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Case report
peer-reviewed

An Extremely Rare Manifestation of Multiple Myeloma: An Immunoglobulin D Secreting Testicular Plasmacytoma



Abstract

Multiple myelomas (MM) of the immunoglobulin D (IgD) subtype is rare amongst plasma cell malignancies. It can present a diagnostic challenge because of the low amount of immunoglobulin in the serum. The amount of monoclonal (M)-protein is often undetectable on electrophoresis. Historically, survival in these patients was typically shorter compared to the immunoglobulin A (IgA) and immunoglobulin G (IgG) subtypes due to advanced disease upon presentation. With the advent of better diagnostic techniques, the prognosis of this disease is changing. We describe a case of an extramedullary testicular plasmacytoma (EMP) of the IgD subtype as the primary feature of MM, which responded well to novel therapy.

A 72-year-old White male presented to the emergency room with a right testicular mass for three months. He subsequently underwent right radical orchiectomy. Pathology of the specimen revealed plasmacytoid cells positive for cluster of differentiation (CD79a), lambda free light chain, IgD, and BCL-1 (Cyclin D1) on immunochemical stains. Urine and serum immunofixation were positive for monoclonal IgD with lambda light chain specificity and Bence Jones proteinuria. Bone marrow biopsy showed large sheets of plasma cells with greater than 90% cellularity. Flow cytometry displayed atypical plasma cells expressing cluster of differentiation (CD38, CD20, and CD56) with cytoplasm and lambda light chain, approximately 20%, consistent with a plasma cell dyscrasia. Stage 3 IgD lambda multiple myeloma was diagnosed. He received novel treatment with Bortezomib and dexamethasone for three months, followed by Lenalidomide. His performance status and lab data improved significantly. He had progression-free survival (PFS) of approximately three years and remained in complete remission low-dose dose of Lenalidomide daily.

IgD myeloma was considered a diagnostic challenge due to undetectable M-protein levels on serum protein electrophoresis (SPEP). With the advent of serum free light chain assay and serum and cytologic examinations, diagnostic accuracy has significantly improved. The IgD subtype is commonly associated with poor clinical outcomes. However, the use of novel agents and autologous transplant has changed the prognosis of this disease.



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Case report
peer-reviewed

An Extremely Rare Manifestation of Multiple Myeloma: An Immunoglobulin D Secreting Testicular Plasmacytoma


Author Information

Ashish Sharma

Internal Medicine, Aventura Hospital and Medical Center

Tina Binazir Corresponding Author

Internal Medicine, Aventura Hospital and Medical Center

Alexandre Sintow

Internal Medicine, Aventura Hospital and Medical Center

Chi Chan Lee

Internal Medicine, Aventura Hospital and Medical Center

Sameer Shaharyar

Internal Medicine, Aventura Hospital and Medical Center

Jason Tache

Oncology, Westside Regional Medical Center


Ethics Statement and Conflict of Interest Disclosures

Human subjects: Consent was obtained by all participants in this study. Ethics approval and consent to participate

Statement of waiver on ethic approval and consent to participate

I believe that this article is eligible for waiver ethics approval and consent because the article meets all of the following criteria:
1.The article is a case report.
2.The research presents no more than “minimal risk” of harm to subjects.
3.The waiver or alteration will not adversely affect the rights and welfare of the subjects.
Consent for publication
Consent for publication is waived since unfortunately, the patient passed away before we start writing the case report. We attempted to contact his family several times through phone calls but failed. We would continuously try to acquire consent from his family.
. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.


Case report
peer-reviewed

An Extremely Rare Manifestation of Multiple Myeloma: An Immunoglobulin D Secreting Testicular Plasmacytoma


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Case report
peer-reviewed

An Extremely Rare Manifestation of Multiple Myeloma: An Immunoglobulin D Secreting Testicular Plasmacytoma

Ashish Sharma">Ashish Sharma, Tina Binazir">Tina Binazir , Alexandre Sintow">Alexandre Sintow, Chi Chan Lee">Chi Chan Lee, Sameer Shaharyar">Sameer Shaharyar, Jason Tache">Jason Tache

  • Author Information
    Ashish Sharma

    Internal Medicine, Aventura Hospital and Medical Center

    Tina Binazir Corresponding Author

    Internal Medicine, Aventura Hospital and Medical Center

    Alexandre Sintow

    Internal Medicine, Aventura Hospital and Medical Center

    Chi Chan Lee

    Internal Medicine, Aventura Hospital and Medical Center

    Sameer Shaharyar

    Internal Medicine, Aventura Hospital and Medical Center

    Jason Tache

    Oncology, Westside Regional Medical Center


    Ethics Statement and Conflict of Interest Disclosures

    Human subjects: Consent was obtained by all participants in this study. Ethics approval and consent to participate

