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Lymphomatoid Papulosis: A T-Cell Dyscrasia with a Propensity To Transform into Malignant Lymphoma

Fernando Cabanillas, MD; James Armitage, MD; William C. Pugh, MD; Dennis Weisenburger, MD; and Madeleine Duvic, MD
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From The University of Texas M.D. Anderson Cancer Center, Houston, Texas; and the University of Nebraska Medical Center, Omaha, Nebraska. Requests for Reprints: Fernando Cabanillas, MD, Department of Hematology, University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Boulevard (Box 068), Houston, TX 77030.

Copyright ©2004 by the American College of Physicians

Ann Intern Med. 1995;122(3):210-217. doi:10.7326/0003-4819-122-3-199502010-00009
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Objective: To describe the diagnostic difficulties, response to therapy, and clinical features of lymphomatoid papulosis and the cumulative frequency of transformation to lymphoma.

Design: Case series.

Setting: University hospitals.

Methods: The records of 21 patients with lymphomatoid papulosis who were seen from 1986 to 1993 were retrieved from the archives of two institutions. The entry criteria for the study were lymphomatoid papulosis misdiagnosed at the time of original presentation or lymphomatoid papulosis that later developed into lymphoma.

Results: When lymphomatoid papulosis tissues are pathologically examined, they are frequently confused with lymphoma, melanoma, or carcinoma. Eight of the 19 patients whose condition was misdiagnosed as malignant received either chemotherapy or radiotherapy. Although lymphomatoid papulosis responded to cytotoxic chemotherapy, the remissions were transient and promptly recurred after or during treatment. However, all five cases that became malignant responded to chemotherapy and have not recurred. Five of 21 patients (24%) developed lymphoma, but the cumulative risk for transformation after 15 years was 80%.

Conclusions: Lymphomatoid papulosis can only be diagnosed accurately through a careful history in which the characteristic waxing and waning of the skin lesions is identified and through proper communication between clinicians and pathologists. Patients with lymphomatoid papulosis have an increased risk for developing lymphoma that is much higher than the 15% to 20% quoted in the literature. Patients who develop lymphoma respond well to cytotoxic chemotherapy and can be cured with appropriate therapy. Internists and oncologists need to be aware of lymphomatoid papulosis and its characteristic clinical features so that this disorder is accurately diagnosed and so that unnecessary and potentially hazardous treatment is avoided.


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Figure 1.
Multiple skin papules on the right arm.

The small lesions, characteristic of lymphomatoid papulosis, eventually regressed spontaneously.

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Figure 2.
Willemze type A morphology characterized by pleomorphic large lymphoid cells, including Reed-Sternberg-like cells.-

Neoplastic cells expressed an aberrant T-cell phenotype (CD2+, CD3 , and CD30+) and showed clonal rearrangement of T-cell receptor genes. The lesion is indistinguishable from anaplastic large-cell lymphoma.

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Figure 3.
Willemze type B morphology comprising medium-sized lymphoid cells, many with cerebriform nuclear contours.

Epidermotropism was more pronounced in other fields. The lesion histologically resembles mycosis fungoides.

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Figure 4.
Cumulative plot of the risk for transformation to lymphoma.

After 15 years, the risk approaches 80% ± 18%.

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Figure 5.
Lesions on the right elbow showing transformation from Willemze type A lymphomatoid papulosis into Willemze type B (mycosis fungoides type).

The diagnosis was established on clinical grounds, including the size of the lesion and its persistent growth without spontaneous regression.

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