170 Chronic Lymphocytic Leukemia TREATMENT ISSUES MANAGEMENT (CONT’D) allogeneic SCT if HLA matched donor available and eligible for transplant; otherwise, consolidation chemotherapy MAINTENANCE THERAPY POMP (6 mercaptopur ine daily, methotrexate weekly, vincristine and pre dnisone monthly) or dexamethasone for years, except for patients who received allogeneic SCT HIGH RISK SURVIVORSHIP ISSUES risk of secondary malig nancies, neurologic sequelae, cardiotoxicity, inferti lity, depression, anxiety, and fatigue Related Topics Febrile Neutropenia (p 236) Tumor Lysis Syndrome (p 228) Chronic Lymphocytic Leukemia NEJM 2005 352:8 DIFFERENTIAL DIAGNOSIS OF LYMPHOCYTOSIS INVESTIGATIONS NEOPLASTIC BASIC CHRONIC LYMPHOCYTIC LEUKEMIA (CLL, most com mon cause) PROLYMPHOCYTIC LEUKEMIA mantle cell lym phoma, lymphoplasmacytic lymphoma, follicular lymphoma, marginal zone lymphoma, hairy cell leukemia LEUKEMIC PHASE OF LYMPHOMAS CBCD, smear (smudge cells), lytes, urea, Cr, Ca, PO4, Mg, uric acid, LDH, b2 microglo bulin, albumin, quantitative immunoglobulin, serum protein electrophoresis, urinary protein electrophoresis LABS PERIPHERAL BLOOD FLOW CYTOMETRY FOR SURFACE MARKERS LARGE GRANULAR CELL LYMPHOCYTE LEUKEMIA SPECIAL INFECTIONS pertussis, infectious mononucleo sis, hepatitis, toxoplasmosis BONE MARROW BIOPSY LYMPH NODE BIOPSY monospot test, hepatitis serol ogy if need to rule out other causes MICROBIOLOGY PATHOPHYSIOLOGY WHO CLASSIFICATION CLL is identical to small lymphocytic lymphoma (SLL, mature B cell non Hodgkin’s lymphoma) Traditionally, CLL diagnosis is made from peripheral blood, while SLL diagnosis is made from lymph node biopsy TRANSFORMATION OF CLL prolymphocytic leu kemia 10%, diffuse large B cell lymphoma (Richter’s transformation) 10%, Hodgkin’s disease 0.5%, mul tiple myeloma 0.1% CLINICAL FEATURES ORGAN INFILTRATION lymphadenopathy (80%), splenomegaly (50%), hepatomegaly, skin and lung infiltration, gastric erosions PERIPHERAL BLOOD lymphocytosis with smudge cells, anemia, thrombocytopenia CONSTITUTIONAL weight loss, fever, night sweats, fatigue, anorexia ASSOCIATED SYNDROMES ITP, hemolytic anemia, pure red cell aplasia, cryoglobulinemia, MPGN, hypo gammaglobulinemia, monoclonal gammopathy SECOND MALIGNANCIES non melanoma skin cancer 4.7%, sarcomas 3.3%, kidney 2.8%, lung 2%, prostate 1.5% DIAGNOSTIC AND PROGNOSTIC ISSUES NCI WORKING GROUP DIAGNOSTIC CRITERIA PERIPHERAL BLOOD absolute lymphocyte count in the >5Â103/mL, with !1 B cell marker (CD19, CD20, CD23) and CD5; >55% atypical cells BONE MARROW a normocellular to hypercellular marrow with >30% lymphocytes Interstitial/nod ular pattern (70%) has a better prognosis than diffuse/extensive pattern (30%) IMMUNOPHENOTYPE CD5+, CD19+, CD20+, CD23+, CD43+, CD10 , SIg+ NOTE for patients with lymphocyte count 10Â103/mL, lymphocyte phenotyping is required RAI STAGING lymphocytosis in blood or bone marrow Median survival >150 months I lymphocytosis + lymphadenopathy Median survival 101 months II lymphocytosis + organomegaly Median survival 71 months III lymphocytosis + anemia ( 10 cm [>3.9 in.] max imum dimension of a nodal mass A no B symptoms B weight loss >10% over months, fever >388C [>100.48F], drenching night sweats INVESTIGATIONS BASIC CBCD, peripheral smear, lytes, urea, Cr, AST, ALT, ALP, bilirubin, Ca, LDH, ESR, albumin, quantitative immunoglobulin, serum protein electrophoresis, HCV, HBV, and HIV serology IMAGING CXR, CT chest/abdomen/pelvis, PET scan LYMPH NODE BIOPSY referral to surgery SPECIAL BONE MARROW BIOPSY if B symptoms, Hb