Chronic lymphocytic leukemia clinical staging

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]

Overview

According to the Rai Staging System, there are five stages of chronic lymphocytic leukemia based on the degree of lymphocytosis, hemoglobin level, platelet level, presence of splenomegaly, and presence of lymphadenopathy. According to the Binet Staging System, there are three stages of chronic lymphocytic leukemia based on the degree of lymphocytosis, the presence of anemia or thrombocytopenia, and the involvement of three or more lymphoid regions. The tumor stage is considered one of the important factors that determine the optimal management protocol of chronic lymphocytic leukemia patients.

Staging System

Rai Staging System:

According to the Rai Staging System, there are five stages of chronic lymphocytic leukemia based on the degree of lymphocytosis, hemoglobin level, platelets level, presence of splenomegaly, and presence of lymphadenopathy.[1][2][3][4]

Stage Hemoglobin Level Platelets Level Lymphocytosis Splenomegaly Lymphadenopathy Median Survival

Stage 0

>11 g/dL

>100,000/L

Present

Absent

Absent

>150 months

Stage 1

>11 g/dL

>100,000/L

Present

Absent

Present

101 months

Stage 2

>11 g/dL

>100,000/L

Present

Present

Present or absent

71 months

Stage 3

<11 g/dL

>100,000/L

Present

Present or absent

Present or absent

19 months

Stage 4

<11 g/dL or >11g/dL

<100,000/L

Present

Present or absent

Present or absent

19 months

The staging is further modified into 3 groups:[5]

  • Low Risk: Stage 0
  • Intermediate Risk: Stages 1 and 2 combined
  • High Risk: Stage 3 and 4 combined

Binet Staging System

According to the Binet Staging System, there are three stages of chronic lymphocytic leukemia based on the degree of lymphocytosis, the presence of anemia or thrombocytopenia, and the involvement of three or more lymphoid regions: cervical, axillary and inguinal lymph nodes, and spleen and liver.[6][7][8][9]

Stage Lymphocytosis Anemia or Thrombocytopenia Lymphadenopathy Median Survival

Stage A

Present

Absent

2 or less lymphoid regions

Almost normal age and sex adjusted survival rate

Stage B

Present

Absent

>3 lymphoid regions

7 years

Stage C

Present

Present

>3 lymphoid regions

2 years

References

  1. Rai KR, Sawitsky A, Cronkite EP, Chanana AD, Levy RN, Pasternack BS (August 1975). "Clinical staging of chronic lymphocytic leukemia". Blood. 46 (2): 219–34. PMID 1139039.
  2. MacDougall BK, Weinerman BH (1988). "Staging and prognosis of chronic lymphocytic leukemia: the Manitoba experience". J Gen Intern Med. 3 (2): 139–43. PMID 3357070.
  3. Zwiebel JA, Cheson BD (February 1998). "Chronic lymphocytic leukemia: staging and prognostic factors". Semin. Oncol. 25 (1): 42–59. PMID 9482526.
  4. Hallek M (September 2017). "Chronic lymphocytic leukemia: 2017 update on diagnosis, risk stratification, and treatment". Am. J. Hematol. 92 (9): 946–965. doi:10.1002/ajh.24826. PMID 28782884.
  5. Hallek M, Cheson BD, Catovsky D, Caligaris-Cappio F, Dighiero G, Döhner H, Hillmen P, Keating MJ, Montserrat E, Rai KR, Kipps TJ (June 2008). "Guidelines for the diagnosis and treatment of chronic lymphocytic leukemia: a report from the International Workshop on Chronic Lymphocytic Leukemia updating the National Cancer Institute-Working Group 1996 guidelines". Blood. 111 (12): 5446–56. doi:10.1182/blood-2007-06-093906. PMC 2972576. PMID 18216293.
  6. Binet JL, Auquier A, Dighiero G, Chastang C, Piguet H, Goasguen J, Vaugier G, Potron G, Colona P, Oberling F, Thomas M, Tchernia G, Jacquillat C, Boivin P, Lesty C, Duault MT, Monconduit M, Belabbes S, Gremy F (July 1981). "A new prognostic classification of chronic lymphocytic leukemia derived from a multivariate survival analysis". Cancer. 48 (1): 198–206. PMID 7237385.
  7. MacDougall BK, Weinerman BH (1988). "Staging and prognosis of chronic lymphocytic leukemia: the Manitoba experience". J Gen Intern Med. 3 (2): 139–43. PMID 3357070.
  8. Zwiebel JA, Cheson BD (February 1998). "Chronic lymphocytic leukemia: staging and prognostic factors". Semin. Oncol. 25 (1): 42–59. PMID 9482526.
  9. Hallek M (September 2017). "Chronic lymphocytic leukemia: 2017 update on diagnosis, risk stratification, and treatment". Am. J. Hematol. 92 (9): 946–965. doi:10.1002/ajh.24826. PMID 28782884.



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