Bailey.Glen at 21:32, 4 December 2023

2023-12-04T21:32:23Z

← Older revision		Revision as of 16:32, 4 December 2023
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	==Links==		==Links==

	[[Monoclonal B-cell ~~Lymphocytosis~~]]		[[HAEM5:Monoclonal B-cell lymphocytosis]]

	==References==		==References==

Bailey.Glen at 18:50, 3 November 2023

2023-11-03T18:50:02Z

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 {{Under Construction}}
 ==Primary Author(s)*==
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 <nowiki>*</nowiki>The hierarchical tumour classification structure displayed on this page is reproduced from the [https://tumourclassification.iarc.who.int/welcome/ WHO Classification of Tumours] with permission from the copyright holder, ©International Agency for Research on Cancer.

Bailey.Glen: Created page with "{{Under Construction}} ==Primary Author(s)*== Jamie Nagy, PhD, University of Iowa Honey Reddi, PhD TOC ==Cancer Category/Type== Mature B-cell neoplasms ==Cancer Sub-Cl..."

2023-11-03T18:10:31Z

Created page with "{{Under Construction}} ==Primary Author(s)*== Jamie Nagy, PhD, University of Iowa Honey Reddi, PhD __TOC__ ==Cancer Category/Type== Mature B-cell neoplasms ==Cancer Sub-Cl..."

New page

{{Under Construction}}
==Primary Author(s)*==
Jamie Nagy, PhD, University of Iowa

Honey Reddi, PhD
__TOC__

==Cancer Category/Type==

Mature B-cell neoplasms

==Cancer Sub-Classification / Subtype==

Put your text here

==Definition / Description of Disease==

This is a distinct entity in the 2016 World Health Organization (WHO) classification system<ref>Campo E, et al., (2017). Chronic lymphocytic leukemia/small lymphocytic lymphoma, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. Revised 4th Edition. IARC Press: Lyon, France, p216-221.</ref>. Chronic Lymphocytic Leukemia (CLL) is a chronic lymphoproliferative disorder characterized by monoclonal B cell proliferation.

'''CLL Tables''' - A list of clinically significant and/or recurrent CNAs and CN-LOH with potential or strong diagnostic, prognostic and treatment implications in CLL. Table derived from Chun et al., 2018 [<ref>{{Cite journal|last=K|first=Chun|last2=Gd|first2=Wenger|last3=A|first3=Chaubey|last4=Dp|first4=Dash|last5=R|first5=Kanagal-Shamanna|last6=S|first6=Kantarci|last7=R|first7=Kolhe|last8=Dl|first8=Van Dyke|last9=L|first9=Wang|date=2018|title=Assessing copy number aberrations and copy-neutral loss-of-heterozygosity across the genome as best practice: An evidence-based review from the Cancer Genomics Consortium (CGC) working group for chronic lymphocytic leukemia|url=https://pubmed.ncbi.nlm.nih.gov/30554732/|language=en|pmid=30554732}}</ref>] with permission from Cancer Genetics. See [[CLL Tables: Regions of Recurrent Copy Number Change and CN-LOH]].

==Synonyms / Terminology==
Chronic lymphocytic leukaemia, B―cell type; chronic lymphoid leukaemia; chronic lymphatic leukaemia

==Epidemiology / Prevalence==

It is the most common adult leukemia in Western populations, comprising 25% to 30% of all leukemias in the United States. The median age at diagnosis is 70 years. CLL occurrence is more prevalent in anglo americans and much lower in asian populations<ref name=":0">Taneja A, Master SR. (2017) Cancer, Leukemia, Lymphocytic, Chronic (CLL) SourceStatPearls [I. Treasure Island (FL): StatPearls Publishing. <nowiki>https://www.ncbi.nlm.nih.gov/books/NBK470433/</nowiki>.</ref>.

==Clinical Features==

Most (90%) patients with CLL are asymptomatic and only 5-10% of patients with CLL present with symptoms of fever, weight loss, night sweats, and/or fatigue<ref name=":0" />.

