.
what are the implications of indolent lymphoma
indolent means they are more mature which means they are much less agressive so they tend to cause less problems than other cancers. but because they are more mature they do not respond to most modern therapies that target rapidly dividing cells. so they are generally not curable.
what is a centrocyte
a cleaved follicle center
what is a centroblasts
a large noncleaved folicle center cell
how do you grade follicular lymphoma
grade 1 0-5 centroblasts per HPF
grade 2 6-15 centroblasts per HPF
grade 3 >15 centroblasts per HPF

basically the more centroblasts the higher the grade.
what do higher grades of follicular lymphoma indicate
the higher the grade the more likely that the lymphoma will turn into diffuse B cell lymphoma which is much more dangerous.
how do you treat grade 3 follicular lymphoma
the same way you treat diffuse B cell lymphoma
what are the markers for follicualr lymphoma
all B cell markers are the same so just like DBCL there are (CD19,20,22,79a) but the one that is characteristic of follicular lymphoma is CD10 which indicates follicluar center origin
what other cancer can be identified by CD10 markers
precursor B acute lymphoblastic leukemia/lymphoma.`
what is the main gene translocation in follicular lymphoma
t(14;18)
BCL2 gene inhibits apoptosis, so follicular center cells have polonged survivial.
what are the features of follicular lymphoma
exclusively to adults
indolent
can progress to diffuse B cell lymphoma
discribe the extranodal marginal zone b cell lymphoma of MALT
also called maltoma
orginates from marginal zone of lymphnoded then spreads out of lymphnode
mostly seen in GI tract mainly in stomach can be caused by helicobacter pylori gastritis

can be treated by antiboitic

may progress to DLBCL with t(11;18) then resistant to antibiotics
discribe the small lympocytic lympoma
same as CLL but all in the lymph node
mainly in male around 65yo
What is mantle cell lynmphoma
B cell neoplasm composed of small to medium sized cells
most commin invovles lympnodes but may be extranodal in spleen bonemarrow gi or waldeyer ring
male 60yo 3-5year survival
what gene is associate with mantle cell lymphoma?
cyclin D1(11)-promotes G1 to S phase

t(11;14)(q13;q32)
discribe Burkitt's lymphoma
highly agressive
doubles overnight
all cells dividing
seen with EBV
same cell found in ALL
found extranodally
what are the three types of burrkitts lymphoma
endemci-starts in jaws and facial bones found in africa with EBV

sporadic - presents with abdominal masses

immunodeficiency associated with AIDS nodal loclization and bone marrow.
what is tumor lysis syndrome
when cells are so sensitive to chemotherapy that it produces rapid cell death and hyperkalemia

can be found in burketts lymphoma
what is the morphology of Burkketts lymphoma
clear halo around the cells
called stars in the sky
lots of mitotic figures
what is the gene most often afected in burketts lymphoma
c-MYC gene- thought to affect progression from G2 in cell cycle

t(8;14), t(2;8, t(8;22)
what are some characteristics of mature T and NK cell neoplasms
usually agressive
more common in asia, carribean and Mexico
only 5 year expected survival
classified by flow cytometry
clicical presention
and t cell receptor rearrangements
what are the defining elements of the reed sternberg cells of Hodgkins lymphoma?
reed sternberg cells that draw reactive inflammatory cells into lymphnodes

RS typically binucleated but can be mono or multi nucleated as well

all nuclei have a single larg eosinophilic neucleoli

inflammatory cells that surround the RS cells are benign lymphocyts, macrophages, granulocytes, eosinophils
what is this?
reed sternberg cell
what lineage of cell type does the reed sternberg cell come from?
B cell
what are the typical clinical features of Hodgkins lymphoma?
nodal based disease with contiguous spread(spreads from node to node in close proximity as opposed to malignant lymphoma which will spread all over above and below the diagphram, HS typically is either above or below the diaghram.)

painless enlargment of lymphnodes

fever nightsweats unexplained wieghtloss
what are the B symptoms?
fever night sweats unexplained wieght loss
what two thing determine prognosis for hodgkins lymphoma?
subtype and staging
what is the order of subtypes by worsening prognosis
excellent: lymphocite rich
nodular sclerosis

good;
mixed cellularity

intermediate to unfavorable:
lymphocyte depleted
what is the most common subtype of HL
nodular sclerosis Hodgkins lymphoma
what is the character of Nodular sclerosis Hodgkin lymphoma?
collagen bands that surround nodules and lacunar type HRS cells

