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Path Leukemia Lymphoma Plasma Cell
KYCOM Block 14
72
Pathology
Graduate
03/03/2014

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Term
define leukemia
Definition
proliferation of T/B cell or precursors that spill into the blood
Term
cause of acute leukemias
Definition
blast cells unable to differentiate causing accumulation of blasts in marrow (>20% blasts required)
Term
clinical signs of ALL
Definition
abrupt onset
fatigue, bleeding, fever infection
bone pain and tenderness
generalized lymphadenopathy
HSM
CNS signs: headache, vomiting, nerve palsies (meningeal spread)
Term
cause of B-ALL (2)
Definition
most common: child t(12;21) TEL1-AML1 fusion

less common: adult transformation from CML t(9;22 phaldalphia) BCR-ABL increases TK activity
Term
lab markers for B-ALL 5
Definition
TdT
B Cell markers (CD 10, 19, 20)
blasts in peripherial blood
WBC <10,000 >100,000 or aleukemic
pancytopenia
Term
what does a ALL look like on a gemisa stain 6
Definition
lymphoblasts
coarse clumped chromatin
2 nucleoli
little agranular cytoplasm
glycogen granules
acid shiff positive
Term
treatment and prognosis of B-ALL
Definition
excellent response to chemo
prophylaxis in CNS and scrotum needed
2-10y 80% chance with chemo
child form has better prognoses
better prognosis in females
Term
3 cause of T-ALL
Definition
activaating NOTCH1 mytation
NUP214-ABL fusion increases TK
hyperploidy
Term
lab markers for T-ALL 5
Definition
TdT
T cell markers CD2-8
blasts in peripherial blood
WBC <10,000 or >100,000 or aleukemic
pancytopenia
Term
what is the most common age and gender for T-ALL
Definition
15-20 male
Term
treatment and prognosis of T-ALL
Definition
2-10yo male 80% chance success with chemo
not within range worse prognosis
Term
cause of CLL/SLL and 4 pathological changes in result
Definition
high BCL2 stops B cell apotosis regulators
trisomy 21, deletion 11 or 12, or translocation

hypogammaglobulinemia: B cell can't make IgG

immune hemolytic anemia: if it does make Ig it is autoreactive to platelets and RBC

marrow destruction: tumor cells proliferation until displace normal marrow causeing pancytpenia

mutation of genoma transforms into diffuse large B cell lymphoma
Term
symptoms of CLL/SLL 9
Definition
presenting: fragility, weight loss, anorexia
lymphadenopathy: SLL
infections
anemia
bleeding
HSM
Term
lab markers for CLL/SLL 4
Definition
CLL WBC>4000 (not in SLL)
B cell markers CD 19, 20, 23
surface Ig heavy and light chains
CD5!!! (normally T cell, only other seen in mantle cell lymphoma)
Term
prognosis and complications of CLL
Definition
median survival is 4-6y
slow growing
transformation into prolymphocytic leukemia, diffuse large B cell lymphoma
Term
cause of hairy cell leukemia
Definition
mature B cell proliferation infiltrates spleen and marrow
due to activation mutation in serine TK BARF
Term
lab markers for hairy cell leukemia 5
Definition
hair cytoplasmic processes
TARP positive
B cell markers CD19, 20
surface Ig
markers CD11c CD103
Term
clinical signs of hairy cell leukemia 4
Definition
splenomeagly massive (sequestration and extracellular hematopoesis)
older male
pancytopenia (B cells infiltrate marrow)
dry tap: fibrosis of marrow

NO lymphadrnopathy
Term
tx and prognosis of hairy cell leukemia
Definition
2-CDA purine: adenosine deaminase inhibitor causes adenosine accumulation killing neoplastic B cells
excellent prognosis
Term
what are the 3 T cell non-hodgkins lymphomas
Definition
cutaneous T cell lymphoma (mycosis fungoides)
adult T cell lymphocytic lymphomia
peripherial T cell lymphomia
Term
what are the 6 B cell non-hodgkins lymphomas
Definition
follicular
mantle
extranodal/marginal
burkitt
diffuse large B cell
SLL
Term
cause of follicular lymphoma
Definition
t(14;18) BCL2-IgH causes BCL2 overexpression allowing more B cells to pass somatic hypermutationdue to apoptosis supression
Term
clinical signs of follicular lymphoma 2
Definition
patient >50yo
painless lymphadenopathy
usually generalized
Term
what do the follicles look like in follicular lymphoma
Definition
hyperplasia in cortex that disrupts node architecture
no necrotic cells or tangible macrphages conducting apoptosis
small neoplastic cells with cleaved nuclei, infoldings, condensed chromatin
large non-neoplastic cells with vesicular chromatin and many nucleoli
Term
lab martkers for follicular lymphoma 3
Definition
B cell markers
BCL6 (germinal center marker)
BCL2
Term
prognosis and complications of follicular lymphoma
Definition
median survival 7-9y
no cure
pick up additional mutations and transform to diffuse large B cell lymphoma
Term
cause of mantle cell lymphoma
Definition
t(11;14) cyclin D1 - IgH allows G1/S transition
more small B cells in mantle of lymph node
Term
clinical signs of mantle cell lymphoma
Definition
lesions in marrow, blood, spleen, liver, node
lymphomatoid polyposis: lesion in GI that resembles polyp
Term
lab markers for mantle cell lymphoma 4
Definition
surface Ig
B cell markers
CD!! (normally in T cells, only other B cell vesion seen in CLL)
cyclin D1
Term
prognosis of mantle cell lymphoma
Definition
agressive
no cure
3-5y
Term
cause of extranodal/marginal lymphoma
Definition
marginal zone is potential psace. enlarged by chronic inflammation.

