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Journal of Clinical Oncology, Vol 24, No 18 (June 20), 2006: pp. 2960-2961 © 2006 American Society of Clinical Oncology. DOI: 10.1200/JCO.2005.04.3133
Thymoma Associated With Keratoconjunctivitis, Lichen Planus, Hypogammaglobinemia, and Absent Circulating B CellsDepartment of Ophthalmology, Prince of Wales Hospital, Department of Cardiothoracic Surgery, Grantham Hospital, and Departments of Pathology and Medicine, Queen Mary Hospital, Shatin, China A 66-year-old Chinese woman presented with recurrent bilateral scleritis with left corneal opacity (Fig 1A, green arrow). She had a history of chronic lichen planus and tongue ulcers (Fig 1A, black arrow) for 10 years, which were unresponsive to antibiotics, colchicine, and steroids. Left cataract extraction with intraocular lens insertion and corneal graft was performed but failed rapidly due to ischemia, resulting in complete blindness in the left eye. Histology of the native cornea showed extensive fibrosis. Recurrent bilateral anterior segment inflammation (Fig 1B; arrow) persisted without evidence of endophthalmitis. An immunologic work-up showed markedly reduced levels of immunoglobulins ([Ig] IgG, 395 mg/dL; normal 819 to –1,725; IgA, < 7 mg/dL; normal, 70 to 386; IgM, 18 mg/dL; normal, 55 to 307) and low lymphocyte counts (0.46 x 109/L; normal, 1.2 to 3.2). A peripheral blood flow cytometry showed pan-lymphopenia with absent B cells (B cells, 0/µL; normal, 87 to 536; CD4 T cells, 204/µL; normal, 376 to 1,292; CD8 T cells, 179/µL; normal, 216 to 1,100; natural killer cells, 17/µL; normal, 93 to 840). Family screening of her five children was normal. Further extensive tests showed normal neutrophil function, negative HIV/HTLV-1 serology, normal marrow, and negative autoimmune markers. However, a 5 cm thymic mass (Fig 1C; arrow, anterior mediastinal tumor) was revealed on computerized tomography scan and resection showed a Masaoka stage II type B2 thymoma (Fig 1D) with microscopic capsular invasion (Fig 1D; arrow) and vascular permeation by spindle tumor cells admixed with small lymphocytes (Fig 1D; inset). Total thymectomy was performed and her lymphocyte count as well as CD4 and CD8 T cell subsets normalized 1 month after operation, with subsidence of oral and eye inflammation and infective episodes. However, her immunoglobulin, B cell, and natural killer cell levels remained static at 1 year follow-up.
Immunologic complication is a classical paraneoplastic presentation of thymoma, and is attributed to cytokines secreted by the thymic growth and its associated T cells.1 Classical autoimmune associations are myasthenia gravis and pure red cell aplasia.2 Depending on the subset of lymphocytes affected, immunodeficiency may also be a feature. Lichen planus was reported in 1.1% of thymoma cases, accounting for 6% of thymoma related skin manifestations.2 In some cases, panhypogammaglobulinemia and other autoimmune diseases were also present.3-5 However, cicatrizing keratoconjunctivitis is rare.6 Since the tumor is usually slow growing and borderline malignant, the onset of symptoms can be insidious. In contrast, it is life threatening (and in our case sight threatening) and both the tumor and immune defect are curable. Hence, a high index of suspicion and repeated screening is warranted in patients with unusual clinical features that may point to such diseases. Authors' Disclosures of Potential Conflicts of Interest The authors indicated no potential conflicts of interest.
REFERENCES
1. Thomas CR, Wright CD, Loehrer PJ: Thymoma: State of the art. J Clin Oncol 17:2280-2289, 1999 2. Gibson LE, Muller SA: Dermatologic disorders in patients with thymoma. Acta Derm Venereol 67:351-356, 1987[Medline] 3. McManus KG, Allen MS, Trastek VF, et al: Lipothymoma with red cell aplasia, hypogammaglobulinemia, and lichen planus. Ann Thorac Surg 58:1534-1536, 1994[Abstract] 4. Tan RS: Thymoma, acquired hypogammaglobulinaemia, lichen planus, alopecia areata. Proc R Soc Med 67:196-198, 1974[Medline] 5. Ng PP, Ng SK, Chung HH: Pemphigus foliaceus and oral lichen planus in a patient with systemic lupus erythematosus and thymoma. Clin Exp Dermatol 23:181-184, 1998[CrossRef][Medline] 6. Hahn JM, Meisler DM, Lowder CY, et al: Cicatrizing conjunctivitis associated with paraneoplastic lichen planus. Am J Ophthalmol 129:98-99, 2000[CrossRef][Medline]
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Copyright © 2006 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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