Brentuximab Vedotin Is Effective in Sever and Diffuse Lymphomatoid Papulosis, Case Reports

Background:

Lymphomatoid papulosis is a chronic lymphoproliferative disorder and a subtype of cutaneous T cell lymphoma. It has tendency to relapse and self-limit. The histopathological hallmark of lymphomatoid papulosis is the CD30 expression on the atypical lymphoid cells. The trans membranous receptor, CD30 belongs to tumor necrosis factor receptor family which is accountable to carry variable cellular function in addition to proliferation.

The latest evolution of Brentuximab Vedion (BV), a monoclonal antibody designed to target CD30 expressed cells. It characterized by inhibition of microtubular polymerization leading to cell arrest and eventually to cell death. This innovative therapy targets the atypical cells, contribute to the beneficial approach.

We report two diffuse and relapse disfiguring cases of lymphomatoid papulosis LPD treated successfully at our center with Brentuximab Vedion

Methods and result:

Case1: A 28-year-old man, presented with pruritic skin eruption started in the axilla then spread to involve the body and face. Due to unfortunate response to topical and systemic treatment options and in addition to progression and new lesions evolution. Skin punch biopsy undertaken from the left forearm lesion, showed Cutaneous ulcer associated with increased CD30-positive lymphocytes, concerning for CD30-positive T-cell lymphoproliferative disorder in favor of Lymphomatoid papulosis type A (CD-30 positive type). The Immunohistochemical Stains: Few CD30+ cells in a background of small T cells (CD20+) and rare B cells (CD20+) with numerous CD1a+ Langerhan cells and macrophages (CD68+). His examination was unremarkable apart from widespread erythematous skin rash over truck and extremities and palms. The initial blood investigations were normal, systemic disease or concomitant malignancy were excluded by PET CT. No response was reported after two months of Narrow-Band UVB. Due to failure of skin directed therapy and systemic treatment with brentuximab vedion was started every 3 weeks. BV was tolerated well without neuropathy or adverse events. Moreover, the rash and the pruritis resolved. Complete remission (CR) was established after 6 cycles.

Case2: A 47-year-old woman, presented initially with self-limiting localized ulcerative skin lesion over the neck and the abdomen. The initial biopsy showed CD30 positive cutaneous T cell lymphoproliferative lesion, Clonal T cell gene rearrangements were amplified by PCR analysis. No FDG avid lesion detected by PET CT nor clonality of bone marrow excluding the Systemic involvement. Due to self-resolving lesions, the patient was kept on watch and wait. After 3 years, a new large ulcer developed occupying large area on the lateral chest wall with skin necrosis a re-biopsy was in favor of persistent lymphomatoid papulosis, Ancillary Studies: CD30, CD43, CD8, CD7, CD25, CD5, CD4, CD3 and CD2: Positive in the atypical lymphocytes; PAX5, EMA, CD20, ALK, HSV1 and HSV2: Negative. The recurrence of cutaneous large FDG lesion at the left lateral and posterior chest skin and left axillary lymph nodes was documented by PET CT scan. Systemic therapy with brentuximab vedion (BV) was initiated and a total of 10 cycles were administered. Migraine with aura provoked by BV for which 25% dose reduction done. Apart from that, therapy tolerated well and the lesion healed. PET CT follow up confirmed CR.

Conclusion:

Brentuximab vedion (BV) in the management of relapsed or diffused Lymphomatoid papulosis LPD has significantly improved treatment-outcomes, resulting in maintained complete response as well as safety and tolerability. These two cases demonstrate the potential of BV to provide disease control and remission induction after failure of conventional treatment modality.

No relevant conflicts of interest to declare.