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Session 6
Disease of WBC, Lymph...
41
Medical
Graduate
02/24/2011

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Cards

Term
Does not contain Reed-Sternberg cells
Definition
Diffuse Large Cell Lymphoma & Follicular Lymphoma
Term
Eval for Classical HL
Definition
-H & P
-CBC with differential, CMP, LDH, ESR
-CT scan, Ab, Pelcis PET-CT
-BM biopsy
...
Term
What is the malignant cell in Hodgkins Lymphoma?
Definition
Reed- Sternberg Cell (B Cell)
-eosinophils, t cell, MP and NK cells are all reactive cells
Term
Associated with Hodgkin's Lymphoma
Definition
-EBV
-Family history
-High standard of living
-Immunosuppression
-Young age
-Old age
Term
Staging of HL (I, II, III, IV)
Definition
I: 1 LN area either above or below diaphragm
II: 2 LN on same side of diaphragm
III: LN on both sides of diaphragm
IV: involvement of extra-lymphatic tissue (BM, Liver, Lung)

"Ann Arbor Staging System"
Term
HL "b" symptoms
Definition
Fever (100.4 or greater at least 1/day)
Night Sweats (drenching)
Weight Loss ( > 10% of baseline weight)
Term
SLIDE 29 flow
Definition
NHL:
-B cell: aggressive, intermediate, indolent
-T cell: peripheral T Cell lymphoma, cutaneous T Cell Lymphoma

HL:
-Nodular sclerosis
-Lymphocyte depleted
-Lymphocyte predominant
-mixed cellularity
Term
HL cells spread in a relatively orderly fashion to contiguous sites
Definition
TRUE
Term
ABVD therapy stands for
Definition
Adriamycin, Bleomycin, Vinblastine, and Dactinomycin
Term
Bleomycin SE
Definition
minimal BM toxicity, dose-related pneumonitis & pulmonary fibrosis
*monitor pulmonary toxicities (ie: SOB, cough, rales)
Term
Vinca Alkaloids: Vinblastine & Vincristine
Definition
MOA: spindle poison: binds tubulin and prevents polymerization of tubulin to form microtubules (disrupts mitotic spindles & decreases mitosis)

Clinical Use:
Vinblastine: HL (ABVD)
Vincristine: acute lymphocytic leukemia (ALL), HL (MOPP)

SE:
Vinblastine: BM depression
Vincristine:
-neurotoxicity (peripheral neuropathy, peresthesis)
-Low incidence of BM depression
Term
Dacarbazine
Definition
SE: myelosuppression-mild, NV

USES: HL (ABVD)

MOA: alkylating agent
Term
What causes Acute Lymphocytic Leukemia (ALL)?
Definition
Unknown
Term
What is most important in the evaluation of suspected ALL?
Definition
Peripheral blood for flow cytometry (fastest & necessary) , BM biopsy and aspirate, BM cytogenetics, BM flow cytometry, BM FISH for common genetic abnormalities
Term
Classification of ALL:
Definition
A. Morphology
L1: uniformity of cell morphology
L2: cariation in size and shape
L3: characteristic cytoplasmic granules (white inclusion bodies)

B. Immunophenotype
Term
Bad prognostic factors for ALL
Definition
-Male
-afroamerican
-WBC > 30,000 Dx
-CNS involvement at Dx
-Mature B cell phenotype
- Ph + (9,22)
-Hypodipoidey

children: 60-80 cure rate
Adults 30-50 cure rate
Term
Management of ALL
Definition
-induction chemotherapy: followed by intensification chemotherapy occasionally with BM transplant from matched sibling or matched unrelated donor
-Drugs 'hyperCVAD' cyclophosphamide, vincristine, daunorubicin, dexamethasone, cytrarabine, methotrexate, intrathecal therapy
Term
cytophosphamide (drug for ALL)
Definition
MOA: alkylating agent, binds DNA and decreases replication
-a prodrug, req activation by hepatic cytochrome P-450
-used orally for ALL (hyperCVAD), HL (CHOP) and ovarian and breast cancer (CMF)

SE: myelosuppression, NV, Hemorrhagic cystitis (Mesna, a thiol drug, is given as uroprotectant to bind to the reactive intermediate and decrease it's toxicity) + VIGOROUS hydration
Term
Adrenocorticosteroids (ie: dexamethasone) for ALL
Definition
MOA: immunosuppressive, interfere w/ lymphocytic proliferation by inhibiting mitosis and have a lympholytic effect that causes regression of lymphatic tissue

