Term
|
Definition
| proliferation of T/B cell or precursors that spill into the blood |
|
|
Term
|
Definition
| blast cells unable to differentiate causing accumulation of blasts in marrow (>20% blasts required) |
|
|
Term
|
Definition
abrupt onset
fatigue, bleeding, fever infection
bone pain and tenderness
generalized lymphadenopathy
HSM
CNS signs: headache, vomiting, nerve palsies (meningeal spread) |
|
|
Term
|
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
|
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
|
Definition
activaating NOTCH1 mytation
NUP214-ABL fusion increases TK
hyperploidy |
|
|
Term
|
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
|
|
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
|
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
|
|
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
|
|
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
|
|
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
|
|
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 |
|
|