28yo

mediastinal invovlment

usually presents stage 2
what do these two pictures portray?
Nodular sclerosis hodgkins lymphoma
what are the characteristics of mixed cellulartiy HL
37yo
male
presents stage III or IV
B symptoms(fever, nighsweats, weightloss)

periperal lymph node invovlement
where do plasma cells come from?
lymphocytes
what is a plasma cell neoplasm
neoplastic plasma cells secreting an intact monoclonal immunoglubulin or fragment (M protein)
what are the subtypes of plasma cell neoplasms
plasma celll myelomo(multiple myelomo)
lymphoplasmactyic lymphoma/waldentrom macroglobulinemia
solitary myeloma(plasmacytoma)
monoclonal gammopathy of uncertain significnace (MGUS)
what is the most common of the plasma cell neoplasms
MGUS
what are the characters of multiple myeloma
its is a neoplasm of a mature plasma cell

produces IgG or IgA

65-70yo

more common in AA

clincical findings due to the immunoglobulins

can infiltrate the bone marrow and cause anemia/thrombocytopenia.
what causes bone pain in multiple myeloma?
tumor cells release ctyokines to upregulate RANKL to activate osteoclasts this can lead to hypercalcemia, urinary stones

most common bones invovled are vertebral bodies, ribs, skill, pelvis, femur, clavicle, scapula
what are the clincical symptoms of multiple myeloma?
bonemarrow infiltration leads to symptoms of anemia, weakness, fatigue

RBC rouleaux:stack of coins -RBC get stuck together by extra protiens in the blood

recurrent bacterial infections

renal insufficiency: immunoglobulin fragments toxic to kidney(bencejones protienuria)

spinal cord compression

neurologic, due to amyloid infiltration into nerves

parasthesias, mental alterations, neuroligc effects of hypercalcemia
what does this picture show
RBC rouleaux
indicative of multiple myeloma
review this chart
what are the four things that multiple myeloma causes and what are their consequences?
monoclonal protein
-positive electrophoresis
-bencejones proteinura
-renal failure
-hyperviscosity
-amyloidosis

immune deficiency
-decreased IgG
-infection

anemia
-caused by bonemarrow infiltration

bone destruction
-fractures
-punched out radiographic bone lesions
-bone pain
-hypercalcemia
-urninary stones
-neurologic issue
what is the diagnostic triad for multiple myeloma
punched out lytic bone lesions

monoclonal protien on electrophoresis:restircted mobility

plasmacystosis of bone marrow aspirate
what does this electrophoreis represent?
monoclonal antibodies because of the single line instead of blurred lines from multple cell lines.
what are the molecular abnormatlities for multiple myeloma
Ig heavy chain rearragnement on 14q32
dyreg of D cyclins
deletion of 13q
what is the major treatments for MM
bone marrow transplant
chemotherapy
thalidomide
describe lymphoplamacytic lymphoma
a lymphoma of Bcells that are turning into plasma cells but arent there yet

they secrete IgM

incurable and progressive

Hyperviscosity (waldenstrom macroglobinemia):super thick bc IgM is so large leads to visual problems bc retina vein congestion, neuroligic headaches dizziness, deafness, bleeding due to intereference with clotting factors, and cryoglobulinema which is precipitation fo IgM at low temps ie Raynaud's and cold urticaria
how is lymphoplasmacytic lymphoma treated
plasmapheresis
anti CD20 antibody

presents as lymphoma with no lytic bone lesions
discribe MGUS
monoclonal immunoglobuin in blood, urine, or both

no clinical signs or symptoms of plasma cell myeloma or lymphoma.

serum M protein level less than 3gm/dL

plasma cells show same chromoaslma trnasloactions as full blown MM

this is most likly an early stage of MM.
how is MGUS followed up
close monitoring for monoclonal protiens watch for bence jones proteinura

1% / year convert to MM
what are the groups of MGUS
group 1: no significant increase M protien(10%)

group 2: M protein increased to > 3g/d:(11%) no plasma cell dyscrasia developed

group 3: died of unrealted cause (53%)

group 4: develped MM or realtead disease median of 10 years.
what two thing help us classify if a patient with MGUS is in class 4 and may have a risk of progressing to MM
IgG M protein

abnormal serum kappa:lambda light chain ratio
discribe solitary myeloma(plasmacytoma
a variation where the plasma cell has an unusual adhesion marker so it stays in one place

can arise in soft tissue or bone
-bone: same places as MM is found
-soft tissue: lung, oronasopharnx, nals sinuse

bone location usually progress to MM soft tissue usually doesnt and can be resected with high chance of cure
discribe plasma cell leukemia
a secondary condition to MM where the disease has progressed so far that the plasma cells spill out in into the blood

poor prognosis

treate with aggressive chemo and bone marrow stem cell transplant.