==Sites of Involvement==
CLL/SLL involves the blood, bone marrow, and secondary lymphoid tissues such as the spleen, lymph nodes, and Waldeyer ring. Extranodal involvement

(e.g. of the skin, gastrointestinal tract, or CNS) occurs in a small subset of cases<ref>{{Cite journal|last=M|first=Ratterman|last2=K|first2=Kruczek|last3=S|first3=Sulo|last4=Td|first4=Shanafelt|last5=Ne|first5=Kay|last6=C|first6=Nabhan|date=2014|title=Extramedullary chronic lymphocytic leukemia: systematic analysis of cases reported between 1975 and 2012|url=https://pubmed.ncbi.nlm.nih.gov/24064196/|language=en|pmid=24064196}}</ref>.

==Morphologic Features==

'''Lymph Nodes:''' Enlarged lymph nodes show diffuse architectural effacement by a proliferation of small lymphocytes with variably prominent scattered paler proliferation centers (pseudofollicles)<ref>Lennert K, editor. (1978). Malignant lymphomas other than Hodgkin’s disease. NewYork: Springer Verlag.</ref>. The predominant cell in the diffuse areas is a '''''typical CLL cell''''' (small lymphocyte with scant cytoplasm, and clumped chromatin). Proliferation centers are composed of small lymphocytes, prolymphocytes, and paraimmunoblasts. Mitotic activity is usually very low.

'''Bone Marrow:''' Biopsy may show interstitial, nodular, mixed (nodular and interstitial), or diffuse involvement. Diffuse involvement is usually associated with more advanced disease<ref>{{Cite journal|last=E|first=Montserrat|last2=N|first2=Villamor|last3=Jc|first3=Reverter|last4=Rm|first4=Brugués|last5=D|first5=Tàssies|last6=F|first6=Bosch|last7=Jl|first7=Aguilar|last8=Jl|first8=Vives-Corrons|last9=M|first9=Rozman|date=1996|title=Bone marrow assessment in B-cell chronic lymphocytic leukaemia: aspirate or biopsy? A comparative study in 258 patients|url=https://pubmed.ncbi.nlm.nih.gov/8611442/|language=en|pmid=8611442}}</ref>. Paratrabecular aggregates are not typical. Proliferation centers can be observed, although they are not as prominent as in lymph nodes, and follicular dendritic cells may be present<ref>{{Cite journal|last=M|first=Chilosi|last2=G|first2=Pizzolo|last3=F|first3=Caligaris-Cappio|last4=A|first4=Ambrosetti|last5=F|first5=Vinante|last6=L|first6=Morittu|last7=F|first7=Bonetti|last8=L|first8=Fiore-Donati|last9=G|first9=Janossy|date=1985|title=Immunohistochemical demonstration of follicular dendritic cells in bone marrow involvement of B-cell chronic lymphocytic leukemia|url=https://pubmed.ncbi.nlm.nih.gov/3891066/|language=en|pmid=3891066}}</ref>. Most cases have > 30% CLL cells in the bone marrow aspirate<ref>{{Cite journal|last=M|first=Hallek|last2=Bd|first2=Cheson|last3=D|first3=Catovsky|last4=F|first4=Caligaris-Cappio|last5=G|first5=Dighiero|last6=H|first6=Döhner|last7=P|first7=Hillmen|last8=Mj|first8=Keating|last9=E|first9=Montserrat|date=2008|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|url=https://pubmed.ncbi.nlm.nih.gov/18216293/|language=en|doi=10.1182/blood-2007-06-093906|pmc=PMC2972576|pmid=18216293}}</ref>.

'''Peripheral Blood''': Smudge or basket cells are typically observed. In most cases, besides typical CLL cells, other lymphoid cells like prolymphocytes are also observed, but they usually constitute < 15% of the lymphoid cells.

==Immunophenotype==
Circulating leukemic B cells express CD19, low surface IgM/lgD, CD20, CD22, and CD79b. Additionally the markers below may be strongly expressed or absent.
{| class="wikitable sortable"
|-
!Finding!!Marker
|-
|Positive (universal)||CD5, CD43 and strongly positive

for CD23 and CD200
|-
|Negative (universal)||CD10 is negative
FMC7 is usually negative or

only weakly expressed.
|-
|Subset||CD5― or CD23―, FMC7+,

strong surface immunoglobulin, or

CD79b+<ref>{{Cite journal|last=A|first=Criel|last2=L|first2=Michaux|last3=C|first3=De Wolf-Peeters|date=1999|title=The concept of typical and atypical chronic lymphocytic leukaemia|url=https://pubmed.ncbi.nlm.nih.gov/10194119/|language=en|pmid=10194119}}</ref>
|}

