Actions

Extra: Difference between revisions

From haematologyetc.co.uk

No edit summary
No edit summary
 
(12 intermediate revisions by the same user not shown)
Line 5: Line 5:
:<span style="color:navy">'''Summary'''</span>
:<span style="color:navy">'''Summary'''</span>


:CD117 is a widely expressed molecule that in haematology is used most often as a marker of primitive myeloid cells where it contributes to a diagnosis of AML. However expression is absent in around 20-30% of typical AML and there is recognised aberrant expression by up to 30% of ALL cases (more frequently in T lineage). CD117 is also expressed by mast cells, and weakly on some plasma cells
: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)


   
   
Line 14: Line 14:
<span style="color:navy">'''Normal expression and function'''</span>
<span style="color:navy">'''Normal expression and function'''</span>


CD117 was originally described in 1987 as c-kit (the cellular homolog of the feline sarcoma viral oncogene v-kit). Stem cell factor (SCF) binds to CD117 initiating signals that variously control apoptosis, differentiation, proliferation, chemotaxis or cellular adhesion. In myeloid cells, CD117 is expressed by early haematopoietic progenitor cells then expression is lost as the cells mature.
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.




Line 21: Line 21:
<span style="color:navy">'''Diagnostic role'''</span>  
<span style="color:navy">'''Diagnostic role'''</span>  


*In acute myeloid leukaemia expression of CD117 is seen in 70-80% of cases including less differentiated forms, it may be expression by any subtype, but is less likely in cases with monocytic, erythroid, or megakaryocytic maturation
*Most often CD138 used in conjunction with CD38 to identify plasma cells in the diagnosis or monitoring of myeloma
*Aberrant expression in acute lymphoid leukaemias is reported - more frequently in T-lineage ALL (particularly more primitive types). Expression in B-lineage ALL is less frequent.
*There is very little aberrant expression, but neoplastic plasma cells may in some cases have weak, variable, or occasionally undetectable expression  
*Expression by mature B cell or mature T cell types is rare, although CD117 expression is described on some CD8 positive mature T cell neoplasms. *Around 30 of myeloma cases express CD117, but often expression is weak which limits its value in this disorder
*Expression is also seen cells that have plasmacytic differentiation in lymphoplasmacytoid lymphoma or marginal zone lymphoma 
*CD117 can be used to identify mast cells, but does not distinguish normal from neoplastic cells.




Line 30: Line 29:
<span style="color:navy">'''Other useful information'''</span>  
<span style="color:navy">'''Other useful information'''</span>  


Outside of the haematopoietic system CD117 has a wide expression that includes germ cells, cajal cells of the gastrointestinal tract and epithelial cells in skin and breast. Gain-of-function mutations are seen in tumours affecting mast cells, myeloid cells, systemic mastocytosis, acute myeloid leukemia or cajal cells gastrointestinal stromal tumors - GOST tumours.  
Outside of haematopoiesis CD138 is expressed by squamous epithelium.


----
----
Line 37: Line 36:




<span style="font-size:90%">'''Note''' The colour and % refer to the proportion of cases expressing the marker. Additional comments on strength, or site of expression (if relevant) are given below the table. For full details of key click [[Key to marker expression table|here]]. For further information about a particular disease state click <<span style="color:blue>'''I'''</span>> adjacent to the condition name</span>
{| class=wikitable style="text-align: center; font-size:80%; width: 87.5%; height 20px;"
 
 
{| class=wikitable style="text-align: center; font-size:80%; width: 100%; height 20px;"
|-
|-
!colspan="8"|<span style="font-size:100%">'''Expression in primitive cell types'''</font>
!colspan="7"|<span style="font-size:100%">'''Expression: primitive cell types''' ''Click (i) on table for additional information'' </span></font>
|-  
|-  
! AML !! CMML !! B ALL !! Burkitt || T ALL !! ETP ALL <'''[[ETP ALL|I]]'''> !! MPAL <'''[[MPAL|I]]'''> || Hgns <'''[[Hgns|I]]'''>
! 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:wblack"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:wblack"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:wblack"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:wblack"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:wblack"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:wblack"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:wblack"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:wblack"|<5%
|-
|-
|}
|}


