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Journal of Clinical Oncology, Vol 23, No 16 (June 1), 2005: pp. 3843-3844 © 2005 American Society of Clinical Oncology. DOI: 10.1200/JCO.2005.04.166
Conditions Suggesting LymphomaCASE 1. Lymphomatoid PapulosisUniversity of Missouri, Ellis Fischel Cancer Center, Columbia, MO
Oncology Alliance, Kenosha, WI A 74-year-old white male presented to dermatology with a 3-week history of a nodule that had developed in an area of chronic rash on the inner aspect of the right thigh. He was treated with antifungal agents and antibiotics without success. A skin biopsy was performed and a diagnosis of anaplastic large-cell lymphoma (ALCL) was made based on the presence of CD30-positive T-lymphocytes. Assuming that the skin lesion was a manifestation of an underlying advanced disease, the patient was referred to medical oncology for evaluation of systemic therapy. The patient denied night sweats, fever, or weight loss. On examination there was a 3 x 5 cm well-demarcated, erythematous plaque with a 5 x 5 mm raised nodule with central umblicated ulceration on the inner right thigh (Fig 1). In addition, there were numerous 1 to 2 mm papules on the lower extremities and dorsal surface of the ankle (Fig 2). The patient recalled that the rash and the papules had been waxing and waning for the previous 2 years without any pruritis. There was no peripheral lymphadenopathy, hepatomegaly, or splenomegaly. Blood counts, chemistry, including lactate dehydrogenase, and computed tomography of chest, abdomen, and pelvis were unremarkable. Biopsies of the erythematous plaque and the papules had similar histopathology, consisting of dense lymphoid aggregates filling and expanding the papillary dermis with numerous background inflammatory cells (Fig 3). Mitoses were frequent. The anaplastic lymphoma kinase stain was negative. The dermal lymphoid infiltrate consisted of polymorphous CD3- and CD4-positive T-lymphocytes, which were also CD30-positive (Fig 4). Many atypical lymphocytes had Reed-Sternberg (RS) -like features. With the history of resolving and remitting skin lesions and CD30-positive T-lymphocytic infiltrate, a diagnosis of lymphomatoid papulosis (LP) was established. Therefore, no chemotherapy was offered; however, topical application of corticosteroid resulted in regression of the large right thigh plaque. The smaller papules remained stable at 1-year follow-up.
LP is a distinct entity in the WHO classification of lymphoid malignancies and is grouped with primary cutaneous ALCL and borderline lesions under the category of cutaneous CD30-positive lymphoproliferative disorders.1 These entities constitute a spectrum of related cutaneous conditions originating from a transformed or activating CD30-positive T-lymphocyte. LP is a chronic recurrent skin disease characterized by appearance of papules and/or nodules, which regress spontaneously, typically within 3 to 6 weeks. Lesions greater than 2.5 cm may not regress completely and may leave residual scars with hypo- or hyperpigmented skin.2 Histologically, there is an atypical T-cell infiltrate, which mimics a T-cell lymphoma. The putative cell of origin is an activated skin homing T-lymphocyte.3 Fully developed papules show wedge-shaped dermal infiltrates of atypical T-cells admixed with varying proportions of inflammatory cells such as neutrophils, eosinophils, macrophages, and small lymphocytes.4 The atypical T-cells may either simulate RS features akin to Hodgkin's lymphoma or their nucleus may resemble the cerebriform appearance of mycosis fungoides. The presence of RS-like cells along with numerous inflammatory cells constitutes type A lesions, whereas accumulation of cells with cerebriform nuclei in the absence of inflammatory cells exemplifies type B lesions.5 Both lesions may exist in the same patient. Immunophenotypic features include CD3-positive, CD4-positive, and CD8-negative T-lymphocytes. CD30 is present in type A lesions but may be absent in type B lesions. Anaplastic lymphoma kinase protein is consistently absent. Although clonally arranged TCR genes have been noted in the majority of type B lesions and occasionally in type A lesions, the t(2;5) translocation, which is a characteristic feature of ALCL, is universally absent.6 Due to the overlapping clinical (waxing and waning in borderline lesions), histologic (RS-like cells in Hodgkin's lymphoma and cerebriform cells in mycosis fungoides), and immunohistochemical (CD30-positive in ALCL and Hodgkin's lymphoma) features, the diagnosis of LP requires vigilance by the hematopathologist and the oncologist to avoid unnecessary cytotoxic therapy, as highlighted in this case. Authors' Disclosures of Potential Conflicts of Interest The authors indicated no potential conflicts of interest.
REFERENCES 1. Raifkiaer E, Delsol G, Willemze R, et al: Primary cutaneous CD30-positive T-cell lymphoproliferative disorders in World Health Organization classification of tumors, in Jaffer ES, Harris NL, Stein H, et al (eds): Tumours of Haematopoietic and Lymphoid Tissues. Lyon, France, IARC Press, 2001
2. Bekkenk MW, Geelen FA, van Voorst Vader PC, et al: Primary and secondary cutaneous CD30+ lymphoproliferative disorders: A report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment. Blood 95:3653-3661, 2000 3. McCarty MJ, Vukelja JV, Sausville EA, et al: Lymphomatoid papulosis associated with Ki-1-positive anaplastic large cell lymphoma. A report of two cases and a review of the literature. Cancer 74:3051-3058, 1994[CrossRef][Medline] 4. Chott A, Vonderheid EC, Olbricht S, et al: The dominant T cell clone is present in multiple regressing skin lesions and associated T cell lymphomas of patients with lymphomatoid papulosis. J Invest Dermatol 106:696-700, 1996[CrossRef][Medline]
5. Siegel RS, Pandolfino T, Guitart J, et al: Primary cutaneous T-cell lymphoma: Review and current concepts. J Clin Oncol 18:2908-2925, 2000
6. Beylot-Barry M, Groppi A, Vergier B, et al: Characterization of t(2;5) reciprocal transcripts and genomic breakpoints in CD30+ cutaneous lymphoproliferations. Blood 91:4668-4676, 1998
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Copyright © 2005 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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