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© 2003 American Society for Clinical Oncology
Unusual Locations of Involvement by MalignanciesCASE 4. BILATERAL HYPOPYON HERALDING CNS RELAPSE OF CUTANEOUS NATURAL KILLER CELL LYMPHOMAQueen Mary Hospital and the Chinese University of Hong Kong, Hong Kong Eye Hospital, Hong Kong
A 78-year-old woman presented with a 7-cm left elbow ulcer. A biopsy showed severe necrosis and extensive infiltration with large lymphoma cells, with foci of angioinvasion. The cells were positive for CD3
Apart from B- and T-lineage diseases, NK cell lymphoma is now recognized as a distinct lymphoma subclass.1 The most common entity, previously known as lethal midline granuloma, is characterized by nasal predilection, Epstein Barr virus, and Asian ancestry, as well as local angioinvasion and destruction. Extranasal nasal-type disease may also occur in skin, testes, and gut and portends a grave prognosis.2 Because of anatomic proximity, reactive inflammatory ocular symptoms may be present in up to 25% of cases.3 Hypopyon resulting from direct lymphoma involvement has occasionally been reported in primary and relapsed lymphoma, and an orbital sanctuary mechanism may be involved.4,5 To our knowledge, this is the first report of hypopyon complicating NK cell lymphoma and also the first report of direct ocular and CNS invasion for this disease entity. Lymphomatous uveitis is a classic ophthalmologic masquerade syndrome and enters the differential diagnosis of all bilateral refractory uveal lymphocytic inflammations.6 However, the distinction of clonal from reactive T or B cells in aqueous or vitreous aspirates can be difficult.7 In our case setting, however, the infiltration of NK cells is definitely pathologic. The association between intraocular and cerebral lymphoma is well known, and involvement of either site should prompt examination and prophylaxis of the other area.8 Unfortunately, both long-term survival and visual prognosis remain poor.
REFERENCES 1. Jaffe ES, Rulfkider E, Stein H, et al: Mature T cell and NK cell neoplasms, in Jaffe ES, Harris NL, Stein W, Vardiman JW (eds) Tumours of Haematopoietic and Lymphoid Tissues, World Health Organization Classfication of Tumours 2001. Lyon, France, International Agency for Research on Cancer Press, 2001, pp 190–199
2. Chan JK, Sin VC, Wong KF, et al: Nonnasal lymphoma expressing the natural killer cell marker CD56: A clinicopathologic study of 49 cases of an uncommon aggressive neoplasm. Blood 89:4501–4513, 1997 3. Hon C, Kwok AK, Shek TW, et al: Vision-threatening complications of nasal T/NK lymphoma. Am J Ophthalmol 134:406–410, 2002[CrossRef][Medline] 4. Jan NA, Einzig AI, Suhrland MJ, et al: Non-Hodgkin lymphoma in acquired immunodeficiency syndrome manifesting as bilateral hypopyon. J Clin Oncol 22:82–83, 1999[Medline] 5. Verity DH, Graham EM, Carr R, et al: Hypopyon uveitis and iris nodules in non-Hodgkin’s lymphoma: Ocular relapse during systemic remission. Clin Oncol (R Coll Radiol) 12:292–294, 2000[CrossRef] 6. Rothova A, Ooijman F, Kerkhoff F, et al: Uveitis masquerade syndromes. Ophthalmology 108:386–399, 2001[CrossRef][Medline]
7. White VA, Gascoyne RD, Paton KE: Use of the polymerase chain reaction to detect B- and T-cell gene rearrangements in vitreous specimens from patients with intraocular lymphoma. Arch Ophthalmol 117:761–765, 1999 8. Peterson K, Gordon KB, Heinemann MH, et al: The clinical spectrum of ocular lymphoma. Cancer 72:843–849, 1993[CrossRef][Medline]
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Copyright © 2003 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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, CD56, and Epstein Barr virus–expressed RNA, compatible with stage IE nasal-type natural killer (NK) cell lymphoma. Marked regression was obtained with monthly combination therapy cyclophosphamide, adriamycin, etoposide, prednisolone, cytosine-arabinoside, bleomycin, vincristine, and methotrexate (Promace-Cytabom) and local radiotherapy. Three months after initial presentation, the patient presented with bilateral blurred vision. Physical examination showed bilateral hypopyon (Fig 1A