<span style="font-size:90%">'''Notes:''' ''**''While a good marker of B lineage it may not be expressed in more primitive phenotype ALL, there is also a recognised abberrant expression in AML (particularly cases with t)8;21) </span>




{| class=wikitable style="text-align: center; font-size:80%; width: 100%; height 20px;"
{| class=wikitable style="text-align: center; font-size:80%; width: 100%; height 20px;"
|-
|-
!colspan="8"|'''Expression in mature B cell neoplasms'''
!colspan="8"|'''Expression: mature B cell neoplasms'''
|-  
|-  
! CLL !! MCL !!FL!! MZL !! HCL !! DLBCL || LPL !! PCL
! CLL !! MCL !!FL!! MZL !! HCL !! DLBCL || LPL !! PCL
|-
|-
|style="width: 12.5%; background: #E6FAFF; color:wblack"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:wblack"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:wblack"|<5%
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:wblack"|<5%
|style="width: 12.5%; background: #66e0ff; color:black"|5-20%
|style="width: 12.5%; background: #004466; color:white"|80-100%**
|style="width: 12.5%; background: #E6FAFF; color:black"|<5%
|style="width: 12.5%; background: #E6FAFF; color:wblack"|<5%
|style="width: 12.5%; background: #66e0ff; color:black"|5-20%
|style="width: 12.5%; background: #E6FAFF; color:wblack"|<5%
|style="width: 14.25%; background: #006699; color:white"|40-80%
|style="width: 12.5%; background: #E6FAFF; color:wblack"|<5%
|style="width: 12.5%; background: #004466; color:white"|80-100%
|-
|-
|}
|}


<span style="font-size:90%">'''Notes:''' *typically expressed in HCL where it will be detected in most cases; generally absent in other LPD although rarely expression is detected so the overall pattern is important </span>
<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>




Line 83: Line 77:
{| class=wikitable style="text-align: center; font-size:80%; width: 62.4%; height 40px;"
{| class=wikitable style="text-align: center; font-size:80%; width: 62.4%; height 40px;"
|-
|-
!colspan="7"|'''Expression in mature T cell neoplasms*'''
!colspan="7"|'''Expression: mature T cell neoplasms'''
|-  
|-  
! ATLL !! CTCL/Sezary !! T-PLL !! T-LGL !! NK-LGL
! ATLL !! CTCL Sezary !! T-PLL !! T-LGL !! NK-LGL
|-
|-
|style="width: 14.25%; background: #E6FAFF; color:black"|40%
|style= "width: 20%; background: #FFE4E1; color:black"|limited
|style="width: 14.25%; background: #E6FAFF; color:black"|<5%
|style="width: 20%; background: #FFE4E1; color:black"|limited
|style="width: 14.25%; background: #E6FAFF; color:black"|<5%
|style="width: 20%; background: #FFE4E1; color:black"|limited
|style="width: 14.25%; background: #E6FAFF; color:black"|<5%
|style= "width: 20%; background: #FFE4E1; color:black"|limited
|style="width: 14.25%; background: #E6FAFF; color:black"|<5%
|style="width: 20%; background: #FFE4E1; color:black"|limited
|-
|-
|}
|}


<span style="font-size:90%">'''Notes:''' Sporadic expression is recognised in many T-cell disorders at around 5% of cases, expression is more frequent in ATLL where up to 40% of cases may express CD103 </span>
<span style="font-size:90%">'''Notes:''' limited reports are consistent with expression being very uncommon in T-lymphoproliferative disorders</span>
 
----
----
summary tables 
----  summary tables 
2. expression by b-lymphoproliferative disorders file
rare  cll 
rare  pll 
rare  mcl 
rare  fl 
hi  hcl
hi  hclv 
rare  mzl 
rare  lpl 
rare  pcs 
link flow cytometry key  considered a strong indicator to distinguish hcl and hclv from mzl

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