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Secondary skin involvement by CLL, in progression, probably EBV-associated. (5438)
Secondary skin involvement by CLL, in progression, probably EBV-associated.closed
Subtitle: B08-9419
Type:
skin
Sender:
ugnius
2008-04-11 11:24
INCTR - EBMWG Hematopathology Online
80 yrs woman was biopsied and recently operated due to tumor inthe sacral skin.  
HISTO: B marginal zone lymphoma with plasmoid features.  
IH B POPULATION: CD20(++) 100%, Ki67 low 30% (++), CD23(++) 40% (large cells), CD43(+++) 100%, Bcl6(+/++) 10% (rudimentary FC?), CD10(-), CD5(+/++) 40%, IgM(+++) 100%, IgD(+++) 90%, Mum1(-)(plasmacells +), CyclinD1(-). CD21+ FDC network absent (only rudimentary nests in ocupated follicles). Multiple CD30(+) large immunoblasts 10%, CD15-. Single EBV LPM1(++)cells. Ig kappa/lambda without restriction.  
 
PROPOSAL: B marginal zone lymphoma in the skin with aberant immunophenotype CD23?  
FINAL: CLL CD23+CD5-/+ infiltration in the skin (in ulcer/lymphoid hyperplasia background)  
SPECIAL QUEST: CD30+ large immunoblasts (reactive), single EBV+ cells, CD23+ expression?  
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ugnius
2008-04-11 11:25
Please, inform me, if accessibility of virtual slide is problematic. Max resolution 20x. Thank you for patience. "Ordinary" slides will be apended soon.
Mueller-Hermelink
2008-04-11 13:18
I only could get access to the virtual slide ( but not to the immunostains) from the description I do not understand whether secretory plasma cells are polytypic or Lambda restricted. The morphology could fitto marginal zone lymphoma - but in view of the reported phenotype CLL has to be excluded which can present in a marginalzone pattern. EBV positive blast cells could also be interpreted as a sign of progression in that case.Could you add the CD 23 immunostain ?
ugnius
2008-04-11 13:52
Please find some IH (loading). Appologies for copying/mistyping/translating (from local report in lithuanian) mistakes for Ig kappa/lambda. If virtual slide would work properly we will try to append some to every case. Thank you.
Mueller-Hermelink
2008-04-13 12:02
As far as I see the plasma cells are polytypic . On the other hand the infiltrate and CD43 positivity speaks in favor of lymphoma . Without doubt there is a strong inflammatory, reactive background. I cannot decide whether this is marginal zone B cell lymphoma of the skin or CLL (co-) infiltrating a pseudotumorous inflammatory reaction. I believe we need some further clinical information ( old decubital ulcer ? PBL? etc.)
schulze
2008-04-14 20:32
I fully agree with the comments of Prof. Müller-Hermelink. Because of the "multifocal" distribution of the dermal infiltrate I favor secondary skin involvement of B cell lymphoma.
ugnius
2008-04-15 14:54
RETROSPECTIVE info from regional hospital (not included in previous consult material): CLL history for 4 yrs clinically (based lymphocytosis in myelogramm and blood). The patient rejected threpine biopsy. Persisting peripheral lymphadenopathy. 1 year before Leuceran based therapy was applied.  
The decubital? nonhealing "ulcer" in the buttock skin with "cauliflower shaped" tumor. The patient reject further chemotherapy.  
The skin lession was resected in gen surg dept, which is not familiar with hemato diagnoses...  
Appologies for incomplete information.  
Thank you for comments, targeting on CLL CD23+ in reactive lession.
ugnius
2008-04-15 18:05
Please find PREVIOUS BIOPSY from the same place FOLDER with similar infiltrates, reach in large immunoblasts CD30+CD15-. Some EBV LMP1+. The caliber of CD23+ CD20+ and fiant CD5-/+ cells rae medium. Without resection material the interpretation would ber aggravated. Thank you for your comments.
hurwitz
2008-04-16 19:09
1.Access to the virtual slide. I tried but at the moment we are staing in a rural area, where the internet intensity is varying, Today it is low so it took ages to open and I finally gave-up.  
2.Thanks for the additional clinical information about preexisting CLL. Now it the case is clear, and the progression, as Konrad pointed out is probably EBV associated.
ugnius
2008-04-17 12:37
The one question left: what do you mean "progression"- we have not solid DLBCL quality for Richter's interpretation? How it must be formulated? Thanx.
hurwitz
2008-04-17 13:34
Progression is a continuous process of transformation of a low grade lymphoma to high grade lymphoma, which finally leads to full blown high grade lymphoma.  
I agree that this case still does not fulfill the criteria of DLBCL.
hurwitz
2008-04-17 13:45
Final diagnosis  
Secondary skin involvement by systemic lymphoproliferative disorder, CLL (known since 4 years). Evidence for large cell transformation, possibly EBV associated.  
 
Thanks to the submittor and for the discussion
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Last modified: 2008-04-11 11:24:02