HAEM5:Primary cutaneous marginal zone lymphoma: Difference between revisions

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!Finding!!Marker
!Finding!!Marker
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|Positive
|CD20
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|Positive
|CD22
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|Positive
|CD79a
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|Positive||BCL2
|Positive||BCL2
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*The reactive germinal centers B cells are BCL6 positive and BCL2 negative.
* Associated reactive germinal centers B cells  
*CD123 positive plasmacytoid dendritic cells.
** BCL6 positive  
*Plasma cells have monotypic expression of immunoglobulin light chains often. Heavy chain class-switched form IgG, IgA, or IgE and have no expression of CXCR3. If non-class-switched forms are present, IgM and CXCR3 are expressed.
** BCL2 negative
**Approximately 90% of cases have IgG, IgA, or IgE positivity
*Plasmacytoid dendritic cells
**Approximately 10% of cases have IgM positivity
**CD123 positive
*IgG4 expressed by plasma cells in 13-35% of cases, though not related to IgG4-related disease.
*Plasma cells  
**CD138 and CD79a positive
**Plasma cells often have monotypic expression of immunoglobulin light chains  
**The immunoglobulin heavy chain can be either non-class-switched or class-switched
***Non-class-switched heavy chain
****Produce IgM
****Positive for CXCR3 expression
****Approximately 10% of cases have IgM positivity
***Class-switched heavy chain
****Produce IgG, IgA, or IgE  
****No expression of CXCR3
****Approximately 90% of cases have IgG, IgA, or IgE positivity
**IgG4 expressed by plasma cells in 13-35% of cases, though not related to IgG4-related disease.


==Chromosomal Rearrangements (Gene Fusions)==
==Chromosomal Rearrangements (Gene Fusions)==
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==Additional Information==
==Additional Information==


* Favorable prognosis
*Favorable prognosis
** 5-year disease-specific survival rate >98%
**5-year disease-specific survival rate >98%
* Recurrence is common
*Recurrence is common
* 4% of patients will have extracutaneous spread, particularly in patients with longstanding multifocal disease
*4% of patients will have extracutaneous spread, particularly in patients with longstanding multifocal disease


==Links==
==Links==