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© 2001 American Society for Clinical Oncology
Unusual Locations for LymphomasCase 3. Successive Occurrence of Peripheral T-Cell Lymphoma With Bilateral Conjuctival Involvement in a Patient With Low-Grade B-Cell LymphomaThe John Hopkins University School of Medicine, Baltimore, MDCopyright © 2001 American Society of Clinical Oncology An 81-year-old Japanese man with a history of low-grade upper gastrointestinal (GI) lymphoma was referred for the evaluation of new skin nodules and bilateral conjunctival masses. His lymphoma had been diagnosed 3 years ago when he presented with recurrent GI bleeding. A duodenal biopsy revealed malignant lymphoma, follicular, predominantly small cleaved-cell type; B-cell markers and bcl-2 were expressed. The gene rearrangement studies showed a clonal B-cell population, which was expressing µu heavy chain. He was subsequently treated with external-beam radiation therapy, which resulted in stable disease status since then. He subsequently noted two small lumps on his left elbow. A skin biopsy was obtained and reported to be cutaneous T-cell lymphoma other than Mycosis fungoides. It was CD30 (Ki-l)–negative. The skin nodules, which were reddish-purple in color, progressively increased in size and he noted a similar nodule and plaque-like erythematous skin lesions on the sternum, right arm, and other areas of his extremities and anterior trunk. Two months before presentation at our center, he also developed redness in his left eye with decreased visual acuity, which was initially diagnosed as scleritis, and treated with topical corticosteroids. However, his symptoms persisted along with increasing size of swelling in both his eyes (Fig 1). A slit-lamp examination of the eye disclosed diffuse conjunctival and episcleral injection, along with pebbly appearance of the left lower forniceal conjunctiva (Fig 2A and 2B). A conjunctival biopsy disclosed a CD3-positive (Fig 3A) moderately intense infiltrate of normal-appearing, intermediate size lymphocytes and scattered plasma cells in the substantia propria (Fig 3B). A partial loss of CD5 and CD7 and the predominance of CD8 were noted. Overall, the findings supported the diagnosis of conjunctival involvement by peripheral T-cell lymphoma (PTCL). He had no palpable lymphadenopathy. Skin examination was notable for a nodular skin lesion with erythematous background measuring approximately 5 x 6 cm on his left upper extremity, which was reddish-purple in color with no scaling. A similar lesion was also noted on his lower parasternal area measuring approximately 4 x 3 cm (Fig 4). He also had several small patchy skin plaques with the same color and texture noted. His abdomen was soft with no hepatosplenomegaly or any other abdominal mass. Computed tomography scan of the chest, abdomen, and pelvis revealed the presence of enlarged adrenal glands, consistent with infiltration by lymphoma. There was a minimal thickening of the residual stomach.
The patient’s skin biopsy revealed a dense dermal infiltrate of abnormal lymphoid cells, which were intermediate in size and had convoluted nuclear contours (Fig 5A). Immunostaining evaluation demonstrated the neoplasm to be CD3-positive T-cells (Fig 5B). CD5 was largely lost on the majority of the neoplastic T cells. In addition, the T cells were positive for CD8 and negative for CD30 and CD15. Serologic evaluation of human T-cell lymphotrophic virus type 1 (HTLV-1) antibody was negative. The patient was diagnosed as having peripheral T-cell lymphoma involving his skin, conjunctiva, and possibly adrenal glands. Chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone was carried out. He had a near complete response after two cycles of chemotherapy with resolution of his skin lesions and conjunctival mass. However, his treatment course had to be interrupted because of significant GI bleeding. A gastric biopsy obtained from the bleeding ulcer site revealed PTCL with no morphologic evidence of B-cell lymphoma. His skin and conjunctival lesions started to progress while he was off chemotherapy. He received palliative external-beam radiation to his bilateral conjunctivae because of the tumor progression and intractable glaucoma. The patient died of progressive systemic disease shortly after the radiation therapy.
