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Hematology

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