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INTRODUCTION

Epidemiology

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Epidemiology
Incidence: 21,040 (male: 12,930; female: 8110. Estimated new cases for 2020 in the United States). 191,000 (worldwide). Incidence rates: 6.0 and 3.3 cases per 100,000 population per year among men and women respectively Stage at Presentation (Rai):
Stage 0: 31%
Stage I/II: 59%
Deaths:

4060 (male: 2330; female: 1730. Estimated deaths for 2020 in the United States).

Estimated 61,000 per year worldwide

Stages III/IV: 10%
Median age: 70 years  
Male to female ratio: 1.3:1

Global Burden of Disease Cancer Collaboration et al. JAMA Oncol 2017:3:524–548

Siegel R et al. CA Cancer J Clin 2020;70:7–30

Surveillance, Epidemiology and End Results (SEER) Program, available from http://seer.cancer.gov (accessed in 2020)

Work-up

Essential

  1. Medical history and PE: attention to node-bearing areas, including the Waldeyer ring, and to size of liver and spleen

  2. Performance status

  3. B-symptoms

  4. Laboratory work-up: CBC with differential, LDH, comprehensive metabolic panel

Useful in certain circumstances

  1. Quantitative immunoglobulins

  2. β2-Microglobulin

  3. Reticulocyte count, haptoglobin, direct Coombs test

  4. Uric acid

  5. Chest, abdomen, and pelvis CT scans with contrast of diagnostic quality prior to initiation of therapy

  6. Hepatitis B testing if CD20 monoclonal antibody contemplated

  7. Pregnancy testing in women of child-bearing age (if chemotherapy planned)

  8. Unilateral bone marrow biopsy + aspirate at initiation of therapy

  9. MUGA scan/echocardiogram if anthracycline-based regimen is indicated

  10. PET/CT scan to direct nodal biopsy, if histologic transformation is suspected

  11. Discussion of sperm banking and fertility issues

Informative for prognostic and/or therapy determination

  1. CpG-stimulated metaphase karyotype

  2. FISH analysis to detect: +12, del(11q), del(13q), del(17p)

  3. TP53 sequencing

  4. Molecular analysis to detect immunoglobulin heavy-chain variable gene (IGHV) mutation status

Diagnosis

NCI working group diagnostic criteria

  1. Absolute lymphocytosis in the peripheral blood with a count of ≥5 × 109 B lymphocytes and cells morphologically mature in appearance

  2. The clonality of the B cells must be confirmed by flow cytometry

  3. The monoclonal B-cell lymphocytes express low levels of surface immunoglobulins, simultaneously with CD5, CD23, CD19, and CD20

 

Hallek M et al. Blood 2008;111:5446–5456

Prognosis

Unfavorable risk factors

  1. Advanced clinical stage

  2. Rapid lymphocyte doubling time (<6 months)

  3. TP53 mutation by DNA sequencing, and/or del(17p)

  4. Unmutated IGHV genes (≤2 % mutation)

  5. Diffuse bone marrow lymphocytic involvement

  6. del(11q) by interphase (FISH) cytogenetics (normal karyotype and trisomy 12 have an intermediate prognosis, while 13q deletion has a good prognosis)

  7. Complex karyotype (≥3 unrelated) on conventional karyotyping

  8. CD38 (>30%), ZAP-70 expression (>20%), CD49d (>30%) measured via flow cytometry

 

Döehner H et al. N Engl J Med 2000;343:1910–1916

Staging and Survival

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Staging and Survival
...
Kay NE et al. Hematology Am Soc Hematol Educ Program 2002:193–213
Rai Lymphocytosis Lymph Node Enlargement Spleen/Liver Enlargement Hemoglobin <11 g/dL Platelets <100,000/mm3 Survival (Years)
0 Yes No No No No >13
I Yes Yes No No No 8
II Yes ± Yes No No 6
III Yes ±

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