Relapse of Plasmablastic Lymphoma With Cutaneous Involvement in an Immunocompetent Male

Abstract

Plasmablastic lymphoma (PBL) is a rare type of non-Hodgkin lymphoma frequently found in the context of immunosuppression and infection with human immunodeficiency virus (HIV) and/or Epstein-Barr virus (EBV). A 33-year-old immunocompetent male presented with recurrent episodes of epistaxis and a growing intranasal mass. Excisional biopsy of the mass revealed an immunohistochemical profile diagnostic of PBL. Upon completion of chemoradiation, he underwent a transnasal endoscopic mucosal flap tissue rearrangement to restore patency for both functional and surveillance purposes. There was no endoscopic evidence of residual or recurrent disease. However, 8 months later, he was found to have a relapse involving the skin. The nasal cavity is one of the most common sites affected by PBL. Involvement of the nasal cavity may present with symptoms of persistent epistaxis accompanied by an enlarging mass. A plasmablastic immunophenotype in combination with HIV or EBV positivity can aid diagnosis.

Scattered Violaceous Papules on the Trunk of an Adult Female: Answer

“…Lesional EBV-encoded RNA (EBER) positivity should prompt consideration of PBL, with EBV reported detectable in 75%–100% of all PBL cases. 3,6,8 By contrast, EBV is only detectable in 10% of the PT-PCM cases. 3 In PBL, MYC translocation has been observed in up to 50% of the cases; however, MYC translocations are also reported in approximately 15% of the plasma cell neoplasms and are present at even higher frequencies in extramedullary lesions.…”

“…3,4 For PBL, the predominant risk factor is immunosuppression, most commonly from HIV, but also from advanced age, immunosuppressive medications, or chemotherapeutics. 6,9,10 In cases where MM has been previously diagnosed or later discovered on marrow biopsy, the diagnosis is presumed to be PT-PCM. 3,13 In patients without underlying MM, features such as renal dysfunction, significant paraproteinemia, osteolytic lesions, and hypercalcemia also favor a diagnosis of PT-PCM.…”

“…3,10 Clinically, they both present with erythematous to violaceous papulonodules. 1,6,11,12 Primary cutaneous PBL favors the lower extremities, but no specific trend for the location of cutaneous involvement is seen in systemic PBL or PT-PCM. 3,6 PT has been reported to occur in approximately 2% of late stage PCMs and can be the initial presentation that leads to a diagnosis of MM.…”

“…1,6,11,12 Primary cutaneous PBL favors the lower extremities, but no specific trend for the location of cutaneous involvement is seen in systemic PBL or PT-PCM. 3,6 PT has been reported to occur in approximately 2% of late stage PCMs and can be the initial presentation that leads to a diagnosis of MM. 3,4 For PBL, the predominant risk factor is immunosuppression, most commonly from HIV, but also from advanced age, immunosuppressive medications, or chemotherapeutics.…”

A Fast-Growing Tumor on the Head of an Elderly Woman: Answer

“…7 In those cases, cutaneous involvement presents as rapidly growing nodular lesions or erythematoviolaceous tumors, which usually affect the head and neck areas. 8,9 By contrast, primary effusive lymphoma and ALKpositive DLBCL are easily distinguishable from PBL and PBM because they have significantly different clinical behavior and immunophenotype. 1,4 The former presents with malignant effusions in serous cavities with an absence of tumor masses.…”