[1347] Marginal Zone Lymphoma in Multiple Locations: A Progression or a De Novo Disease?

Artur Rangel Filho, Jennifer R Chapman, Offiong F Ikpatt. University of Miami, FL; Jackson Memorial Hospial, Miami, FL

Background: Extra nodal marginal zone lymphomas (MZL) occurring in more than one anatomical site in the same patient are traditionally expected to be a progression of same tumor but may represent the development of a different disease.
Design: We examined six consecutive cases of extra-nodal marginal zone lymphoma (MZL) diagnosed in at least two distinct anatomical sites over a period of time. Clonality assay for IgH/light chain gene rearrangements were performed by PCR followed by capillary electrophoresis.
Results: The time lapse between the first diagnosis and subsequent tumor ranged from 4 months to 4.5 years.
Of these, 3 (50%) had a discordance in the specific IGH gene rearrangement (allele sizes) between the tumors in same patient.
The body parts involved in these cases were:
Patient 1: gastric then cutaneous
Patient 2: ocular then lung
Patient 3: ocular then retroperitoneum
Additionally we attempted to demonstrate any reproducible recurrent patterns of rearrangement in MZL seen at specific sites in 105 cases of MZL by analyzing the frequency of framework region involvement and allele size distribution (Table 1).
Site of MZL......... FR1+/-.......... FR2+/ -......... FR3 +/-......... Kappa +/-/na
ocular .................39 / 10 ........ 35 / 14 ..........35/ 14 ............ 13 / 2 /34
brain ................. 3 / 3 ............ 2 / 4 ............. 2 / 4 .............. 4/ 0 / 2
gastric ............... 17 / 5 .......... 13 / 9 ........... 16 / 6 ............ 5 / 0 / 17
duodenum .......... 2 /1 ............ 3 / 0 ............ 3 / 0 ............. 0 / 0 / 3
cutaneous ........... 2 / 2 ........... 2 / 2 ............. 3 / 1 ............. 1 / 0 / 3
lung .................... 10 / 1 ........ 10 / 1 ............ 9 / 2 ..............1 / 0 / 10
parotid................. 3 / 4 .......... 4 / 3 ............. 5 / 2 ...............2 / 2 / 3
No reproducible recurrent patterns were identified in this sample.
Conclusions: The finding of different allele sizes and therefore different gene rearrangements suggests a de novo tumor rather than a recurrence of the patient's previous lymphoma.
While rare, this phenomenon may have significant implications in protocols for patient treatment and follow-up, especially protocols based on allele specific RT-PCR technology.
Category: Hematopathology

Wednesday, March 2, 2011 1:00 PM

Poster Session VI # 197, Wednesday Afternoon

 

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