Lymphomas Flashcards
What are the lymphocytes
T cells: most of the lymphocytes, mature in thymus
B cells: mature in marrow, go into circulation
NK cells
What are the primary lymphatic organs
Where B and T cells mature and differentiate Bone marrow (b cells) Thymus (t cells)
What are secondary lymphatic organs
Where mature antigen specific B and T cells interact with each other
where APC that recognize antigens mount a response
Spleen, lymph nodes, tonsils, MALT
What are NK cells
Sniff out cancer cells all the time
Live for 40-60 years
What is your primary vs secondary response
Primary: IgM
Secondary: IgG
What causes lymphomas
Arise from any step of maturation-
Antigen exposure from EBV or HL
Chronic irritation in extra nodal marginal zone
Random mutations (BCL6)
What is Non-hodgkins lymphoma
has a lot of sub-types range from indolent to very aggressive Painless LAD* Fever, night sweats, weight loss Classification based on biology and cytology
What is Hodgkins lymphoma
Bimodal (20’s and 50’s)
Reed sternberg cells
Painless LAD
fever, weight loss, night sweats, generalized pruritis
What is the NHL staging system (or any lymphoma?)
I: one node II: two nodes, same side of diaphragm III: crosses diaphragm IV: in BM or liver Type A: absence of B Sx Type B: fever, night sweats, weight loss
NHL labs may show
CBC: abnormal CMP: normal LDH: elevated Beta 2 microglobulin: often elevated Uric acid: elevated
Where can NHL arise form
T cells: cutaneous or peripheral lymphoma
B cells: diffuse large B cell, follicular, mantle cell
How do you diagnose NHL
CT to check for nodes PET/CT check biologic activity BM Bx (BM involvement?) LP (meningeal involvement?) *must Bx nodes to find out type of lymphoma Skin Bx for cutaneous T cell lymphoma
How does Cutaneous T cell lymphoma present
Scaly psoriasis-like rash that dos not clear with steroids or vitamin D analogs
How do you treat NHL
Watchful approach: good for low grade, elderly, little node involvement- Follow w/ CT and PET
Chemo low grade (after watchful): CVP! cyclophosphamide, vincristine, prednisone
Chemo high grade: CHOP +/- rituxan (cyclophosphamide, doxyrubicin, oncovin, prednisone)- and local radiation
Very high risk: Autologous SCT as soon as MF possible
What is the prognosis of NHL
Indolent: 6-8 years (can progress to aggressive)
Elderly, high LDH, high stage, poor performance: worse
What is Hodgkin’s disease
May be caused by EBV* outbreak
presents with fever, night sweats, wt loss, painless LAD (nodes hurt after drinking EtOH*)
Get a CBC and EBV titer to r/o mono
When can you use the watchful waiting approach
ONLY if elderly, or like about to die
How do you treat Hodgkins lymphoma
Radiation: early stage or bulky disease
Standard Chemo: AVBD (adriamycin, bleomycin, vinblastin, dacarbazine)
What is the prognosis of hodgkins lymphoma
Ia-IIa: 10 year is 80%
IIIb-IV: 5 yr is 50%
How exactly do you implement Hodgkins Tx in different stages
I/IIA: ABVD x 6 cycles (or 2-4 cycles w/ IFR)
I/IIB: involved field radiation (IFR) or extended field radiation (EFR)
III-IV: ABVD x 6-8 cycles, IFR. Autologous SCT if relapsed. Bretuximab if relapse after SCT
Patients with hodgkins are at increased risk for
other lymphomas Leukemia gastric CA lung CA breast CA (esp in women 30-35); radiation to chest has same risk as BRCA mutation
If a patient presents with AKI, keep this in mind on your DDx
Multiple myeloma!
in MM kidney disease may be 2/2 hypercalcemia or accumulation of gammaglobulins (specific light chains on immunoglobulins)
What is multiple myeloma
Plasma cells increase abnormally from a single clone; cells don’t need to be triggered by an antigen to mature
Cells expand causing anemia, bone pain, kidney disease
Characteristics of multiple myeloma disease are
Clones of plasma cell proliferate
MC 65 y/o
Plasma cells secrete OAF (osteoclast activating factors) that eat the marrow and cause cytopenias, anemias, and bony pain
Immunoglobulins produce hyperviscosity
What are plasmacytomas
Plasma cells in multiple myeloma that can cause tumors (like her pt that came in with a “tumor” on his clavicle
S/Sx of multiplemyeloma are
fatigue
bony pain
frequent infections (immunoglobulins decreased 2/2 low plasma cells)- is this like all others are low besides the 1 that is proliferating?