H. pylori causes maltoma in marginal zone of GI malt nodes

t(11;18) MALT1-IPA2
Term
where does extranodal/marginal lymphoma cause lesions 6
Definition
stomach, salivary glands, bowel, lungs, orbit, breast
Term
tx and prognosis of extranodal/marginal lymphoma
Definition
H. pylori caused can be tx with antibiotics
may need radiotherapy or local excision
if caused by translocation often wont respond
Term
cause of burkitt lymphoma and location of lesions
Definition
t(8;14) IgH-cMYC increases B cell growth
latent EBV infection

african: 100% EBV caused, lesion in mandible or maxilla

sporatic: 25% EBV caused, lesion in bowel, retroperitoneal, ovary
Term
what do the cells look like on a smear in burkitt lymphoma
Definition
B cells with round ovoid nuclei
lipid vacules
basophillia
STARRY SKY PATTERN: high rate of proliferation and apoptosis recruits macrophages that eat away a clear space
Term
lab markers in burkitt lymphoma
Definition
surgace IgM
B cell markers
BCL8: germinal center marker
CD10: germinal center marker
Term
prognosis of burkitt lymphoma
Definition
fast growing, majority cured esp when young
Term
2 cause of diffuse large B cell lymphoma
Definition
BCL6 rerangement or mutation
t(14;18)IgH-BCL2 (transformed from follicular lymphoma)
Term
clinical signs of traditional diffuse large B cell lymphoma
Definition
lesion in GI, brain, retroperitoneal
NOT commin in liver spleen or marrow
Term
what do the cells look like in diffuse large B cell lymphoma
Definition
very large
diffuse growing in sheets (not follicle, mantle, etc)
poor differentiation (this is rare for lymphoid cells their either Blast or grown up)
Term
lab markers fo diffuse large B cell lymphoma 2
Definition
B cell markers
surface Ig
Term
what are the three conditions that diffuse large B cell lymphoma is associated with, how does the disease present when combined with them
Definition
lymphoma + EBV + immune comp (AIDS): transforms to clonal large B cell lymphoma in months

karposki + lymphoma: pleural, pericardial, and periosteal effusions

mediastina: in young females, spreads to abdominal viscera and CNS
Term
prognosis of diffuse large B cell lymphoma
Definition
rapidly fatal if untrated
60% complete remission with chemo
Term
cause of mycosis fungoides
Definition
proliferation of CD4 T cells that invade skin
Term
clinical signs of mycosis fungoides
Definition
erythrodermic rash > plaque > tumor > sezary syndrome (hematogenous spread)
Term
lab markers for mycosis fungoides
Definition
CD4T cells in epidermis and upper dermis make pautrier microabscesses

cerebri form nuclei when sezary
Term
prognosis of mycosis fungoides
Definition
erythrodermic: years
plaque, tumor, sezary: 1-3y
Term
cause of adult T cell lymphocytic lymphoma
Definition
CD4 proliferatioon to to HTLV-1
Term
clinical signs of adult T cell lymphocytic lymphoma
Definition
rash
generalized lymphadenopathy
HSM
lytic bone lesions and hypercalcemia
transverse myelitis: demyelination of CNS
Term
lab markers of adult T cell lymphocytic lymphoma
Definition
malignant lymphocytes in peripheral blood
CD4, CD25, IL2
Term
prognosis adult T cell lymphocytic lymphoma
Definition
median survival 8mo
15% have chronic course (same as mycosis fungoides)
Term
cause of peripheral T cell lymphoma
Definition
heterogenous group of tumors of functional T cells
Term
peripheral T cell lymphoma signs
Definition
disseminated disease due to tumor derived from inflammatory products
Term
prognosis peripheral T cell lymphoma
Definition
agressive
responds poorly to therapy
Term
cause of hodgkins lymphoma
Definition
proliferation of reed sternburg cells from a germinal center B cell
Term
what do reed sternburg cells look like
Definition
owl eye
multilobed nuclei
enclosed acidophilic nuclelous
clear zone around

