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{{Chronic lymphocytic leukemia}}
{{Chronic lymphocytic leukemia}}
{{CMG}} {{AE}}{{HL}}
==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.


{{CMG}}; {{AE}} {{RT}}
==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, [[platelet]]s level, presence of [[splenomegaly]], and presence of [[lymphadenopathy]].<ref name="pmid11390392">{{cite journal |vauthors=Rai KR, Sawitsky A, Cronkite EP, Chanana AD, Levy RN, Pasternack BS |title=Clinical staging of chronic lymphocytic leukemia |journal=Blood |volume=46 |issue=2 |pages=219–34 |date=August 1975 |pmid=1139039 |doi= |url=}}</ref><ref name="pmid3357070">{{cite journal |vauthors=MacDougall BK, Weinerman BH |title=Staging and prognosis of chronic lymphocytic leukemia: the Manitoba experience |journal=J Gen Intern Med |volume=3 |issue=2 |pages=139–43 |date=1988 |pmid=3357070 |doi= |url=}}</ref><ref name="pmid9482526">{{cite journal |vauthors=Zwiebel JA, Cheson BD |title=Chronic lymphocytic leukemia: staging and prognostic factors |journal=Semin. Oncol. |volume=25 |issue=1 |pages=42–59 |date=February 1998 |pmid=9482526 |doi= |url=}}</ref><ref name="pmid28782884">{{cite journal |vauthors=Hallek M |title=Chronic lymphocytic leukemia: 2017 update on diagnosis, risk stratification, and treatment |journal=Am. J. Hematol. |volume=92 |issue=9 |pages=946–965 |date=September 2017 |pmid=28782884 |doi=10.1002/ajh.24826 |url=}}</ref>


==Overview==
{| style="border: 0px; font-size: 90%; margin: 3px; width: 1050px"
| valign="top" |
|+
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|'''Stage'''}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|'''Hemoglobin Level'''}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|'''Platelets Level'''}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|'''Lymphocytosis'''}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|'''Splenomegaly'''}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|'''Lymphadenopathy'''}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|'''Median Survival'''}}


==Classification==
|-
===Clinical staging===
Staging is done with the Rai staging system and the Binet classification (see details<ref name="NCI-CLL-page2">{{cite web |url=http://www.cancer.gov/cancertopics/pdq/treatment/CLL/HealthProfessional/page2 |title=Chronic Lymphocytic Leukemia (PDQ®) Treatment: Stage Information |author=National Cancer Institute |accessdate=2007-09-04 |format= |work=}}</ref>).


==== '''Rai staging system'''====
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |
'''Stage 0'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
>11 g/dL
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
>100,000/L
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
'''Present'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
Absent
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
Absent
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
>150 months


{|style="width:80%; height:110px" border="1"
|style="height:100px"; style="width:25%" border="1" bgcolor="LightSteelBlue" | '''Stage 0'''
|style="height:100px"; style="width:75%" border="1" bgcolor="Beige" | Absolute lymphocytosis (>15,000/mm3) without adenopathy, hepatosplenomegaly, anemia, or thrombocytopenia.
|-
|-
|-bgcolor="LightSteelBlue"
 
| '''Stage 1'''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |
|bgcolor="Beige"| Absolute lymphocytosis with lymphadenopathy without hepatosplenomegaly, anemia, or thrombocytopenia.
'''Stage 1'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
>11 g/dL
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
>100,000/L
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
'''Present'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
Absent
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
'''Present'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
101 months
 
|-
|-
|-bgcolor="LightSteelBlue"
 
| '''Stage 2'''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |
|bgcolor="Beige"| Absolute lymphocytosis with either hepatomegaly or splenomegaly with or without lymphadenopathy.
'''Stage 2'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
>11 g/dL
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
>100,000/L
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
'''Present'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
'''Present'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
Present or absent
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
71 months
 
|-
|-
|-bgcolor="LightSteelBlue"
 
| '''Stage 3'''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |
|bgcolor="Beige"| Absolute lymphocytosis and anemia (hemoglobin <11 g/dL) with or without lymphadenopathy, hepatomegaly, or splenomegaly.
'''Stage 3'''
|-
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
|-bgcolor="LightSteelBlue"
'''<11''' g/dL
| '''Stage 4'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
|bgcolor="Beige"| Absolute lymphocytosis and thrombocytopenia (<100,000/mm3) with or without lymphadenopathy, hepatomegaly, splenomegaly, or anemia.
>100,000/L
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
'''Present'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
Present or absent
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
Present or absent
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
19 months
 
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |
'''Stage 4'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
<11 g/dL '''or''' >11g/dL
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
'''<100,000'''/L
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
'''Present'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
Present or absent
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
Present or absent
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
19 months
|}
|}


