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Case Report
The devolution of a mature plasma cell dyscrasia into a fatal plasmablastic lymphoma
1 DO, MSc, Resident, Internal Medicine, San Antonio Uniformed Services Health Education Consortium, San Antonio, Texas, United States
2 MD, Resident, Pathology, San Antonio Uniformed Services Health Education Consortium, San Antonio, Texas, United States
3 DO, Fellow, Hematology/Oncology, San Antonio Uniformed Services Health Education Consortium, San Antonio, Texas, United States
4 MD, Attending, Pathology, San Antonio Uniformed Services Health Education Consortium, San Antonio, Texas, United States
5 MD, Attending, Hematology/Oncology, San Antonio Uniformed Services Health Education Consortium, San Antonio, Texas, United States
Address correspondence to:
Morgan P Pinto
DO, MSc, San Antonio, Texas,
United States
Message to Corresponding Author
Article ID: 100124Z10MP2023
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How to cite this article
Pinto MP, Thorneloe NS, Brown MR, Stalons ML, Stoll KE, Holmes AR, Pathan M, Gonzales PA. The devolution of a mature plasma cell dyscrasia into a fatal plasmablastic lymphoma. J Case Rep Images Oncology 2023;9(2):7–14.ABSTRACT
Introduction: Plasmablastic lymphoma is a rare, aggressive, non-Hodgkin’s lymphoma with an untreated prognosis as poor as three months. There exists scant literature describing transformation of plasmablastic lymphoma from a more benign dyscrasia, the mature plasmacytoma. This case report describes the transformation of plasmablastic lymphoma from a mature plasma cell neoplasm/plasma cell myeloma in an atypical combination of patient characteristics.
Case Report: A 66-year-old man presented with acute onset right lower extremity pain and rapidly progressive mobility loss. He was found to have a lytic lesion in the lateral right iliac wing. Biopsy revealed the lesion to be plasmablastic lymphoma with Epstein–Barr virus (EBV) positivity by in situ hybridization with a Ki-67 proliferation index >99%, and strongly staining CD138 and MUM-1. CD20 and PAX-5 were negative. A bone marrow biopsy from the right iliac crest showed mature plasma cells without evidence of plasmablastic lymphoma cytology found in the initial specimen. These specimens showed CD138 positivity with 15–20% plasma cells with Kappa positive clonality by in situ hybridization, and diffusely Epstein–Barr virus negative by in situ hybridization. Further plasma cell fluorescence in situ hybridization study showed a clone with a TP53 deletion and an immunoglobulin heavy chain gene rearrangement that did not translocate to one of the common plasma cell dyscrasia translocation partners (FGFR3, CCND1, MAF, or MAFB). Additionally, a near-tetraploid subclone was observed in approximately 60% of nuclei. Also, there was gain of BCL2 gene or chromosome 18/18q, gain of BCL6 gene or chromosome 3/3q and MYC amplification. There was no MYC and BCL2 and/or BCL6 rearrangements. Our patient was neither HIV-positive nor immunocompromised, rather Epstein–Barr virus positive with a quantitative polymerase chain reaction level greater than 67,000. He was started on Daratumumab combined with etoposide, vincristine, doxorubicin, cyclophosphamide, and prednisone.
Conclusion: This case exhibits a unique presentation of plasmablastic lymphoma in terms of disease presentation, unique risk factors, including HIV-negativity and male-assigned sex, and the creativity of treatment utilized.
Keywords: Daratumumab, EPOCH, HIV negative, Plasmablastic lymphoma, Plasmacytoma
SUPPORTING INFORMATION
Acknowledgments
The authors would like to thank the combined efforts of the Hematology/Oncology and Pathology Departments at Brooke Army Medical Center, and the efforts of Dr. Muhammad Pathan at the Veteran’s Affairs Hospital in San Antonio.
Author ContributionsMorgan P Pinto - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Nicholas S Thorneloe - Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Mark R Brown - Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Molly L Stalons - Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Kristin E Stoll - Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Allen R Holmes - Analysis of data, Interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Muhummad Pathan - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Paul A Gonzales - Analysis of data, Interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Guaranter of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2023 Morgan P Pinto et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