(THESE DONT MAKE THE MASS they draw inflammatory cells which give it mass like shape)
Term
signs of hodgkins lymphoma
Definition
stage IA-IIA: painless lymphadenopathy on single or chain of nodes

Stage IV+: fever chills, night sweats, puritis, anemia
Term
what are the 5 types of hodgkins lymphoma and their prognosis
Definition
lymphocyte depleted - worse
lyphocyte - best
lymphocyte predominate
nodular sclerosis
mixed cellularity
Term
lymphocyte predominate hydgkins lymphoma: clinical and lab signs, prognosis
Definition
cervical and axillary lymphadenopathy

small lymphocytes and histocytes

lymphocytic varient RS cells: have B cell markers

popcorn RS cells: puffy nuclei

excellent prognosis
Term
nodular sclerosis: cause, signs, lab markers, prognosis
Definition
can be caused by EBV

in adolescent or young, supraclavicular cervical or mediastinal nodes

BANDS OF FIBROSIS in node
lacunar cells: RS sit in open spaces
no T/B cell markers

excellent prognosis
Term
mixed cellularity hodgkins lymphoma: cause, signs, lab markers
Definition
EBV causes hyperactivity of NH-kB TG promoting B cell proliferation and protecting from apoptotic signals

>50yo, male, fever, night sweats, weight loss

heterogenous infiltrates, lymphocyes, eosinophils, plasma cells, histocytes

classic RS cells with no T/B cell markers
Term
cause of multiple myeloma
Definition
proliferation of plasma cells in bome marrow that secrete complete or partial Ig (called M proteins)

due to cyclin D1, cyclin D3, FGF receptor 3, or MyC translocation next to IgH

fibroblasts and macrophages produce excess IL-6 to support the tumor
Term
6 effects of multiple myeloma and why
Definition
bone pain and hypercalcemia: tumor secretes IL-1, TNF, IL-7 which singals RANK and causes osteoclast bone reabsorption causing punched out lytic lesions on X ray and increased risk for fracture

immune supression: plasma cells secrete dysfunction Ig increasing risk for infection

renal dysfunction: dysfunctional Ig are toxic to the DCT and CD and make linear amyloid deposits of bence jones proteins allowing for bacterial pyelonephritis, metastastic calcification, and renal failure

hyperviscosity due to increased dysfunctional Ig in blood

reticuloendothelial take over: dysfunctional plasma cells take over spleen, marrow, kidney, liver, lungs, nodes, soft tissue (may turn into leukemia) causing pancytpenia
Term
lab markers of multiple myeloma
Definition
increased IL-6
M proteins: usually IgG (some IgA or M)
Ig parts usually free light chains
Term
prognosis of multiple myeolma
Definition
4-5y
no cure
Term
solitary/localized plasmacytoma: clinical signs, prognosis
Definition
solitary skeletal or soft tissue tumor that makes M proteins
5-10y progression to multiple myeloma
often cured by local resection
Term
monoclonal gamopathy of undetermined significance: cause, clinical signs, prognosis
Definition
cells have same translocations as multiple myeloma. pt has M proteins in serum and but no lytic lesion or other symptoms

develop into multiple myeloma at rate of 1% per year
Term
lymphoblastic lymphoma: cause, presentation
Definition
B cell lymphoma makes IgM
generalized lymphadenopathy
waldenstorm macroglobulinemia: hyperviscosity causes visual impairment due to hemorrhage and exudates, neurological issues, bleeding, and cryoglobulinemia
Term
lab markers of lymphoblastic lymphoma
Definition
B cells with mixture of types: small, paraneoplastic, normal
behave like indolent B celll lymphoma in marrow, nodes, spleen
produce IgM with normal heavy/light chains (NO bence jones)
hyperviscosity
Term
prognosis of lymphoblastic lymphoma
Definition
4-5y
Term
heavy chain diseasse: cause, presentation
Definition
varient of multiple myleoma
IgA: in lymphoid tissue, SI, respiratory
OR
IgG: in nodes, liver, spleen (HSM)
Term
primary immunocyte associtated amyloidosis: cause, presentation
Definition
varient of multiple myeloma that only makes light chain
causes amyloid deposits
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