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Lymphoplasmacytic lymphoma (3804)
Lymphoplasmacytic lymphomaclosed
Subtitle: B07-24176
Type:
hemato
Sender:
ugnius
2007-09-25 11:32
INCTR - EBMWG Hematopathology Online
63 yrs woman with clinical diagnosis LPL/Waldenstrom from 2002. Recently it was noticed weight loss and IgG raises up.  
HISTORY: 2002 anemia and ESR elevation (113). Blood: Leu 11,9; Hb 86g/l;RBC 3,22; PLT 294; PROTEINS: general 95,2g/l(albumins 35,5%; globulins 65.5%; ... gama globulins 42.2% with monoclone); IgM 25.58g/l, IgG 24.87g/l. Urine protein 0.27g/l. BM imprint: 68% small lymphos.  
HISTO: B lymphoplasmacytic lymphoma with IgM expression. II° reticulin fibrosis.  
IH: CD20(++) 80% (citopl. r-ja), CD23(-), CD3/CD5(-)(except T), Ki67+ 5%, CyclinD1/Bcl6/CD10/Mum1(-), IgM(+/+++) 60%, IgD(-).  
CD138(+++)plasmacytes 20%: Ig kappa (+++)/Ig lambda apie 2-3/1, Mum1(++). Mastocytes CD117+.  
 
QUESTION: IgM and IgD expression in both ("CLL and LIKE: part1 and 2") cases with plasmacytoid differentiation.  
Thank you for participation.
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diane.c.farhi
2007-10-01 18:25
[comment sent by email]
My impression is that this is either lymphoplasmacytic lymphoma (LPL) or B cell small lymphocytic lymphoma (SLL), which I believe are part of a continuous spectrum without a firm dividing line. The distinction depends on the degree of plasmacytic differentiation, which is hard for me to assess on these photos. On the whole I favor LPL. One photo shows a Russell body, which can be seen in either LPL or SLL. The presence of peripheral blood involvement, or chronic lymphocytic leukemia (CLL), would favor SLL, as LPL doesn't usually have a leukemic phase. Serum protein electrophoresis (SPEP) with appropriate followup studies for clonality (e.g., immunofixation), if a suspicious band is present, should be done to evaluate the possibility of presence of hyperviscosity (Waldenstrom's macroglobulinemia). We would also do urine PEP, looking for Bence Jones protein. Serum viscosity studies could also be done; we don't do them very often here in my lab. Thank you for allowing me to review this case.
 

 

 

 
tzankov
2007-10-03 10:55
for me a clear cut lyphoplasmocytic immunocytoma.  
 
The cases presented here illustrate the DD lymphplasmocytoid CLL vs. lyphoplasmocytic immunocytoma (LPL) and "secretory" marginal zone lymphoma (the latter two considered by Müller-Hermelink as the same entity). lymphplasmocytoid CLL is often IgM and IgD+, while LPL, like in the present case is IgM+ but IgD-.  
 
would you please add pictures of the IgM and igD stains of the case "63 yrs woman, part 2".  
 
did the serum IgG or the M-gradient raise? are these IgGs mono- or polyclonal?
Went
2007-10-12 10:42
i agree with dr. tzankov. the diagnosis of this case, as already mentioned by Dr. Mickys, is LPL, which is frequently IgD-.
ugnius
2007-10-15 09:03
Thank you all for the comments. Appologies for delay: some clinical details of this LPL please find above.
anpo
2007-10-20 23:20
The clinics and morphology in the bone marrow fit well with Morbus Waldenstrom diagnosis - I attach a recent review.
attachment: MW.pdf
ugnius
2007-10-21 11:22
Thank you, dr.Anna for comment and review.
hurwitz
2007-11-12 00:35
The final diagnosis is this case is as all contributors agreed:  
Lymphoplasmacytic lymphoma.  
Thanks to Ugnius for contributing both cases CLL lymphoplasmacytic variant and this case of a typical LPL demonstrating the difference between both conditions. thanks also to all who took part in the discussion.
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Last modified: 2007-09-25 11:32:14