Used: acute and chronic LL

SE: cushings habitus
Term
Pyrimidine antagonists (cytarabine)
Definition
MOA: inhibits DNA Pol
Used: IV, AML and HL
SE: myelosuppression
Term
Folic Acid antagonists (methotrexate) (Tx ALL)
Definition
MOA: competitively inhibits DHF Reductase (thus cannot get tetrahydrofolate for DNA synth), inhibition reversed by folinic acid

Uses: ALL: intrathecally to decrease relapse of CNS leukemia
DOC for Tx of choriocarcinoma

SE: dose-related myelosuppression, hepatic cirrhosis & fibrosis, and Teratogenesis!!!
Term
Least likely DDx for CLL case 3?
Definition
-Follicular Cell Lymphoma b/c doesn't circulate and has no lymphadenopathy

CLL, MCL, HCL, MZL all circulate.
Term
What is the most important test in the initial veal of suspected CLL?
Definition
-peripheral blood for flow cytometry MOST IMP
but also imp...
-BM biopsy, aspirate, cytogenetics etc...
Term
Staging for CLL
Definition
Stage 0- low risk: lymphocytosis
Stage I- intermediate: lymphocytosis, LAN
Stage II - high: Lymphocytosis + organomegally
Stage III- high Lymphocytosis+ anemia
Stage IV- high Lymphocytosis+ thrombocytopenia
Stage A- < 3 LN bearing sites
Stage B- > 3 LN bearing sites
Stage C- anemia or thrombocytopenia

Case 3: Stage 0 or Stage A
Term
T/F
Proliferation centers are pathognomonic for CLL/SLL?
Definition
TRUE
Term
Prognostic Criteria for CLL
Definition
Significant: genetic abnormalities, VH gene mut, CD38, ZAP-70, Beta-2 microglobulin, lymphocyte doubling time
Term
Tx for CLL:
Definition
-FCR: fludarabine, cytoxan, rituxan
-Rituxan-Bendamusine
-Rituxan single agent
-Campath
-CHOP chemo
-Allogeneic BM transplant
Term
For CLL:
Fludarabine
Definition
MOA: purine analog and interferes with DNA synth and repair by --I DNA pol

Used: CLL

SE: dose-lmting myelosuppression
Term
for CLL:
Rituximab
Definition
MOA: monoclonal Ab targeted against CD20 Ag on B cells

SE: infusion reactions (ie: hypotension...)
Term
for CLL:
Alemtuzumab
Definition
MOA: recomb humanized monoclonal Ab that is directed against CD52--deplete cells by Ab-dependent lysis

Uses: CLL in patients with refractory to FCR regimen--IV

SE:monitor for opportunistic infxn
Term
CHOP for CLL
Definition
Cyclophasphamide
Doxorubicin (Hydroxydaunorubicin)
Vinicristine (Oncovin)
Prednisone
Term
Issues with Management for CLL
Definition
Infxn: pneumonia, cellulitis

Autoimmune complications: hemolytic anemia, immune thrombocytopenia (ITP), can be refractory tx
Term
Score for Follicular lymphoma:
Definition
FLIPI Score
(follicular lymphoma international prognostic index)
slide 146... know??
Term
Pathophys of MM:
Definition
mature terminally diff plasma cells which secretes Ig as its job.
Term
What test should you do for MM?
Definition
Serum prtn electrophoresis (mjr prtn should be albumin) if we see a monoclonal Ab spike....
Term
MGUS & solitary plasmacytoma
Definition
MGUS:
-present in 2% of 50 y/o
- no clinical criteria to meet dx of plamacytoma or myeloma
-1% risk/year of progression to a clonal disorder

Solitary Plasmacytoma:
-no clinical criteria of myeloma
- radiation is curative
-25% chance of getting myeloma (think!?!)
Term
MM staging system:
Definition
I: hgb > 10, serum Ca normal, xray normal or solitary bone plasmacytoma only
II: neither stage I or III
III:
A: normal renal fxn cr < 2.0 mg/dl
B: abnormal renal fxn due to creatinine level

FINISHN 166
Term
MM Prognosis & Tx:
Definition
markers: increase in B2
FINISH 167
Term
MM Chemo
Definition
MP regime: Melphalan (alkylating agent) Prednisone

VAD regime: Vincristine, Doxorubicin (Adriamycin), Dexamethasone

NEW:
Thalidomide
Term
MM Tx: Thalidomide
Definition
Chemistry: teratogenic effects (during peg: phocomelia)

MOA: --I angiogenesis and is anti inflammatory and immunomodulatory, --I TNF alpha decreasing phagocytosis b neutrophils and increased CMI

USes: MM and Erythema nodosum leprosum

SE:
Teratogenic (closely regulated)
Term
MM Tx: Bortezomib
Definition
MOA: reversible proteosome --I

Uses: MM
SE: N, V, asthenia, peripheral neuropathy
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