Lymphomas Flashcards

1
Q

What are the lymphocytes

A

T cells: most of the lymphocytes, mature in thymus
B cells: mature in marrow, go into circulation
NK cells

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2
Q

What are the primary lymphatic organs

A
Where B and T cells mature and differentiate 
Bone marrow (b cells) Thymus (t cells)
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3
Q

What are secondary lymphatic organs

A

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

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4
Q

What are NK cells

A

Sniff out cancer cells all the time

Live for 40-60 years

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5
Q

What is your primary vs secondary response

A

Primary: IgM
Secondary: IgG

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6
Q

What causes lymphomas

A

Arise from any step of maturation-
Antigen exposure from EBV or HL
Chronic irritation in extra nodal marginal zone
Random mutations (BCL6)

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7
Q

What is Non-hodgkins lymphoma

A
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
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8
Q

What is Hodgkins lymphoma

A

Bimodal (20’s and 50’s)
Reed sternberg cells
Painless LAD
fever, weight loss, night sweats, generalized pruritis

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9
Q

What is the NHL staging system (or any lymphoma?)

A
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
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10
Q

NHL labs may show

A
CBC: abnormal 
CMP: normal 
LDH: elevated 
Beta 2 microglobulin: often elevated 
Uric acid: elevated
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11
Q

Where can NHL arise form

A

T cells: cutaneous or peripheral lymphoma

B cells: diffuse large B cell, follicular, mantle cell

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12
Q

How do you diagnose NHL

A
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
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13
Q

How does Cutaneous T cell lymphoma present

A

Scaly psoriasis-like rash that dos not clear with steroids or vitamin D analogs

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14
Q

How do you treat NHL

A

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

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15
Q

What is the prognosis of NHL

A

Indolent: 6-8 years (can progress to aggressive)

Elderly, high LDH, high stage, poor performance: worse

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16
Q

What is Hodgkin’s disease

A

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

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17
Q

When can you use the watchful waiting approach

A

ONLY if elderly, or like about to die

18
Q

How do you treat Hodgkins lymphoma

A

Radiation: early stage or bulky disease

Standard Chemo: AVBD (adriamycin, bleomycin, vinblastin, dacarbazine)

19
Q

What is the prognosis of hodgkins lymphoma

A

Ia-IIa: 10 year is 80%

IIIb-IV: 5 yr is 50%

20
Q

How exactly do you implement Hodgkins Tx in different stages

A

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

21
Q

Patients with hodgkins are at increased risk for

A
other lymphomas 
Leukemia 
gastric CA 
lung CA
breast CA (esp in women 30-35); radiation to chest has same risk as BRCA mutation
22
Q

If a patient presents with AKI, keep this in mind on your DDx

A

Multiple myeloma!
in MM kidney disease may be 2/2 hypercalcemia or accumulation of gammaglobulins (specific light chains on immunoglobulins)

23
Q

What is multiple myeloma

A

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

24
Q

Characteristics of multiple myeloma disease are

A

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

25
What are plasmacytomas
Plasma cells in multiple myeloma that can cause tumors (like her pt that came in with a "tumor" on his clavicle
26
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)
27
On PE of MM you may see
``` Hepatosplenomegaly LAD* retinal vein enlargement AKI Hypercalcemia ```
28
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
29
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)
30
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*) ```
31
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.. ```
32
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
33
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
34
Indications to treat multiple myeloma are
``` CRABI! Calcium- rising Renal insufficiency Anemia Bone lytic lesion Infections ```
35
MM treatment is based on
high vs intermediate vs low risk | Chromosomes, stage, and presentation (AKI or hypercalcemia)
36
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
37
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)
38
What are S/Sx of hypercalcemia
``` stones (calcium stones) bones (lytic lesions) groans (constipation) moans physh overtones (confusion) ```
39
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
40
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
41
How can you diagnose Waldenstrom macroglobinemia
SPEP: IgM spike*** PE: hepatosplenomegaly, retinal vein occlusion (sausage link)
42
How do you treat Waldenstrom macroglobinemia
Emergent electropharesis | Chemo CHOP- Cyclophosphamide, Doxarubicin, Oncovin, Prednisone