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Spontaneous Regression Of Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg Type

Toberer et al., 2018Lymphoma

Toberer, F., Mechtersheimer, G., Jaschinski, H., Enk, A., Hakim-Meibodi, L., & Haenssle, H. A. (2018). spontaneous Regression of Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg Type. Acta dermato-venereologica, 98(6), 608–609. https://doi.org/10.2340/00015555-2921

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Abstract

We report a case of histologically confirmed primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT) that subsequently underwent spontaneous regression in the absence of systemic treatment. The case showed an atypical lymphoid infiltrate that was CD20<sup>+</sup> and MUM-1<sup>+</sup> and CD10<sup>-</sup>. A subsequent biopsy of the spontaneously regressed lesion showed fibrosis associated with a lymphocytic infiltrate comprising reactive T cells. PCDLBCL-LT is a cutaneous B-cell lymphoma with a poor prognosis, which is usually treated with chemotherapy. We describe a case of clinical and histologic spontaneous regression in a patient with PCDLBCL-LT who had a negative systemic workup but a recurrence over a year after his initial presentation.

Case Details

Disease Location

Left lower leg

Personal Characteristics

66 -year-old male history of diabetes mellitus type ii, arterial hypertension, and chronic venous insufficiency

Clinical Characteristics

Primary cutaneous diffuse large b-cell lymphoma, leg type (pcdlbcl-lt) presented with a 6 week history of a purple tumor on his left lower leg histopathology revealed a dense, diffuse infiltrate of immunoblasts and centroblasts admixed with small reactive lymphocytes within the entire dermis neoplastic cells showed positivity form CD20, 79a, pax5, bcl-2, and mum-1. Ki-67 proliferation index was ~90%. Monoclonal rearrangement of igh chains was found and mutational analysis of exon 5 (p.l265p) of the myd88 gene showed the point mutation c.794t>c. Whole body CT ruled out ln or visceral involvement radiotherapy was recommended 2 months later, the tumor had almost completely regressed and a second biopsy was taken histopathology revealed dermal fibrosis and a less pronounced atypical lymphoid infiltrate showing an identical immunophenotype in comparison with the initial biopsy. In addition, a predominantly CD8+ t-cell infiltrate was evident radiotherapy was initiated

Remission Characteristics

2 months after the diagnostic biopsy, the tumor had almost completely regressed a second biopsy was taken at this time after radiotherapy, there was a complete clinical and histopathological remission

Treatment & Mechanisms

Proposed Remission Mechanisms

CD8+ t-cells appear to play a crucial role in sr apoptosis, reactions of the host's immune system to infections and particular conditions of the tumor microenvironment modulation of the host immune system due to infection and/or traumatic effects

Clinical Treatment

Radiotherapy

Non-Clinical Treatment

None reported