CLL Cases Flashcards
most common leukemia in adults
Chronic lymphocytic leukemia
tx CLL
allogenic stem cell transplant only curative therapy
Decision to treat based on age, performance status, symptoms, disease stage
Pathophysiology CLL
an uncontrolled clonal accumulation of mature lymphocytes.
Unclear mechanisms of initiation
Molecular characterization of CLL correlates with clinical outcomes
Mutated Ig VH genes
Unmutated Ig VH genes
Flow cytometry CLL
CD5+CD19+CD20+CD23+
Mantle cell lymphoma flow cytometry
CD5+CD23- cyclinD1+
CLL is the same as
SLL
Sxs CLL
Often indolent.
Progressive adenopathy –> fatigue, malaise, weight loss, fevers
Progressive BM involvement –> severe cytopenias, infections
AI sequelae and Richter’s transformation
Autoimmune complications of CLL
AIHA
Pure red cell aplasia
Immune-mediated thrombocytopenia
Neutropenia
Rai staging
Rai 0: lymphocytosis
Rai 1: lymphadenopathy
Rai 2: splenomegaly
Rai 3: HgB
Binet staging
A: lymphocytosis +/- 1-3 sites lymphadenopathy
B: lymphocytosis with > 3 sites lymphadenopathy
C: lymphocytosis + anemia and/or thrombocytosis
Poor prognostic factors CLL
Advanced stage at diagnosis Rapid lymphocyte doubling time Diffuse marrow infiltration Advanced age/male gender CLL-PLL (secondary PLL) Abnormal karyotype Elevated b2-microglobulin, soluble CD23 CD38+ or ZAP-70+
CLL cytogenetic abnormalities
13q- Normal karyotype Trisomy 12 11q- 17p- (nasty) ALWAYS get FISH studies with CLL
CD38 and CLL
surrogate marker for Ig gene mutations
CD38+ = unmutated Ig genes = poorer prognosis
CD38-= mutated Ig genes = better prognosis (>20 yrs median survival)
ZAP-70 expression in CLL
Correlates with Ig VH gene mutations
Correlates CD38+ expression
ZAP-70+ = Poorer prognosis
CLL: when to treat
Constitutional symptoms Progressive lymphocytosis Progressive lymphadenopathy Progressive splenomegaly Progressive bone marrow failure Autoimmune complications Richter’s transformation or secondary PLL Your patient is uncomfortable with observation