Lymphoproliferative Neoplasms Staging and Prognosis PDF

Lymphoproliferative Neoplasms Staging and Prognosis Stage 0 I II III IV Stage A B C Risk Low Intermediate Intermediate High High Rai Staging (USA) Features at Diagnosis Blood, marrow lymphocytosis Lymphocytosis w adenopathy Lymphocytosis + spleno/hepatomegaly w/wo adenopathy Lymphocytosis and ane...

Lymphoproliferative Neoplasms Staging and Prognosis Stage 0 I II III IV Stage A B C Risk Low Intermediate Intermediate High High Rai Staging (USA) Features at Diagnosis Blood, marrow lymphocytosis Lymphocytosis w adenopathy Lymphocytosis + spleno/hepatomegaly w/wo adenopathy Lymphocytosis and anemia Lymphocytosis and thrombocytopenia Binet Staging (Europe) Features at Diagnosis Blood/marrow lymphocytosis + <3 areas of adenopathy Lymphocytosis + ≥ 3 areas of adenopathy Lymphocytosis + ≥ 3 areas of adenopathy + anemia or thrombocytopenia Chronic Lymphocytic Leukemia (B-CLL, T-CLL) • CLL is defined by monoclonal population of B lymphocytes • CD5, CD19, CD20, CD23 Only lymphocytosis Involves surrounding structures Involves other blood disorders Involves other blood disorders Leukemia or Lymphoma • CLL is blood-born (liquid because leukemia) • SLL-Small Lymphocytic Lymphoma (solid because lymphoma) Clinical Features • “Smudge cells” are common and raise suspicion of diagnosis • Anemia and thrombocytopenia in advanced stages • Posterior iliac crests are common sites for bone marrow aspiration and biopsy • Mature lymphoid cells are present in bone marrow • Patients have low antibodies o Hypogammaglobulinemia o Low on IgG, IgM heavy chain; kappa/lambda light chain • Diagnosis o FISH allows detection of cyto-genetic variants to determine poor prognosis forms (17p and 11q deletions) o Flow Cytometry determines poly or monoclonal: CLL cells express CD19, CD20, and CD5 Immunologic Abnormalities • Hypogammaglobulinemia is common • Imbalance between T4 Helper cells and T8 Suppressor cells in advanced disease • Other autoimmune diseases in CLL: o Rheumatoid Arthritis o Lupus o Hashimoto’s Thyroditis o Grave’s Disease CLL Treatment Indications • Treatment needed in Rai’s high stages III and IV, or Binet stage B or C • Richter Transformation: Evolution of CLL into aggressive leukemias or lymphomas • Specific reasons to treat: o progressive adenopathy o splenomegaly o rapid doubling time of lymphocyte o recurring infections o anemia o thrombocytopenia o autoimmune issues o evolution to PLL or Richter transformation • Treatment: Stem cell transplantation