    Statement of waiver on ethic approval and consent to participate

    I believe that this article is eligible for waiver ethics approval and consent because the article meets all of the following criteria:
    1.The article is a case report.
    2.The research presents no more than “minimal risk” of harm to subjects.
    3.The waiver or alteration will not adversely affect the rights and welfare of the subjects.
    Consent for publication
    Consent for publication is waived since unfortunately, the patient passed away before we start writing the case report. We attempted to contact his family several times through phone calls but failed. We would continuously try to acquire consent from his family.
    . Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

    Acknowledgements


    Article Information

    Published: June 27, 2017

    DOI

    10.7759/cureus.1400

    Cite this article as:

    Sharma A, Binazir T, Sintow A, et al. (June 27, 2017) An Extremely Rare Manifestation of Multiple Myeloma: An Immunoglobulin D Secreting Testicular Plasmacytoma. Cureus 9(6): e1400. doi:10.7759/cureus.1400

    Publication history

    Received by Cureus: April 09, 2017
    Peer review began: May 03, 2017
    Peer review concluded: June 20, 2017
    Published: June 27, 2017

    Copyright

    © Copyright 2017
    Sharma et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 3.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

    License

    This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Abstract

Multiple myelomas (MM) of the immunoglobulin D (IgD) subtype is rare amongst plasma cell malignancies. It can present a diagnostic challenge because of the low amount of immunoglobulin in the serum. The amount of monoclonal (M)-protein is often undetectable on electrophoresis. Historically, survival in these patients was typically shorter compared to the immunoglobulin A (IgA) and immunoglobulin G (IgG) subtypes due to advanced disease upon presentation. With the advent of better diagnostic techniques, the prognosis of this disease is changing. We describe a case of an extramedullary testicular plasmacytoma (EMP) of the IgD subtype as the primary feature of MM, which responded well to novel therapy.

A 72-year-old White male presented to the emergency room with a right testicular mass for three months. He subsequently underwent right radical orchiectomy. Pathology of the specimen revealed plasmacytoid cells positive for cluster of differentiation (CD79a), lambda free light chain, IgD, and BCL-1 (Cyclin D1) on immunochemical stains. Urine and serum immunofixation were positive for monoclonal IgD with lambda light chain specificity and Bence Jones proteinuria. Bone marrow biopsy showed large sheets of plasma cells with greater than 90% cellularity. Flow cytometry displayed atypical plasma cells expressing cluster of differentiation (CD38, CD20, and CD56) with cytoplasm and lambda light chain, approximately 20%, consistent with a plasma cell dyscrasia. Stage 3 IgD lambda multiple myeloma was diagnosed. He received novel treatment with Bortezomib and dexamethasone for three months, followed by Lenalidomide. His performance status and lab data improved significantly. He had progression-free survival (PFS) of approximately three years and remained in complete remission low-dose dose of Lenalidomide daily.

IgD myeloma was considered a diagnostic challenge due to undetectable M-protein levels on serum protein electrophoresis (SPEP). With the advent of serum free light chain assay and serum and cytologic examinations, diagnostic accuracy has significantly improved. The IgD subtype is commonly associated with poor clinical outcomes. However, the use of novel agents and autologous transplant has changed the prognosis of this disease.



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Ashish Sharma

Internal Medicine, Aventura Hospital and Medical Center

Tina Binazir, M.D., Resident Physician

Internal Medicine, Aventura Hospital and Medical Center

For correspondence:
tinaataian9@gmail.com

Alexandre Sintow

Internal Medicine, Aventura Hospital and Medical Center

Chi Chan Lee

Internal Medicine, Aventura Hospital and Medical Center

Sameer Shaharyar

Internal Medicine, Aventura Hospital and Medical Center

Jason Tache

Oncology, Westside Regional Medical Center

Ashish Sharma

Internal Medicine, Aventura Hospital and Medical Center

Tina Binazir, M.D., Resident Physician

Internal Medicine, Aventura Hospital and Medical Center

For correspondence:
tinaataian9@gmail.com

Alexandre Sintow

Internal Medicine, Aventura Hospital and Medical Center

Chi Chan Lee

Internal Medicine, Aventura Hospital and Medical Center

Sameer Shaharyar

Internal Medicine, Aventura Hospital and Medical Center

Jason Tache

Oncology, Westside Regional Medical Center