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B NHL plasmoid cyclinD1+: biclone? (706880)
B NHL plasmoid cyclinD1+: biclone?new
Subtitle: B15-47529
Type:
HEMA
Sender:
ugnius
2016-01-16 11:33
INCTR - EBMWG Hematopathology Online
UPLOADED 100%: WELLCOME:  
 
73 yrs old male underwent threpine biopsy due to suspition of LPL (WM).  
CLINICS: Consultation case due to anemia. Proteinemia 120 g/l, ~ 50% M gradient.  
HISTO: Cellularity 90%, up to 60% difusse lymphoid infiltration with Ig inclussions. additionally- coarse plasma cell clusters up to 20%. No fibrosis.  
 
IH: CD21/CD23(-)(FDC absent, single lymphos CD23+); CD20(+/++)(heterogenous) 80%; cyclinD1(++)(brand. r-ja)(up tp 20- 25% in probably plasmoid cells); CD79a(+/+++) 100% (plasma cells more prominent); IgM(+/+++) 100% (both populations); IgD(-)(pavieniai limfocitai silpnai +); CD43(-)(granuliopoezė +);  
CD3/CD5+ small medium interstitial lymphos;  
CD138/CD38+ plasmoid infiltrates up to 20%: CD56(-); IgG+ singles only; Ig kappa(+/+++) >> Ig lambda; CD117 (-)(mastocytes +).  
 
MOLECS and FLOW (BM): pending;  
 
TREATMENT: a) plasmapharesis; b) hemotransfussion; c) CycloPhos+ steroids; d) throbo-prophylaxis, ABs, hyperuricaemia correction. IgM going down, slight lymphocytosis in blood transforming into lymphopenia;  
 
PROPOSAL: DIFFICULT TO CLASSIFY:  
 
a) B NHL with plasmoid diff., 60% BM. IH: CD20/CD79a/Pax5+; LCA+; CD138/CD38/CD23/CyclinD1(-); IgM+; IgG/IgD(-); Ig kappa/lambda(-)(?);  
b) Plasmoid hyperplasia, 20% BM with IH: CD20/CD79a+; CD138/CD38+; IgM+; LCA+; Pax5(-)(?); Ig kappa+; CyclinD1+; IgD/IgG(-).  
FLOW and molecular evaluation recommended(MYD88? subpopulation clonal analysis?).  
Comments:  
 
1. CyclinD1+ not compatible with WM, but general IH fits.  
2. MM lymphoplasmacytic variant: not compatible due to focal CD138+; IgM+; IgG(-);  
3. 2 diseases possible?: B LPL (WM) ir MGUS/MIELOMA?  
 
Thank you beeing close.
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tzankov
2016-01-19 16:19
I would favor hypothesis 3 and start with a CCND1/CD38 doublestain as well as ask for FACS results on potentially abnormal plasma cells. Finally a PCR-based IGH analysis may be helpful to detect biclonal population(s). CD19 may be helpful as well, since MM plasma cells are -, while LPL plasmycytoid cells may be +.
ugnius
2016-01-20 07:02
Thank you. Unfortunatelly our lab is rigid enough for "double stains" and I have not CD19...  
MYD88 was found, FACS in progress- I'll append new data, if appears.
tzankov
2016-01-20 21:29
MYD88 fits with LPL. let´s see what the FACS analysis shows... is the M-gradient mono or biclonal?
ugnius
2016-01-22 14:06
Specially for you: CyclinD1+ and CD138+ DOUBLE- but surprisingly on DIFFERRENT populations...
tzankov
2016-01-22 17:42
well you fasified your hypothesis 3, then you can turn to hypothesis 1. some CLL have CCND1+ proliferation centers, some MZL (SMZL) express CCND1...
Juskevicius
2016-03-22 16:06
You may consider a possibility of concurrent hairy cell leukemia which may explain subtle interstitial infiltration pattern of CCND1+ small B cells. I have seen several cases of CLL with concurrent HCL but have not seen LPL with HCL. FACS should be very helpful with that.
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Last modified: 2016-03-22 07:36:31