Definitionaccululation of mature B cells that have escaped programmed cell death and undergone cell-cycle arrest at G0/G1 phase
Epidemiology commonest leukemia (>25%) M:F = 2:1 mutations, trisomies, deletions influence risk may be triggered by pneumonia
History incidental finding may be anemic / infection-prone if severe: weight loss, sweats, anorexia
Physical Examinationenlarged, rubbery, non-tender LNs
Investigation findings markedly elevated lymphocytes (later) autoimmune haemolysis, marrow infiltration (low Hb, low neutrophils, low platelets)
Complications autoimmune haemolysis increased infection d/t hypogammaglobulinemia (decreased IgG)- bacterial- viral, esp. herpes zoster marrow failure
Treatment**more likely to be indicated if symptomatic, immunoglobulin genes (IGHV) are unmutated, 17p deletions (-> consider intensive Rx) fludarabine + cyclophosphamide is better than chlorambucil alemtuzumab steroids help autoimmune hemolysis Radiotherapy to relieve lymphadenopathy / splenomegaly (supportive care) transfusions, IV human Ig if recurrent infection stem cell transplant can be tried
Prognosis 1/3 never progress, 1/3 progress in time, 1/3 are actively progressing some remain status quo for years, or even regress usually nodes slowly enlarge (+/- lymphatic obstruction) death is often d/t1. infection- pneumococcus- haemophilus- meningococcus- Candida- aspergillosis2. transformation to aggressive disease (Richter's syndrome)
CLL
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