Consequences of misdiagnosis of lymphomatoid papulosis

Abstract

We report two patients with lymphomatoid papulosis (LyP), who were initially diagnosed as systemic T-cell lymphoma. The patients presented with recurrent self-healing cutaneous lesions and skin biopsies showed a lymphocytic infiltrate with malignant features. Clinico-pathological correlation of findings was not performed and results of staging investigations were misinterpreted. Consequently, both patients were unnecessarily treated with multi-agent chemotherapy, radiotherapy and stem cell/bone marrow transplants and sustained long-term adverse effects. The clinical and histological features of LyP are described and appropriate management discussed in detail. Factors leading to the unnecessary treatment of both patients are examined and several learning points highlighted such as the importance of a multidisciplinary approach.

EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma*

“…13,78 A variety of multiagent chemotherapeutic regimens are able to induce regression of LYP lesions, but almost all patients had relapses shortly after withdrawal. 4,8,10,11,15,66,83 …”

“…The histologic findings of both LYP and PCALCL with large pleomorphic and anaplastic lymphoid tumor cells and the clinical appearance with rapidly growing or multiple lesions may result in misinterpretation as a highly malignant cutaneous or even systemic T-cell non-Hodgkin lymphoma, leading to recommendations for multiagent chemotherapy or even bone marrow transplantation. 10,11 In addition, the increased incidence of second lymphoid neoplasms in LYP patients has been used as support for early and more intense treatment of LYP. 12,13 Although a broad spectrum of therapy regimens has been reported, these have been limited to small cohort series or case reports.…”

Single-Fraction Radiotherapy for CD30+Lymphoproliferative Disorders

“…Due to high relapse rates, maintenance therapy may be used but may be accompanied by long-term complications including a higher incidence of nonmelanoma skin cancer and possible development of hepatic complications from chronic methotrexate use [ 12 ]. In addition, misinterpretation of the clinical presentation of CD30 + LPD for a more aggressive disease (i.e., lymphoma, melanoma, or carcinoma) and the increased incidence of secondary lymphoid neoplasms in LYP patients have led to treatment with systemic chemotherapy or even bone marrow transplantation [ 3 , 13 ]. Kempf et al [ 4 ] consensus guidelines for the treatment of CD30 + LPD recommend consideration of RT for persistent, larger lesions greater than 2 cm.…”

Not bitten by the bug: A CD30+ lymphoproliferative disorder masquerading as arthropod bites

“…Clinicians must incorporate clinical history and exam to distinguish LyP from pcALCL to avoid invasive diagnostic and therapeutic procedures not indicated in LyP . The histology of LyP may also be mistaken for systemic Hodgkin or non‐Hodgkin lymphoma, also leading to unnecessary imaging studies and use of multi‐agent chemotherapy . CD30 + proliferation can also be present in infectious processes, drug reactions, and even arthropod bites …”