OFTALMOPATIA BILATERALIS AS UNUSUAL PRESENTATION OF A LONG TERM LOCALIZED HODGKIN’S LYMPHOMA

Lymphomas predominantly affect the lymph nodes but can present at unusual sites, which are termedas extranodal lymphomas. Ocular adnexal involvement with lymphoma accounts for 1%–2% of non-Hodgkinlymphoma and 5%–15% of extranodal lymphoma. Ocular adnexa infiltration by Hodgkin lymphoma is usually amanifestation of advanced or relapsing disease. Up to date only few cases of Hodgkin lymphoma with the primaryinvolvement of the retrobulbar muscles are reported.Here, we report an unusual case of primary ocular Hodgkin lymphoma which was for a period of 10 yearslocalized and miss diagnosed until the involvement of the peripheral lymph nodes with Hodgkin’s disease. Ourpatient is 59-year-old male with ten-year history, from 2000 year of marked bilateral periorbital edema, bilateralconjunctival swelling, exophthalmos and diplopia. The initial ocular examination (orbital ultrasoundexamination and MRI) showed bilateral diffuse infiltrative lesions of retro-bulbar muscles, retro-bulbar tumormass and exophthalmos. During the period of 10 years the patient was assigned as isolated ocular form ofMorbus Graves or Oftalmopatia bilateralis and periodically treated with corticosteroids and orbital radiotherapy(2006), which resulted with short-term moderate improvement of periorbital edema.In June 2011 he presented at our department with bilateral cervical lymphadenopathy, periorbital edema,conjunctival erythema and exophthalmos, complaining of night sweating and scratching. On examination he hadbilateral cervical lymphadenopathy not fixed to overlying skin or to underlying structures. The largest lymphnode measured 3 cm. Rest of the systemic examination was unremarkable except marked cardiac arrhythmia.Lymph node biopsy from the cervical mass revealed Hodgkin lymphoma-mixed cellular type. CT of the chestand abdomen showed enlarged mediastinal and abdominal lymph nodes. The patient was staged as stage IIIBand treated with systemic chemotherapy. He received 8 cycles of ABVD regimen and responded completely totreatment with resolution of all signs and symptoms. The periorbital edema disappeared completely after he firstdose of the first cycle of ABVD.In May 2012, the bilateral cervical lymph nodes and periorbital edema appeared again. The PET scan revealed moderate increased metabolic activity at the cervical and mediastinal lymph nodes and active retro-bulbar mass with increased activity in the bulbar muscles. No abnormal metabolic activity was noted in the rest of the body. The patient received another 8 cycles of ABVD chemotherapy in combination with Rituximab. Control PET scan 3 months later showed complete resolution of the increased activity in comparison with the previous PET scan. The patient was in complete remission until February 2015 when he succumbed due to the acute hearth failure.Experience from our case shows that diagnosis of the ocular lymphoma was made secondary after the achievement of complete remission and complete resolution of the retro-bulbar tumor mass after administrationof the systemic chemotherapy. Although primary ocular Hodgkin lymphoma are very rare and difficult for diagnosis, always, in all cases of orbital tumor mass a high degree of suspicions is mandatory and microsurgicalapproach to the orbit is advise to be essential diagnostic tool.