The patient’s history dates back to 2006 when she had multiple skin lesions that were diagnosed as PTCL and subsequently excised. Submandibular and parotid lymph nodes were palpable, but there was no swelling of the liver or spleen. Blood tests were negative for adult T-cell leukemia antibody and human immunodeficiency virus (HIV) antibody and soluble interleukin-2 receptor was within normal range. No other lesion was detected by gallium scintigraphy or systemic computed tomography. A bone marrow trephine biopsy did not show the involvement of lymphoma. Immunostaining revealed the cells to be positive for CD3 and CD5, but negative for CD30, CD10, and CD75. Based on the findings, the patient was classified as PTCL Stage IVB according to the new World Health Organization classification.5
As the patient was chemorefractory against initial treatment with combined cyclophosphamide, deoxorubicin, vincristine, and prednisone, she underwent allogenic stem cell transplantation from a matched non-sibling donor in March 2007 after salvage treatment with cyclophosphamide, cytosine arabinoside, etoposide, and dexamethasone. Transplant-conditioning therapy was cyclophosphamide, 2900 mg/day for three days, plus total body irradiation for three days (total 12 Gy). Graft-versus-host disease prophylaxis was provided with cyclosporine A and prednisolone. Cyclosporin levels were maintained between 300 and 400 μg/L until tapering was initiated. Initial dose of prednisolone was 17.5 mg per oral.
In January 2008, the patient presented to our department to check for any ocular manifestations of the graft-versus-host disease. At that time, significant superficial punctate keratopathy was noted in both eyes. Systemic skin and eyelids were macroscopically normal. Systemic lymph nodes were not palpable, and there was no swelling of the liver or spleen. The superficial punctate keratopathy significantly improved with eyedrops containing hyaluronan.
On July 11, 2008, the patient noted an induration in the skin of her left lower eyelid (). Conjunctival congestion and pain were present. Upon diagnosis of an acute chalazion, conservative management was used. However, the condition worsened rapidly, and swelling of the lower eyelid became remarkable ().
Figure 1 A) Patient with chalazion-like induration of the left lower eyelid on July 11, 2008. B) High magnification of the lower eyelid with chalazion-like presentation. C) After one month, induration of the lower eyelid became rapidly enlarged. Note a diffuse, (more ...)
On August 25, 2009, the patient underwent an excisional biopsy of the palpebral lesion after informed consent. A retention cyst in chalazion was not detected during the intraoperative microscopic examination. Pathological examination demonstrated that medium-sized lymphoma cells with round nuclei () had infiltrated subcutaneously. Immunostaining revealed the cells to be positive for CD3 (). Considering these results, the palpebral lesion was diagnosed as recurrence of PTCL.
Figure 2 A) Light micrograph of hematoxylin and eosin stained eyelid biopsy specimen. Lymphoma cells were infiltrated into the subcutaneous tissue. P, palpebral skin; Bar = 200 μm. B) High magnification of the lymphoma cells infiltrated in the orbicular (more ...)
As the lesion was limited to the left lower eyelid, 25.2 Gy electron irradiation was applied as a radical treatment. Globe shielding was used to help prevent radiation retinopathy. The eyelid tumor was dramatically improved after irradiation (, ). The prognosis was satisfactory without recurrence at 10 months after the last irradiation.