Immunosuppression-associated primary cutaneous plasmablastic lymphoma secondary to romidepsin

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

“…3,6,8,10 PBL is a rare and aggressive variant of diffuse large B-cell lymphoma with characteristic EBV positivity and is usually seen in the immunocompromised. 3,9 It is reported to involve the skin in approximately 6% of cases. 10,13,14 PT-PCM and PBL can have virtually identical histologic features creating a diagnostic challenge.…”

“…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.…”

“…[1][2][3][4]15 Although intensive chemotherapy regimens have been attempted, there seems to be no survival benefit compared with CHOP (cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisone) or CHOP-like regimens. 9,10,15,17…”

“…Mature B-cell markers, including CD19, CD20, and PAX-5, and T-cell markers are typically negative or weak. 2,3,[6][7][8][9][10][11][12][13] CD79A expression is variable. [13][14][15] Aberrant T-cell marker expression however has been reported.…”