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B-cell lymphoma with features intermediate between DLBCL and Burkitt lymphoma (8250) » Bcl2.jpg
Filename: Bcl2.jpg
[B-cell lymphoma with features intermediate between DLBCL and Burkitt lymphoma]
STORY: 56 yrs old Lady (russian citizen) underwent full hysterectomy with adnexa due to clinical diagnosis "ca ovariorum, ascites, hydrothorax, mts ad hepar". Main symptoms were: ascites, hydrothorax, dyspnoea. CA 125: 412. Gastric endoscopy: normal. Sonoscopy: cystic changes in ovaries and "carcinomatosis" in omentum.  
SURGERY PROTOCOL: Tumor masses in both ovaries with cystic changes and destruction. Nodular deposits in parietal peritoneum (0.5cm), omentum, liver (2cm nodule), paraaortic lymphadenopathy. Multiple jejunal adhesions.  
BLOOD: WBC 17.6; PLT 651; RBC 3.61.  
HISTO: Cervix normal. Myomata. Single mural lymphoma infiltrate. Endometrium resting. TUMOR: difusse infiltrate of small- medium calibered, darka and light "blastoid" cells in endometrium, myometrium. The tumor cells with ovoid, irregular, angulated nuclei, multiple mitoses, minimal cytoplasm. Both adnexa and omentum: Identical infiltrates in tubal mucosa, muscular layer, subserosa. Focal "starry sky" areas and "clearer" nodules/zones. Intravascular spread and prominent angiothropism.  
IH: CD20 (+/++) 95%, CD3/CD5(-), CD10(++/+++) 95%, BCL6(+) 30%, MUM1(+) 5%, Ki67(+++)95%, CD23/CD30(-), EBV LMP1(-), TdT/CD34(-), Bcl2(+++) 100%. CD23+ FDC network isa absent. Slight nodularity in central zones of the infiltrate may be caused in part by artefacts, in part infiltrated structures (vascular spaces, follicles, etc.).  
FOLLOW UP: Soon after surgery and preliminary pathology report (with URGENT SITUATION mentioned) issuing the patient moves to Russia/Moscow for further treatment due to economical reasons. Some days before she falls into the coma (CNS involvement???) and after words of the relatives "leukaemia" was additionally detected and "supportive chemotherapy" was administered. The material will be sent to the Russia after request. Direct contact with hematologists here is absent.  
VIRTUAL SLIDES ARE APPENDED.  
 
PROPOSAL: Diffuse aggressive B lymphoma with "blastoid" (TdT negative) and Burkitt's lymphoma features (retaining FC/FL immunophenotype CD10+; Bcl6+/-; Bcl2+; Mum1-): diffuse large B cell lymphoma with Burkitt like features, most probably double/triple hit?  
 
PROPOSED LITERATURE: 1. Diffuse blastoid B-cell lymphoma: a histologically aggressive variant of t(14;18)-negative follicular lymphoma. Chiu A, Frizzera G, Mathew S, Hyjek EM, Chadburn A, Tam W, Knowles DM, Orazi A. Mod Pathol. 2009 Jul 24. [Epub ahead of print].PMID: 19633642  
2. Blastic/blastoid transformation of follicular lymphoma: immunohistologic and molecular analyses of five cases. Natkunam Y, Warnke RA, Zehnder JL, Jones CD, Milatovich-Cherry A, Cornbleet PJ. Am J Surg Pathol. 2000 Apr;24(4):525-34. PMID: 10757399 [PubMed - indexed for MEDLINE]  
 
Thank you for being 2gether.
Sender: ugnius
2009-08-15 15:54
INCTR - EBMWG Hematopathology Online

Last modified: 2009-08-15 15:54:49