===='''Binet Classification'''====
The staging is further modified into 3 groups:<ref name="pmid18216293">{{cite journal |vauthors=Hallek M, Cheson BD, Catovsky D, Caligaris-Cappio F, Dighiero G, Döhner H, Hillmen P, Keating MJ, Montserrat E, Rai KR, Kipps TJ |title=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 |journal=Blood |volume=111 |issue=12 |pages=5446–56 |date=June 2008 |pmid=18216293 |pmc=2972576 |doi=10.1182/blood-2007-06-093906 |url=}}</ref>
* '''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.<ref name="pmid7237385">{{cite journal |vauthors=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 |title=A new prognostic classification of chronic lymphocytic leukemia derived from a multivariate survival analysis |journal=Cancer |volume=48 |issue=1 |pages=198–206 |date=July 1981 |pmid=7237385 |doi= |url=}}</ref><ref name="pmid33570702">{{cite journal |vauthors=MacDougall BK, Weinerman BH |title=Staging and prognosis of chronic lymphocytic leukemia: the Manitoba experience |journal=J Gen Intern Med |volume=3 |issue=2 |pages=139–43 |date=1988 |pmid=3357070 |doi= |url=}}</ref><ref name="pmid94825262">{{cite journal |vauthors=Zwiebel JA, Cheson BD |title=Chronic lymphocytic leukemia: staging and prognostic factors |journal=Semin. Oncol. |volume=25 |issue=1 |pages=42–59 |date=February 1998 |pmid=9482526 |doi= |url=}}</ref><ref name="pmid287828842">{{cite journal |vauthors=Hallek M |title=Chronic lymphocytic leukemia: 2017 update on diagnosis, risk stratification, and treatment |journal=Am. J. Hematol. |volume=92 |issue=9 |pages=946–965 |date=September 2017 |pmid=28782884 |doi=10.1002/ajh.24826 |url=}}</ref>
 
{| style="border: 0px; font-size: 90%; margin: 3px; width: 1050px"
| valign="top" |
|+
! style="background: #4479BA; width: 250px;" | {{fontcolor|#FFF|'''Stage'''}}
! style="background: #4479BA; width: 250px;" | {{fontcolor|#FFF|'''Lymphocytosis'''}}
! style="background: #4479BA; width: 500px;" | {{fontcolor|#FFF|'''Anemia or Thrombocytopenia'''}}
! style="background: #4479BA; width: 250px;" | {{fontcolor|#FFF|'''Lymphadenopathy'''}}
! style="background: #4479BA; width: 700px;" | {{fontcolor|#FFF|'''Median Survival'''}}


{|style="width:80%; height:100px" border="1"
|style="height:100px"; style="width:25%" border="1" bgcolor="LightSteelBlue" | '''Clinical stage A'''
|style="height:100px"; style="width:75%" border="1" bgcolor="Beige" | No anemia or thrombocytopenia and fewer than three areas of lymphoid involvement (Rai stages 0, I, and II).
|-
|-
|-bgcolor="LightSteelBlue"
 
| '''Clinical stage B'''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |
|bgcolor="Beige"| No anemia or thrombocytopenia with three or more areas of lymphoid involvement (Rai stages I and II).
'''Stage A'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
Present
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
Absent
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
2 or less lymphoid regions
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
Almost normal age and sex adjusted survival rate
|-
|-
|-bgcolor="LightSteelBlue"
 
| '''Clinical stage C'''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |
|bgcolor="Beige"| Anemia and/or thrombocytopenia regardless of the number of areas of lymphoid enlargement (Rai stages III and IV).
'''Stage B'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
Present
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
Absent
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
>3 lymphoid regions
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
7 years
 
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |
'''Stage C'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
Present
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
Present
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
>3 lymphoid regions
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
2 years
|}
|}


The '''Binet classification''' integrates the number of nodal groups involved with the disease with bone marrow failure. Its major benefit derives from the recognition of a predominantly splenic form of the disease, which may have a better prognosis than in the '''Rai staging''', and from recognition that the presence of anemia or thrombocytopenia has a similar prognosis and does not merit a separate stage. Neither system separates immune from nonimmune causes of cytopenia. Patients with thrombocytopenia or anemia or both, which is caused by extensive marrow infiltration and impaired production (Rai III/IV, Binet C) have a poorer prognosis than patients with immune cytopenias.[4] The International Workshop on CLL has recommended integrating the Rai and Binet systems as follows: A(0), A(I), A(II); B(I), B(II); and C(III), C(IV).
== References ==
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Latest revision as of 15:54, 28 February 2019

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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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