lymphoma Flashcards
multiple myeloma
- increased production of non- functioning plasma cells
- plasma cells produce IgG with excess light chains
RF for MM
- usu dx in 60s
- no clear RF
- assoc with woodworkers, farmers, leather craftsmen
si/sx of MM
- bone pain*
- weakness, fatigue, wt loss
- dyspnea
- fever
- repeated infx
- SC compression
PE findings for MM
- pallor
- organomegaly
- radiculopaty
- peripheral neuropathy
lab findings for MM
- hyper Ca
- increased serum proteins
- anemia
- ARF
- low anion gap
major criteria for MM dx
- plasmacytosis on tissue bx
- BM > 30% plasma cells
- M spike on SPEP or UPEP
M spike levels that are diagnostic for MM
- IgG > 35 on SPEP
- IgA > 20 on SPEP
- > 1g/24 hours on UPEP
minor criteria for MM dx
- BM 10-30% plasma cells
- M spike lower than major criteria
- lytic bone lesion on xray
- abnormally low immunoglobulin levels
dx of MM
- 1 major + 1 minor criteria
OR - 3 minor criteria
what is the classic triad of MM
- lytic lesions
- BM plasma > 30%
- M spike on SPEP or UPEP
what are bad prognostic indicators for MM
- “CRAB”
- hyperCalcemia
- renal failure
- anemia
- boney lytic lesions
what staging criteria are used for MM
- durie salmon (I-IIIb)
- international staging systems ( I-III)
tx for MM
- no cure
- low threshold for infx- treat infx
- HSCT gives pt 5 extra years
- analgesia
- hydration
- plasmapheresis
- xrt
prognosis of MM
- fatal within 4-5 years without HSCT
- death d/t progression, renal failure, sepsis, tx related leukemia or myelodisplasia
what is MGUS
- “pre-MM”
- M spike
- no sx
- < 10% plasma cells
what is smoldering MM
- “pre-MM”
- meets dx criteria for MM but no end organ damage
essential thrombocytosis
- myeloproliferative disorder
- produce too many dysfunctional platelets
- usu dx incidentally
- tx: plasmapheresis
what is the most common hematologic malignancy
- non-hodgkins lymphoma
- common in adults > 50
non-hodkins lymphma (NHL)
- over 35 dif types
- majority are B cell
- classified as indolent, aggressive, or highly aggressive
indolent NHL
- follicular
- survival is years if untreated
aggressive NHL
- Diffuse large B cell lymphoma (DLBCL)
- survival is months if untreated
highly aggresive NHL
- burkitts lymphoma
- survival is weeks if untreated
RF for NHL
- family hx
- prev xrt or chemo
- immunosuppressives
- HTLV1, HIV, EBV, Hep B/C
- inflammatory GI disease
- autoimmune disorders
si/sx of NHL
- unexplained fever*
- night sweats, fatigue, wt loss
- painless LAD
- cough or CP
- bloating
- rashes
- hepato/ splenomegaly
extranodal involvement in NHL
- CNS
- bone
- GIT
- dermis
- testicular- most common testitcular ca if > 60
- waldeyer’s ring
dx of NHL
- excisional LN bx
- bone marrow aspiration
- LP if suspect CNS involvment
- PET for staging
prognosis of NHL
- determined by:
- immunophenotype
- cytogenetics
- growth fraction
- cytokine production
staging I lymphoma
- disease in single region
stage II lymphoma
- 2+ LN on same side of diaphragm
stage III lymphoma
- LN above and below diaphragm
stage IV lymphoma
- widespread disease
- multiple organs
modified ann arbor staging
- used for lymphoma
- e= extranodal
- s= spleen
- x= bulky LN > 10 cm
follicular NHL
- indolent
- grows/ spreads slowly
- second most common type of NHL
what causes follicular NHL
- translocation between chromosome 14 and 18 -> BCL2 gene
- cells dont undergo apoptosis
prognosis of follicular NHL
- based on flipi score
- nodal involvement
- LDH level
- age > 60
- stage III or IV disease
- Hgb < 12
diffuse large B cell lymphoma (DLBCL)
- aggressive NHL
- most common type of NHL
what usu causes DLBCL
- long term immunosuppressive tx
cell markers for DLBCL
- CD19
- CD20
- CD79a
tx for DLBCL
- everyone gets chemo
- stage I and II: 3-4 cycles
- stage III and IV: 6-8 cycles
tx for follicular NHL
- stage I and II: xrt
- stage III and IV: chemo
burkitt’s lymphoma
- highly aggressive NHL
- affects kids in Africa
si/sx of burkitt’s lymphoma
- mandibular/ facial bone tumor
- may have abd mass with ascites
- renal and testicular involvement
- spreads to CNS and bone marrow
- can be assoc with HIV
dx of burkitt’s lymphoma
- starry sky appearance on bx
- MYC translocation- chromo 8 and 24
prognosis of burkitt’s
- rapidly fatal if untreated
- good prognosis/ cure rates with tx
T cell lymphoma
- uncommon type of NHL
- have a lot of cutaneous invovement
subtypes of T cell lymphoma
- precursor T lymphoblastic lymphoma
- peripheral T lymphoma
tx of T cell lymphoma
- topical: cutaneous and oral disease modifiers
- systemic: poor outcomes -> early clinical trials, combo chemo, novel biologics
- Car T cell tx
hodgkin’s lymphoma
- reed sternberg cells
- potentially curable
- subclassified as classical vs nodular lymphocyte predominant HL
- bimodal peak: 25-30 and > 55
reed sternberg cells
- seen in HL
- express CD30 and CD15 antigens
- have multiple nuclei
how does HL spread
- starts in single node/ chain of nodes
- spreads contiguously
RF for HL
- male predominance
- SES and enviornment
- EBV
- mono
- smoking
- immunosuppression
- autoimmune disease
- family hx
decreased risk for HL
- breast feeding
- chicken pox
- MMR
- pertussis
si/sx of HL
- painless LAD above diaphragm*
- cyclic fevers*
- night sweat
- CP/ cough
- pruritis
- LN pain s/p alcohol ingestion
- cerebellar degeneration
- erythema nodosum
dx of HL
- LN bx and histology
- BM bx
- plain xrays show mediastinal mass
- CT, PET
- ESR for staging
- LDH- marker of disease burden
tx of HL
- based on stage and clinical scenario
- early favorable vs. early unfavorable vs advanced
tx for early favorable HL
- 3-4 cycles of chemo
- xrt
tx for early unfavorable HL
- 4-6 cycles of chemo
tx for advanced HL
- 6-8 cycles of chemo
- xrt for bulky disease or residual activity on PET
criteria for favorable early HL
- < 2 sites
- no extranodal invovlement
- no mediastinal mass > 1/3 thoracic diameter
- ESR < 50 or <30 with b sx