Cutaneous Peripheral T-Cell Lymphoma Associated With a Proliferation of B Cells

Abstract

Although the new World Health Organization-European Organization for Research and Treatment of Cancer classification focuses on providing uniformity in the diagnosis of cutaneous lymphomas, cutaneous peripheral T-cell lymphoma (PTL) remains a poorly defined subgroup. As follow-up to a study of systemic PTL complicated by a proliferation of B cells, we studied 16 cases of cutaneous PTL that contained morphologically atypical T cells associated with a significant infiltrate of B cells (about 20%-50%). A clonal T-cell receptor gamma chain gene rearrangement was present in all cases. In contrast, a clonal immunoglobulin heavy chain gene rearrangement was present in only 1 case. Clinical staging in 14 cases identified systemic involvement in 2. At last follow-up, both patients with systemic involvement had died of disease, and the majority of patients with primary cutaneous disease were alive (11/12). The presence of numerous atypical B cells and T cells caused diagnostic confusion in these cases. Comprehensive pathologic studies, coupled with clinical staging, are necessary for the accurate diagnosis of this unusual manifestation of cutaneous PTL.

Primary Cutaneous, Composite, Epstein-Barr Virus-Associated, Diffuse Large B-cell Lymphoma and Peripheral T-cell Lymphoma

“…27 B-cell clonality analysis is very important in these instances because unlike our patient (and those described in category 4 below), the prognosis for these patients is relatively good if the patient did not have systemic disease before or after the time of diagnosis. 27,30 Accordingly, some of these cases are thought to represent primary cutaneous CD4 + small-to mediumsized T-cell lymphoma in patients who had solitary lesions that resolved. 27 Because of the polyclonal nature of the B-cell component in cases in this category, their presence is thought to represent a bystander effect where the expansion of malignant T cells stimulates emigration of B cells.…”

“…Furthermore, cutaneous PTCL cases with EBV-associated B cells rarely demonstrate evidence of B-cell clonality, which is also contrary to the current case. 27 Morphologically, however, these B-cell proliferations often had large B-cell lymphoma characteristics. 27 B-cell clonality analysis is very important in these instances because unlike our patient (and those described in category 4 below), the prognosis for these patients is relatively good if the patient did not have systemic disease before or after the time of diagnosis.…”

“…27 Morphologically, however, these B-cell proliferations often had large B-cell lymphoma characteristics. 27 B-cell clonality analysis is very important in these instances because unlike our patient (and those described in category 4 below), the prognosis for these patients is relatively good if the patient did not have systemic disease before or after the time of diagnosis. 27,30 Accordingly, some of these cases are thought to represent primary cutaneous CD4 + small-to mediumsized T-cell lymphoma in patients who had solitary lesions that resolved.…”

“…[22][23][24][25] More recently, however, the role of EBV in cutaneous lymphoproliferative processes occurring in the setting of T-cell malignancies has become an active area of study. 1,26,27 Therefore, the classification of a cutaneous B-cell lymphoproliferative disorder occurring in the context of a T-cell-rich background/ clonal malignancy requires consideration of the following:…”

“…27,30 Accordingly, some of these cases are thought to represent primary cutaneous CD4 + small-to mediumsized T-cell lymphoma in patients who had solitary lesions that resolved. 27 Because of the polyclonal nature of the B-cell component in cases in this category, their presence is thought to represent a bystander effect where the expansion of malignant T cells stimulates emigration of B cells. 31 The risk of the emergence of a clonal B-cell population portending a worse prognosis is unclear.…”