==Chromosomal Rearrangements (Gene Fusions)==

Put your text here and/or fill in the table

{| class="wikitable sortable"
|-
!Chromosomal Rearrangement!!Genes in Fusion (5’ or 3’ Segments)!!Pathogenic Derivative!!Prevalence
|-
|EXAMPLE t(9;22)(q34;q11.2)||EXAMPLE 3'ABL1 / 5'BCR||EXAMPLE der(22)||EXAMPLE 5%
|-
|EXAMPLE t(8;21)(q22;q22)||EXAMPLE 5'RUNX1 / 3'RUNXT1||EXAMPLE der(8)||EXAMPLE 5%
|}

==Characteristic Chromosomal Aberrations / Patterns==

Put your text here

==Genomic Gain/Loss/LOH==

{| class="wikitable sortable"
|-
!Chromosome Number!!Gain/Loss/Amp/LOH!!Region
|-
|EXAMPLE 8||EXAMPLE Gain||EXAMPLE chr8:0-1000000
|-
|EXAMPLE 7||EXAMPLE Loss||EXAMPLE chr7:0-1000000
|}

Put your text here and/or fill in the table

'''CLL Tables''' - A list of clinically significant and/or recurrent CNAs and CN-LOH with potential or strong diagnostic, prognostic and treatment implications in CLL. Table derived from Chun et al., 2018 [<nowiki>PMID 30554732</nowiki>] with permission from Cancer Genetics. See [[CLL Tables: Regions of Recurrent Copy Number Change and CN-LOH]].

==Gene Mutations (SNV/INDEL)==

Prevalence (COSMIC, May 2018)
NOTCH1 12%
SF3B1 12%
ATM 12%
TP53 11%
NFKBIE 4%
XPO1 4%
BTK 4%
MED12 3%
BIRC3 3%
MYD88 3%
LRP1b 3%
POT1 3%
BRAF 2%
FAT1 2%

{| class="wikitable sortable"
|-
!Gene!!Mutation!!Oncogene/Tumor Suppressor/Other!!Presumed Mechanism (LOF/GOF/Other; Driver/Passenger)!!Prevalence (COSMIC/TCGA/Other)
|-
|EXAMPLE TP53||EXAMPLE R273H||EXAMPLE Tumor Suppressor||EXAMPLE LOF||EXAMPLE 20%
|}

===Other Mutations===
{| class="wikitable sortable"
|-
!Type!!Gene/Region/Other
|-
|Concomitant Mutations||EXAMPLE IDH1 R123H
|-
|Secondary Mutations||EXAMPLE Trisomy 7
|-
|Mutually Exclusive||EXAMPLE EGFR Amplification
|}

==Epigenomics (Methylation)==

Put your text here

==Genes and Main Pathways Involved==

Put your text here

==Diagnostic Testing Methods==

Put your text here

==Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications)==

Put your text here

==Familial Forms==

Put your text here

==Other Information==

Put your text here

==Links==

[[Monoclonal B-cell Lymphocytosis]]

==References==
<references />

==Notes==
<nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage). Additional global feedback or concerns are also welcome.

<nowiki>*</nowiki>The hierarchical tumour classification structure displayed on this page is reproduced from the [https://tumourclassification.iarc.who.int/welcome/ WHO Classification of Tumours] with permission from the copyright holder, ©International Agency for Research on Cancer.

HAEM4:Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma - Revision history

Bailey.Glen at 21:32, 4 December 2023

Bailey.Glen at 18:50, 3 November 2023

Bailey.Glen: Created page with "{{Under Construction}} ==Primary Author(s)*== Jamie Nagy, PhD, University of Iowa Honey Reddi, PhD __TOC__ ==Cancer Category/Type== Mature B-cell neoplasms ==Cancer Sub-Cl..."

Bailey.Glen: Created page with "{{Under Construction}} ==Primary Author(s)*== Jamie Nagy, PhD, University of Iowa Honey Reddi, PhD TOC ==Cancer Category/Type== Mature B-cell neoplasms ==Cancer Sub-Cl..."