|Year : 2020 | Volume
| Issue : 4 | Page : 254-255
Cutaneous adult T-cell leukemia/lymphoma mimicking primary cutaneous anaplastic large-cell lymphoma
Mariko Takaoka1, Tomonori Takekoshi1, Maasa Kobayashi1, Rina Nakajima1, Hiroaki Asai2, Makoto Sugaya3
1 Department of Dermatology, International University of Health and Welfare Mita Hospital, Minato-ku, Tokyo, Japan
2 Lymphoma/Hematology Center, International University of Health and Welfare Mita Hospital, Minato-ku, Tokyo, Japan
3 Department of Dermatology, International University of Health and Welfare Ichikawa Hospital, Ichikawa, Chiba, Japan
|Date of Submission||22-Feb-2020|
|Date of Decision||20-May-2020|
|Date of Acceptance||09-Jun-2020|
|Date of Web Publication||16-Dec-2020|
Dr. Makoto Sugaya
Department of Dermatology, International University of Health and Welfare Ichikawa Hospital, 6-1-14 Konodai, Ichikawa, Chiba 272-0827
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Takaoka M, Takekoshi T, Kobayashi M, Nakajima R, Asai H, Sugaya M. Cutaneous adult T-cell leukemia/lymphoma mimicking primary cutaneous anaplastic large-cell lymphoma. Dermatol Sin 2020;38:254-5
|How to cite this URL:|
Takaoka M, Takekoshi T, Kobayashi M, Nakajima R, Asai H, Sugaya M. Cutaneous adult T-cell leukemia/lymphoma mimicking primary cutaneous anaplastic large-cell lymphoma. Dermatol Sin [serial online] 2020 [cited 2022 Dec 3];38:254-5. Available from: https://www.dermsinica.org/text.asp?2020/38/4/254/303698
Tumor cells of adult T-cell leukemia/lymphoma (ATLL), which are likely derived from cells with the T-regulatory phenotype, sometimes express CD30 and such cases are clinically and pathologically almost indistinguishable from anaplastic large-cell lymphoma. Here, we report a case of ATLL mimicking primary cutaneous anaplastic large-cell lymphoma (pcALCL).
A 53-year-old Japanese male, who was born in the southwestern part of Japan, presented with indurated masses on the chest. He noticed erythema on the chest 2 months before and it grew rapidly over a period of 10 days. Physical examination showed three well-defined erythematous nodules with erosion on the left anterior chest [Figure 1]a. Laboratory study revealed abnormal lymphocytes in the peripheral blood (1.5%), lactate dehydrogenase level of 221 U/L, calcium level of 9.6 mg/dL, and soluble interleukin-2 receptor (sIL-2R) level of 1110 U/mL. No nodal or organ involvement was detected. All the nodules were excised. Histopathological examination revealed dense infiltrate of pleomorphic large atypical lymphocytes [Figure 1]b, which were CD30 positive [Figure 1]c and anaplastic lymphoma kinase negative, into subcutaneous tissues. The diagnosis in a pathology report was pcALCL. Clinical tumor, node, and metastasis classification was T2aN0M0. However, there were also CD30− atypical small lymphocytes in the periphery of the lesions [Figure 1]d. The fact that the patient was from southwestern part of Japan, a human T-cell lymphotropic virus type I (HTLV-1) endemic area, and that both CD30+ large cells and CD30− small cells expressed CD25 [Figure 1]e raised the possibility of ATLL. Interestingly, CD30+ large cells were negative and a part of small atypical cells were positive for forkhead box P3 (Foxp3) [Figure 1]f. The patient turned out to be HTLV-1 seropositive and the monoclonal integration of HTLV-1 genomes was detected in the lesional skin by Southern blotting, leading to the diagnosis of ATLL (T2aN0M0). Three months after the initial presentation, multiple erythematous papules appeared on the trunk and limbs [Figure 1]g. Histology showed dense infiltrate of atypical cells, some of which were CD30 positive, into the dermis [Figure 1]h. He also suffered from herpes zoster and oral candidiasis, suggesting immunodeficiency associated with HTLV-1 infection. Five months after the initial presentation, acute transformation occurred with sIL-2R level increasing to 19800 U/mL and chemotherapy was started.
|Figure 1: (a) Initial clinical presentation. Three well-defined erythematous nodules with erosion on the left anterior chest. (b) Histology of the nodule. Dense infiltrates of pleomorphic large atypical lymphocytes in subcutaneous tissue (×400). (c) Larger tumor cells positive for CD30 (×400). (d) Scattered CD30+ large cells in the periphery of the lesion (×200). (e) Both CD30+ large cells and CD30- small cells positive for CD25 (×200). (f) CD30+ large cells negative for Foxp3. (g) Erythematous papules on the thigh. (h) Dense infiltrates of dysplastic cells in the dermis|
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We reported a case of cutaneous ATLL mimicking pcALCL. Clinical presentation of ATLL is sometimes indistinguishable from other T-cell lymphomas such as mycosis fungoides/Sézary syndrome and pcALCL. In our case, CD25, commonly expressed by ATLL tumor cells, was expressed not only by CD30+ large cells but also by CD30− small atypical lymphocytes, which was helpful for reaching a correct diagnosis. Foxp3 is reported to be expressed by pleomorphic small and medium cell types and be lost with large cell transformation. Consistently, CD30+ large cells were negative and a part of small atypical cells were positive for Foxp3 in our case [Figure 1]f. Foxp3, a master control transcription factor for regulatory T cells, is a relatively specific maker for ATLL, although it can be expressed by other lymphomas.
PcALCL has a favorable prognosis with 5-year survival rates between 76% and 96% and its initial treatment is surgical excision or radiotherapy, whereas ATLL is associated with a poor prognosis; the mean survival time is 17.3 months for both nodulotumoral and multipapular types. Although our case had only skin lesions at initial presentation, chemotherapy was needed due to the aggressive disease course. If we had only focused on atypical large cells positive for CD30 and negative for Foxp3, we could not have reached the diagnosis. Our case reminds us of importance of ruling out ATLL even if the diagnosis in a pathology report is another type of lymphoma.
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