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Splenic MZL DLBCL transformation 2 (495777)
Splenic MZL DLBCL transformation 2new
Subtitle: B13-18519
Type:
HEMA
Sender:
ugnius
2013-07-30 08:34
INCTR - EBMWG Hematopathology Online
STORY: 76 yrs female. After threpine biopsy (polymorphic clonal lymphoid hyperplasia) splenectomy was performed. ~4 yrs progressing splenomegaly. Sweats, weight loss, elevated LDH 560u/l.  
HISTO:  
Spleen: Nodular and focal diffuse, pleomorphic B infiltrate with focal concenantration of "blasts" with fibrosis and macrophageal admixture.  
Threpine: Slightly irregular follicles with normal architecture and scattered perilipocytic B aggregates.  
IH (spleen): CD20+; CD10(-); Bcl6+; MUM1+; Bcl2+; cMYC(-); IgM/IgD+; Cyclin D1(-); EBER(-).  
MOLECS:  
Threpine: B clonal (not the same clone, as in the spleen).  
Spleen: B clonal (different clone).  
VIRTUAL SLIDES: Aperio.  
 
PROPOSAL: 1. Splenic DLBCL (nodular/micronodular, enreached with Ma/T cells, with sclerosis), transformed from SMZL. 2. Clonal B lymphoid hyperplasia in BM, compatible with SMZL spread (?). 3. The different clones: a. Pseudoclonality in BM??? b. Different DLBCL clone expansion???
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tzankov
2013-08-12 13:49
I would call the splenic and nodal process DLBCL. I am not sure that there is enough evidence to speculate that it progressed form a SMZL, since the pattern is nodular and there is no low grdae component in the spleen and no intrasinusoidal component in the bone marrow. More complicated are the bone marrow infiltrates. At first glance they look more reactive (zonated, well circumscribed, centrally located), but B-cells seem to outnuber the T-cells, some of them occasionally touch the bony trabeculae and they are clonal; I would stain them also for CD23 and CD5 to exclude SLL... for the momenet I would assume two independent processes, the differnet clones also fit with this hypothesis. Does the pateint have aberrant B-cells in the blood?
ugnius
2013-08-12 13:55
Thanx, BM (with different clone): CD5/CD23/CyclinD1(-). I will append clinical summary (requested).
ugnius
2013-08-12 15:11
UPGRADED CLIN DATA: HBV/HCV and HIV(-). Therapy: R-CHOP14 (3rd course). Status improved, B symptoms is gone, hemotransfusions not necessary.
ugnius
2013-08-12 15:23
WHO 3 before treatment.
tzankov
2013-08-12 17:20
negativity for both HBV and HCV fits.
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Last modified: 2013-08-03 18:15:30