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shared:heme:cll

Chronic Lymphocytic Leukemia

Workup

  • Physical Exam
  • Baseline labs
  • Imaging: CT > PET
  • Repeat peripheral flow
  • Bone marrow biopsy
  • HBV, HCV (less important now that rituxamab falling out of favor)

Staging

  • Biret
    • A: <3 lymphoid
    • B: >3 lymphoid
    • C: Hgb < 10 or platelets < 100
  • Rai
    • 0: lymphocytosis
    • I: lymphadenopathy
    • II: hepatosplenomegaly
    • III: Hgb < 11
    • IV: platelets < 100

Diagnosis

  1. B cells > 5 x 10^9
  2. lymphadenopathy or splenomegaly
  3. cytopenia
  4. CLL-related symptoms
  • if meets bottom three criteria, then small lymphocytic lymphoma

Treatment

  • first-line
    • ibutrinib (BTK inhibitor)
      • adverse effects: diarrhea, rash, bleeding, atrial fibrillation
    • venetoclax (now 1st line)
  • refractory:
    • idelalisib
    • immunotherapy
      • allo-SCT
      • CAR-T
      • lenalidomide
      • checkpoint inhibitors

Indications

  • Bone marrow failure: Hgb < 11, platelets < 100
  • Symptoms
    • weight loss >10% over 6 months
    • ECOG ≥ 2
    • T ≥ 38°C for 2 weeks
    • night sweats
  • Splenomegaly (>6 cm or symptomatic)
  • Lymphadenopathy (>10 cm or symptomatic)
  • Progression of disease
    • ALC increase >50% in 2 months
    • ALC doubling time <6 months

Complications

  • infection
  • autoimmune (5-10%)
    • AIHA
    • ITP
  • pure red cell aplasia
  • malignancy
    • DLBCL
    • Skin: 5-10x risk SCC/BCC

Poor prognostic factors

  • 17p13 deletion
  • 11q22.3
  • Trisomy 12
  • 13q14
  • NOTCH, SF3D1?
  • hypermutation?
  • beta-2 macroglobulin
shared/heme/cll.txt · Last modified: 2020/02/11 17:53 by 127.0.0.1