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Incidental AML M5 or BPDCN in the node (569514)
Incidental AML M5 or BPDCN in the nodenew
Subtitle: B14-18729
Type:
HEMATO
Sender:
ugnius
2014-05-28 08:12
INCTR - EBMWG Hematopathology Online
55 yrs female was previously operated due to ovarian mixed poorly dif. (G3) endometrioid- clear cell adenocarcinoma in the endometriosis background.  
At the staging laparotomy multiple peritoneal biopsies and lymph nodes were taken.  
HISTO: Incidentally found medium sized blastic cell infiltration in the sinuses and extranodaly.  
IH (SUM): LCA+; Vimentin+; CD123+; S100+/- (scattered); CD1a/Langerin(-); CD68; CD4(+/+++); CD56+; CD3/MPO/TdT/CD34/CD99(-); Bcl2+; Ki67 ~70%; HLA DR+; Lysozyme+; CD43+.  
BM: reactive changes in biopsy and ANY signs of abberant mono or blastic population is present.  
CLINICALLY: any signs of disease and skin erruptions.  
 
PROPOSAL: Blastic infiltration in the lymph nodes with immunophenotype compatible with AML M5 or blastic plasmacytoid dendritic cell tumor.  
QUESTION: admixture of other (histiocytic) line?  
 
OF NOTE 1: small reactive node near tumorous nodes with pigmented histiocytes in the sinuses display Langerin positivity in the absense CD1a (heavy pigmentation). The question about specificity of Langerin...  
OF NOTE 2: CD123+ (strong) DC2 cells are seen close to tumor mass (CD123+ faint).  
 
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tzankov
2014-05-30 09:49
Difficult case. CD123 is very faint.. is it possible to stain for TCL1? Is there a suspicious population in the FACS of teh PB or the BM? Does the lady have evidence for myelosarcoma (galbladder ect....)? Did a reliable colleague diagnose the poorly diff. endometroid CA? Is the CA intermingled with the same cells?  
 
I think that the process is neoplastic (myelosarcoma), since it destroys the LN capsule and spreads in the perinodal fatty tissue, the cells look ugly, have a high prolferative activity, spntaneous apoptoses and an aberrant phenotype (CD56 is not a feature of normal plasmacytoid monocytes).  
 
I woudl suggest:  
1. revision of all available tissues for blasts  
2. specific FACS gating strategy for those blasts of her PB and BM aspirates  
3. stain for TCL1, CD117 and MPOX (if the latter two are + --> argument for myelosarcoma)  
ugnius
2014-05-31 00:53
Thanx,  
 
1. I've chacked ovarian tumor- only mixed epithelial notghing more.  
2. I will inform lab/hematologists.  
3. MPO (-), CD117(-)(if remember correctly), no TCL1 presents there.  
 
I feel the same (AML/mSA), but without clinical disease our clinicians sometimes stops.  
 
Best wishes from Chicago.
ugnius
2014-06-14 12:55
Last points:  
1. PET scan negative, any signs of disease clinically.  
2. The patient WITHOUT any treatment under watchfull waiting...  
 
So the situation is still unclear.
tzankov
2014-06-16 16:28
a staging BM biopsy and FACS of the PB and the BM with proper gating shuld be done
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Last modified: 2014-05-28 09:06:08