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Lymphoma

Hematology

QuestionAnswer
Hodgkin Disease includes (types): Nodular Sclerosing (80%); LPHD; Classical Hodgkin Dz; Mixed cellularity
Non-Hodgkin Disease includes (types): Follicular; Burkitt; Diffuse lg B-cell; Marginal zone; Cutaneous T-cell; Anaplastic large cell
Group of cancers w/enlarged lymphoid tissue, spleen, liver, & Reed-Sternberg cells Hodgkin Dz
Hodgkin Disease prevalence 7,900 cases/year. Bimodal: 20-40 then after 50; rare in kids <5; more common in men 15-45
Virus found in 40–50% of Hodgkin disease cases EBV
Hodgkin Dz mechanism of spread in pt Usually arises in a single area and spreads to contiguous nodes (“next-door disease”)
Hodgkin Dz S/S Painless cervical , supraclavicular, & mediastinal LAD (pain w/drinking alcohol); constitutional B symptoms; SOB common with mediastinal mass.
Hodgkin Dz findings Nodular sclerosis (esp young pt); Mixed cellularity (esp in older pts); prob infxs etiology in young pts (mononuc, x3 risk); 5-10% = extranodal presentation (lung, liver, bone marrow); Chest Xray: often mediastinal mass
Hodgkin Dz diagnostics CXR, CT chest, abdomen, pelvis; PET scan; LN & bilateral BM bx. HIV test.
Hodgkin Dz Stage I: one lymph node region
Hodgkin Dz Stage II: two lymph node areas on same side of the diaphragm
Hodgkin Dz Stage III: nodal disease, both above and below the diaphragm. Also may be extralymphatic (IIIE) or involving spleen (IIIS) or both (IIISE)
Hodgkin Dz Stage IV: Extranodal disease. A: no sxs. B: sxs. C: bulky disease (eg, mediastinal widening)
Hodgkin Dz mgmt: Stage I and IIa: Radiation alone
Hodgkin Dz mgmt: Stage IIb: Controversial, radiation +/- chemotherapy
Hodgkin Dz mgmt: Stage III: Chemo +/-radiation (ABVD)
Hodgkin Dz mgmt: Stage IV: Chemotherapy
Hodgkin Dz: Five year survival rate: > 80% (Will usually recur in 2 years if at all)
Hodgkin Dz: if relapse: Consider high dose chemo followed by BMT
Hodgkin Dz mgmt Chemo: ABVD; give chemo Q2 weeks x 4-6 cycles. Re-scan after 2-3 cycles to determine response. IFRT follows chemo. Watch for toxicity/neutropenia (use CSFs)
5th most common malignancy in US NHL
NHL prevalence 60,000 new cases annually; median age 50 yrs; incidence higher in pts w/immunodeficiencies, h/o EBV, exposures to pesticides/solvents
Disease arising from cells in lymphoid tissue (90% of cases are derived from B-lympocytes) NHL
3 Grades of NHL Indolent (low grade, slow growing); Intermediate (aggressive, mix of small to large cells); High Grade (very aggressive)
NHL S/S Persistent, painless, isolated or diffuse LAD (retroperitoneum, mesentery, pelvis, extranodal: skin, GI tract); B symptoms (intermediate and high-grade dz); abd pain, N/V, bleeding, edema
NHL findings Normal CBC but poss anemia, thrombocytopenia, & leukopenia; occ lymphoma cells on diff; bulky lymphadenopathy (poss cause jaundice, hydronephrosis, SVC syndrome, bowel obstruction, wasting)
NHL staging CT chest, abdomen, pelvis; PET scan??; Unilateral or bilateral BM bx; LDH (tumor marker); LP if CNS dz is suspected
tumor lysis prophylaxis Allopurinol, 300 mg/day, hydration, diuretics. May be indicated in NHL
Role of surgery in NHL Diagnostic
Mgmt of Low Grade NHL Dynamic observation (watch & wait) average of 6 yrs after dx. Rituximab weekly x4 weeks or R-CHOP or CHOP
Mgmt of Intermediate Grade NHL R-CHOP or R-ICE or Bexxar (radiolabeled I-131) or Zevalin (radioimmunotherapy). ?BMT or SCT in relapse
Mgmt of High Grade Lymphoma (large B-cell lymphoma) R-CHOP or R-ICE. Many lymphomas treated w/specific therapies (Burkitt lymphoma, post transplant lymphoproliferative disorder)
Lymphoblastic lymphomas are treated with: Regimens similar for T-cell ALL
Bulky lymphadenopathy seen in: CLL; NHL
Pel-Ebstein fever pattern is seen in: Hodgkin disease (alternating periods of febrile and afebrile for days-weeks)
Hodgkin disease complications Spinal cord compression. Late-treatment cardio & pulmo dz. 2nd malignancy. Hypothyroid & infertility after radation. Lower immunity.
Ann Arbor system is used to classify stages of: Hodgkin disease and NHL
Working Formulation and REAL classification system are used to classify diagnoses of: NHL (low, intermediate, and high-grade)
Constitutional B symptoms fever, drenching night sweats, wt loss
Rituximab (monoclonal Ab) targets: B-cells SAg CD20 cells
R-CHOP = rituximab plus cytoxan, adriamycin, vincristine, prednisone
Created by: Abarnard