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Binnary mantle cell lymphoma with plasmacytic diff (1087226)
Binnary mantle cell lymphoma with plasmacytic diffnew
Subtitle: B19-17891
Type:
HEMA
Sender:
ugnius
2019-06-15 15:41
INCTR - EBMWG Hematopathology Online
CASE IS OPEN FOR DISCUSSION:  
 
72 yrs old male in 2019 April addmited with abdominal, left/splenic pain and lymphocytisis in PB. Hepatosplenomegaly, abdominal lymphadenopathy in sonoscopy reveled.  
CT: Active neck, mediastinal, abdominal, inguinal ltmphadenopathy and splenic involvement (Lugano III).  
THREPINE: MCL, conventional variant 65% (nodular central ir peritrabecular and diffuse pattern)  
IH homogenously: CD20+; IgM+ > IgD+; CyclinD1+; CD5+; CD23/CD21(-); Sox11(-); Ki67 index 5%.  
FLOW PB and ASPIRATE: MCL.  
BIOCHEMISTRY/SEROLOGY: ImunofiX IgM kappa monoclone.  
LDH (U/L) 280 (125-243)  
B2 miCroglobulin(mg/L) 12,13 (0,97-2,64)  
Ig G (g/L) 33,23 (7,0-16,0)  
Ig M (g/L) 4,18 (Male 0,40-2,40)  
Ig kappa FREE CHAINS(BS) (mg/L) 158,08 (3,30-19,40)  
Ig lambda FREE CHAINS (BS) (mg/L) 133,52 (5,71-26,30)  
 
LN core biopsy (right inguinal):  
- B MCL with EXTREMALLY plasmoid differentiation(diffuse, nodular and mantle zone pattern).  
Imunofenotype 1 (MAIN MASS): CD20+; IgM+ >> IgG+ (only plasma component) > IgD+; Ig kappa+ (plasma component+); CD138(-)(plasma component+); CD5+; MNDA1+; CyclinD1+; CD23/CD21/Bcl6(-); Sox11(-); Ki67 index low 10%.  
- Immunophenotype 2: Minimal Sox11+ CD20+ "MCL in situ" rings in nodules.  
- Immunophenotype 3: plasma conponent (loosing some B Ags, aquiring plasma Ags).  
 
FISH: CCND1 t(11q13.3) break in BOTH components (MLIS not visible).  
MYD88 L265P mutation absent.  
CLONALITY: clonal IGH FR1-3. No different othe clones present (IGH, IGK, IGL, TCRB, TCRG ir TCRD).  
 
ThankYouAlot.
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tzankov
2019-06-17 07:35
I totally agree with you; plasmacytic differentiation of MCL is not that rare, esepcially in SOX11- cases (as yours).  
 
1. CD5-/SOX-11- mantle cell lymphoma with concomitant monotypic plasmacytic differentiation. Zare SY, Wang HY. Blood. 2016 Oct 6;128(14):1904. doi: 10.1182/blood-2016-06-724757. No abstract available.  
 
2. Plasma cell and terminal B-cell differentiation in mantle cell lymphoma mainly occur in the SOX11-negative subtype. Ribera-Cortada I, Martinez D, Amador V, Royo C, Navarro A, Beà S, Gine E, de Leval L, Serrano S, Wotherspoon A, Colomer D, Martinez A, Campo E. Mod Pathol. 2015 Nov;28(11):1435-47. doi: 10.1038/modpathol.2015.99. Epub 2015 Sep 11.  
 
3. Molecular characteristics of mantle cell lymphoma presenting with clonal plasma cell component. Visco C, Hoeller S, Malik JT, Xu-Monette ZY, Wiggins ML, Liu J, Sanger WG, Liu Z, Chang J, Ranheim EA, Gradowski JF, Serrano S, Wang HY, Liu Q, Dave S, Olsen B, Gascoyne RD, Campo E, Swerdlow SH, Chan WC, Tzankov A, Young KH. Am J Surg Pathol. 2011 Feb;35(2):177-89. doi: 10.1097/PAS.0b013e3182049a9c.
ugnius
2019-06-17 08:00
Thanx. What do You think about Sox11+ mantle zone rimming?
tzankov
2019-06-17 13:49
probably some plasticity or secondary SOX11 loss in the plasma cell coompound
ugnius
2019-06-17 15:23
Thanx twice.
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Last modified: 2019-06-15 16:29:24