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Immunoglobulin light chains


Lght chains form part of the b-cell receptor and may therefore be considered as a pan b-cell marker from late lymphoblasts until plasma cell differentiation. If abnormal cells express predomnantly kappa or lambda then clonality can be inferred. Absence of light chain is also abnormal and may incicate clonality.
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normal function and expression 


The antigen-recognising structure of the B-cell receptor is the immunoglobulin molecule formed from either kappa or lambda light chains in combination with immunoglobulin heavy chains. Normal mature b cells therefore express surface light chains as part of their B-cell receptor. During pro-B to pre-B cell development immunoglobulin genes rearrange to form unique structures Kappa or lambda light chains appear on the surface of immature B cells at later stages of development remaining expressed until plasma cell development when immunoglobulin is secreted so expression is within cytoplasmic secretory vesicles. 
:<span style="color:navy">'''Summary'''</span>


what is its diagnostic role 
:CD138 is a marker used to identify normal or malignant plasma cells with a high level of specificity, but does not discriminate normal from abnormal plasma cells and in some cases expression in myeloma may be low compared with normal plasma cells so sensitivity can be an issue. Abberancy of expression is low, but expression may be seen in lymphoplamacytic or marginal zone lymphomas (only on those cells that have plasma cell differentiation)
*acquisition or loss of surface light-chain expression acquisition of surface light chains does not occur until late in b-precursor cell development so may be used to indicate developmental stage. Loss of surface expression indicates plasma cell differentiation.
*clonality malignant b-cells arise from a single parent cell so express light chain of a single type either kappa or lambda - light chain restriction . in almost all cases there will be some normal cells present so light chains usually are a mixture of kappa and lambda. however when there is a malignant clone either kappa or lambda begins to predominate. Studies most frequently suggest that if the kappa to lambda ratio is 3 1 or 3 1 then the presence of a clonal population can be inferred although correlation with other markers is needed.
*weak or absent light chain expression may be seen in DLBCL FL or CLL and more rarely in MCL or MZL. Overall around 10% of B-lymphoproliferative disorders do not express surface light chain.


summary tables  
   
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1. expression by acute leukaemias and by 
rare pro-b all 
poss  early pre-b all 
poss  pre-b all 
hi  b all
rare  pro-t all 
rare  mature-t all
rare  aml 
poss  haemato-gones 
*  expressed by late haematogones


2. expression by b-lymphoproliferative disorders 
 
hi cll
<span style="color:navy">'''Normal expression and function'''</span>
hi  pll**   
 
hi  mcl  
CD138 is a membrane protein that supports cell adhesion or binds cytokines and chemokines. In the hematopoietic system expression of CD138 is acquired by B lymphocytes as they develop into plasma cells.
hi  fl 
 
hi  hcl** 
 
hi  hclv  l
 
hi  mzl 
 
hi  lpl 
<span style="color:navy">'''Diagnostic role'''</span>
poss  pcs
 
*Most often CD138 used in conjunction with CD38 to identify plasma cells in the diagnosis or monitoring of myeloma
*There is very little aberrant expression, but neoplastic plasma cells may in some cases have weak, variable, or occasionally undetectable expression
*Expression is also seen cells that have plasmacytic differentiation in lymphoplasmacytoid lymphoma or marginal zone lymphoma  
 
 
 
<span style="color:navy">'''Other useful information'''</span>
 
Outside of haematopoiesis CD138 is expressed by squamous epithelium.
 
----
 
<span style="color:navy">'''''SUMMARY TABLES'''''</span>
 
 
{| class=wikitable style="text-align: center; font-size:80%; width: 87.5%; height 20px;"
|-
!colspan="7"|<span style="font-size:100%">'''Expression: primitive cell types''' ''Click (i) on table for additional information'' </span></font>
|-
! AML !! B ALL !! Burkitt || T ALL !! ETP ALL ('''[[Early T Precursor ALL (ETP-ALL)|i]]''') !! MPAL ('''[[Mixed Phenotype Acute Leukaemia (MPAL)|i]]) || H.gones ('''[[Haematogones|i]]''')
|-
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|-
|}
 
 
 
{| class=wikitable style="text-align: center; font-size:80%; width: 100%; height 20px;"
|-
!colspan="8"|'''Expression: mature B cell neoplasms'''
|-
! CLL !! MCL !!FL!! MZL !! HCL !! DLBCL || LPL !! PCL
|-
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #66e0ff; color:black"|5-20%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #66e0ff; color:black"|5-20%
|style="width: 14.25%; background: #006699; color:white"|40-80%
|style="width: 12.5%; background: #004466; color:white"|80-100%
|-
|}
 
<span style="font-size:90%">'''Notes:''' expression is usual in myeloma, and will occur on cells with plasma cell differentiation in LPL or MZL</span>
 
 
 
{| class=wikitable style="text-align: center; font-size:80%; width: 62.4%; height 40px;"
|-
!colspan="7"|'''Expression: mature T cell neoplasms'''
|-
! ATLL !! CTCL Sezary !! T-PLL !! T-LGL !! NK-LGL
|-
|style= "width: 20%; background: #FFE4E1; color:black"|limited
|style="width: 20%; background: #FFE4E1; color:black"|limited
|style="width: 20%; background: #FFE4E1; color:black"|limited
|style= "width: 20%; background: #FFE4E1; color:black"|limited
|style="width: 20%; background: #FFE4E1; color:black"|limited
|-
|}
 
<span style="font-size:90%">'''Notes:''' limited reports are consistent with expression being very uncommon in T-lymphoproliferative disorders</span>
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Latest revision as of 20:53, 13 June 2023


Summary
CD138 is a marker used to identify normal or malignant plasma cells with a high level of specificity, but does not discriminate normal from abnormal plasma cells and in some cases expression in myeloma may be low compared with normal plasma cells so sensitivity can be an issue. Abberancy of expression is low, but expression may be seen in lymphoplamacytic or marginal zone lymphomas (only on those cells that have plasma cell differentiation)



Normal expression and function

CD138 is a membrane protein that supports cell adhesion or binds cytokines and chemokines. In the hematopoietic system expression of CD138 is acquired by B lymphocytes as they develop into plasma cells.



Diagnostic role

  • Most often CD138 used in conjunction with CD38 to identify plasma cells in the diagnosis or monitoring of myeloma
  • There is very little aberrant expression, but neoplastic plasma cells may in some cases have weak, variable, or occasionally undetectable expression
  • Expression is also seen cells that have plasmacytic differentiation in lymphoplasmacytoid lymphoma or marginal zone lymphoma


Other useful information

Outside of haematopoiesis CD138 is expressed by squamous epithelium.


SUMMARY TABLES


Expression: primitive cell types Click (i) on table for additional information
AML B ALL Burkitt T ALL ETP ALL (i) MPAL (i) H.gones (i)
<5% <5% <5% <5% <5% <5% <5%


Expression: mature B cell neoplasms
CLL MCL FL MZL HCL DLBCL LPL PCL
<5% <5% <5% 5-20% <5% 5-20% 40-80% 80-100%

Notes: expression is usual in myeloma, and will occur on cells with plasma cell differentiation in LPL or MZL


Expression: mature T cell neoplasms
ATLL CTCL Sezary T-PLL T-LGL NK-LGL
limited limited limited limited limited

Notes: limited reports are consistent with expression being very uncommon in T-lymphoproliferative disorders