This patient is unique in two respects. The occurrence of PTCL in the course of low-grade non-Hodgkin’s lymphoma is rare, and bilateral conjunctival involvement by PTCL is evidently unprecedented. Several cases of combined B-cell and T-cell lymphoproliferative disorders in various forms have been previously reported, but PTCL presenting with unusual site of involvement in the course of an indolent B-cell lymphoma has not been described.1-6 The successive occurrence of PTCL in the course of a low-grade lymphoma may implicate the possible role of genetic derangement affecting the apoptosis in the development or transformation of biologically aggressive lymphomas.7 The other explanation of this association could be infectious etiologies. Many infectious agents such as HTLV-18 and Epstein-Barr virus9 have been implicated in the development of T-cell lymphomas, but our patient did not have any evidence of these infections either by serology or genomic studies in biopsy samples despite the fact that he had a history of living in the area of Japan where the prevalence of HTLV-1 infection is high. The second and maybe more interesting aspect of the case was the unique presentation of his PTCL. Isolated conjunctival T-cell lymphoma is a very rare but known entity, whereas simultaneous skin and bilateral conjunctival involvement with PTCL has not been described. Normally, we would expect the patient’s bilateral conjunctival involvement to be a distant recurrence of smoldering low-grade B-cell lymphoma, because of the fact that low-grade lymphoma of B-cell type, lymphoma of mucosa-associated lymphoid tissue in particular, is the most common ocular adnexal lymphoid neoplasm.10 In view of the similarities of histopathologic findings, the clinical pattern of his skin lesions and conjunctival involvement, we concluded that the lymphoma in his skin and conjunctiva were different manifestations of the same T-cell tumor. Subsequently, his disease progressed in all skin sites involved as well as in his conjunctival tissues despite radiation therapy given to his eye. Interestingly, his stomach was later noted to be involved by PTCL. This rapid progressive course is not unusual for PTCL, which tends to be chemoresistant shortly after the initial therapy and has a high proliferative rate.11 This case shows that PTCL may present with various clinical manifestation, including ocular involvement. Obtaining multiple biopsies from accessible sites may help with identifying these histologically discordant lymphoid neoplasms. REFERENCES 1. Wlodarska I, Delabie J, De Wolf-Peeters C, et al: T-cell lymphoma developing in Hodgkin’s disease: Evidence for two clones. J Pathol 170: 239-248, 1993[Medline] 2. Deane M, Amlot P, Pappas H, et al: Independent clonal origin of T- and B-cell clones in a composite lymphoma. Leuk Res 15: 811-817, 1991[Medline] 3. Abruzzo LV, Griffith LM, Nandedkar M, et al: Histologically discordant lymphomas with B-cell and T-cell components. Am J Clin Pathol 108: 316-323, 1997[Medline]
4.
Micallef IN, Kirk A, Norton A, et al: Peripheral T-cell lymphoma following rituximab therapy for B-cell lymphoma. Blood 93: 2427-2428, 1999 5. Hancock JC, Wells A, Halling KC, et al: Composite B-cell and T-cell lymphoma arising 24 years after nodular lymphocyte predominant Hodgkin’s disease. Ann Diagn Pathol 3: 23-34, 1999[Medline] 6. Strickler JG, Amsden TW, Kurtin PJ: Small B-cell lymphoid neoplasms with coexisting T-cell lymphomas. Am J Clin Pathol 98: 424-429, 1992[Medline]
7.
Cotter FE, Hall PA, Young BD, et al: Simultaneous presentation of T- and B-cell malignant lymphoma with bcl-2 gene involvement. Blood 73: 1387-1388, 1989
8.
Hall WW, Liu CR, Schneewind O, et al: Deleted HTLV-1 provirus and cutaneous lesions of patients with mycosis fungoides. Science 253: 317-320, 1991 9. Jones JF, Shurin S, Abramowsky C, et al: T-cell lymphomas containing Epstein-Barr viral DNA in patients with chronic Epstein-Barr virus infections. N Engl J Med 318: 733-741, 1988[Abstract] 10. Coupland SE, Krause L, Delecluse HJ, et al: Lymphoproliferative lesions of the ocular adnexa: Analysis of 112 cases. Ophthalmology 105: 1430-1441, 1998[Medline] 11. Armitage JO, Weisenburger DD: New approach to classifying non-Hodgkin’s lymphomas: Clinical features of the major histologic subtypes—The Non-Hodgkin’s Lymphoma Classification Project. J Clin Oncol 16: 2780-2795, 1998[Abstract]
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Copyright © 2001 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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