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CLL with plasmoid features, biclone (4874)
CLL with plasmoid features, biclonenew
Subtitle: B08-1479
Type:
HEMATO
Sender:
ugnius
2008-02-27 08:26
INCTR - EBMWG Hematopathology Online
76 yrs woman with clinical diagnosis of B CLL underwent threpine biopsy.  
HISTO/IH: Interstitial and slightly nodular CD20+ CD79a+ CD5+ CD23+ CD43+ IgM+ IgD+ infiltration of small lymphos; More peripheral infiltration of "plasmacytoid" cells with Ig (Dutcher) inclussions CD20- CD79a- Pax-5- CD138+ IgM+ > IgD+.  
HISTORY/CLINICS: 1. Lymphocytosis in peripheral blood 94.8% and bone marrow 72%; Hb in PB 93.4; 2. PLasmocytes in BM 3.5%; 3. Hepatomegaly (sono). IG studies not done up to date (will be cont'd).  
 
INTERESTING FEATURES: 1. Ig lambda restriction on small B cells; Ig kappa restriction on the "plasmoid" cells. 2. CyclinD1+ 100% in"plasmoid" population. 3. FLOW: CD5+ CD23+ FMC7+ CLL population with Ig kappa restriction (12/1);  
 
PROPOSALS: 1. Biclone CLL. 2. CLL + myeloma.  
 
Thank you for collaboration.
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tzankov
2008-02-27 10:10
recently, I had two analogous cases, concurrent CLL and plasma cell neoplasms, the latter being cyclin D1+ (as well as MUM1+, PAX5-, CD138+). I contacted the clinical colleagues to look for M-gradients and both patients had paraproteins.  
 
in your case there is a clear cut CLL and an additional plasmacytic/plasmocytoid neoplasm positive for cyclin D1. I would suggest to ask your clinical colleagues for other signs of concurrent plasma cell dyscrasia (M-grad., osteolyses etc.). nevertheless I consider the case to represent coincidental CLL and myeloma.
Mueller-Hermelink
2008-02-28 18:47
I completely agree with Dr. Tzankov. We have the same experience of concordant BCLL and Multiple Myeloma in BM trephines ( not all are IgM+, CyclinD1+ ). We also consider these cases as composite and not clonally related.
ugnius
2008-02-28 19:12
Thank you for support. What molecular test would be more efficient for confirming 2 clones?
tzankov
2008-02-28 20:47
would be there a practical consequence wheather or not the two entities are clonally related? in this case no...  
the demonstration of two different light chain restrictions is enough to call both neoplasms composite/not clonally related.
hurwitz
2008-03-03 19:56
I agree with the comments of Dr.Tzankov and Müller-Hermelink that these are two clonally unrelated neoplasms as demonstrated by different light chain expression. We would appreciate an update on the clinical data (paraproteins, osteolytic lesions etc).  
Such cases can serve as a strong argument for the necessity for routinely performing bone marrow biopsies as part of bone marrow examination, since focal infiltrates of concordant neoplasms can be missed on the aspirate. I can remember a case similar to yours, where the CLL infiltrate was widely spread, but the MM infiltrate was focal, and did not appear on the aspirate.
diane.c.farhi
2008-03-03 23:43
I appreciate seeing this very well-illustrated case. I would be tempted to consider this two manifestations of the same clone, were it not for the different light chain patterns. I believe there have been reports of a kappa-producing neoplasm switching to a lambda-producing one; however, I would think that a biclonal process would be favored over this rare scenario. Gene rearrangement studies of the heavy chain immunoglobulin gene would be of interest; if there is one clone, one would expect one or two bands, if two, than three or four bands.
ugnius
2008-03-04 11:20
Thank you for kind comments. Clinically (above) it seems to be CLL. It's strange, that by flow Ig kapp restriction was detected (Ig kappa is more expressed in plasma cells in IH). We will proceed with PCR IgH and Ig lab evaluation.
hurwitz
2008-03-04 13:50
Diane has raised an interesting point. Lymphoid neoplasms switching from one light chain to the other, are very rare, but have been observed. Lets wait for your PCR results, Ugnius.  
Probably the CLL is the dominant clinical feature. The Kappa light chain restriction on flow might be due to a sample problem.  
anpo
2008-04-02 23:26
Just want to comment that we had a similar case: coexisting CLL and cyclin D1+ myeloma, clonally unrelated, one kappa , one lambda. These cases do exist!
ugnius
2008-04-03 10:33
Thanx. Just waiting PCR results...
ugnius
2008-05-19 17:47
PCR results: IgH oligoclonal with 2 peaks in Vh-Jh region(most probably biclonal).
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Last modified: 2008-02-27 08:26:17