Chronic lymphocytic leukemia clinical staging

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

↑ 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.
↑ 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.
↑ Zwiebel JA, Cheson BD (February 1998). “Chronic lymphocytic leukemia: staging and prognostic factors”. Semin. Oncol. 25 (1): 42–59. PMID 9482526.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ Zwiebel JA, Cheson BD (February 1998). “Chronic lymphocytic leukemia: staging and prognostic factors”. Semin. Oncol. 25 (1): 42–59. PMID 9482526.
↑ 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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Template:WikiDoc Sources

[pmid11390392-1] 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.

[pmid3357070-2] 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.

[pmid9482526-3] Zwiebel JA, Cheson BD (February 1998). “Chronic lymphocytic leukemia: staging and prognostic factors”. Semin. Oncol. 25 (1): 42–59. PMID 9482526.

[pmid28782884-4] 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.

[pmid18216293-5] 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.

[pmid7237385-6] 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.

[pmid33570702-7] 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.

[pmid94825262-8] Zwiebel JA, Cheson BD (February 1998). “Chronic lymphocytic leukemia: staging and prognostic factors”. Semin. Oncol. 25 (1): 42–59. PMID 9482526.

[pmid287828842-9] 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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