Mucosal bleeding or alterations in mental status (2/2 hyperviscosity)
AKI
Hypercalcemia (Ca 14-15 range)
On PE of MM you may see
Hepatosplenomegaly LAD* retinal vein enlargement AKI Hypercalcemia
Labs for MM may show
Anemia!
hypercalcemia, elevated BUN/Cr
**Rouleaux formation (falsely elevated MCV)
M spike on Sr protein electrophoresis!
Immunofixation (monoclonal Ig- all one type! like IgG)
*Bence Jones proteins on urine electrophoresis
Total protein rises + elevated globulin + decreased albumin
Example MM lab read
Hgb: 9
Hct: 27%
MCV: 89
Total protein: elevated w/ elevated globulin protein
Na+ low (2/2 gammaglobulin levels being high)
Ways to diagnose Myeloma are
Increased Serum Immunoglobulins Bm Bx* (definitive!)- >20-30% plasma cells Rouleaux formation Elevated Sr protein, low albumin -SPEP -Immunofixation (shows type) -24 hr UPEP (urine) -PET scan (check for lytic lesions- esp on skull*)
How do you stage multiple myeloma
Depends on organization! International system: I: Beta 2 micro and albumin >3.5 II: neither I or II III: Beta2 micro >5.5 -i dont get it..
What is the prognosis of MM
10% indolent
15% die in first 3 months of dx
Chronic phase: 2-5 years before terminal phase (BM failure)
46% die in chronic phase (renal failure, sepsis)
26% die in acute terminal phase
What is the acute terminal phase of MM
They are no longer eligible for treatment so you just do palliative care and wait until they pass
Indications to treat multiple myeloma are
CRABI! Calcium- rising Renal insufficiency Anemia Bone lytic lesion Infections
MM treatment is based on
high vs intermediate vs low risk
Chromosomes, stage, and presentation (AKI or hypercalcemia)
Common MM drug regimens are
“-ide” + steroid
Velcade / Revlamid / Thalidomide / Pomolidamide + Decadron
End stage: Kyrpolis- steroids rupture cells
Younger pt: Autologous SCT
Monthly IV Zoledronic acid (bisphosphanate) for lytic lesions
Xgeva (?)
local radiation for plasmacytoma
F/u with nephrology
AKI in MM can be due to
gammaglobulins getting stuck in tubules, can be reversed (myeloma kidney may need dialysis and plasmapharesis)
Extreme dehydration from hypercalcemia (**Vigorous hydration, zoledronic acid)
What are S/Sx of hypercalcemia
stones (calcium stones) bones (lytic lesions) groans (constipation) moans physh overtones (confusion)
What is MGUS
Monoclonal Gammopathy of Undetermined Significance
similar to MM!
Elevated Immunoglobulin on SPEP w/ lytic lesions, increased plasma cells in BM, or light chains in urine (bence jones)
BUT*** some will develop more severe dz- myeloma, amyloidosis, CLL, or lymphoma
What is Waldenstrom Macroglobinemia
Diseased B cells containing lymphocytic and plasma cell components (AKA- Lymphoplasmacytic lymphoma)
Arise from BM but does NOT cause lytic lesions
Proliferation of IgM (large, macromolecule)
S/Sx are weakness, fatigue, epistaxis, dizziness
How can you diagnose Waldenstrom macroglobinemia
SPEP: IgM spike***
PE: hepatosplenomegaly, retinal vein occlusion (sausage link)
How do you treat Waldenstrom macroglobinemia
Emergent electropharesis
Chemo CHOP- Cyclophosphamide, Doxarubicin, Oncovin, Prednisone
