Abstract
Here, we report a case of Burkitt’s lymphoma in an HIV-positive patient presenting with features suggestive of Vogt-Koyanagi-Harada disease (VKHD), which in retrospect was likely a misdiagnosis. We hope to describe a rare presentation of lymphoma in order to prevent misdiagnosis and promote early recognition. The patient was a 25-year-old male who initially presented with right eye pain and blurry vision. He was found to have bilateral serous retinal detachments and was diagnosed with VKHD and started on prednisone. He stopped taking the prednisone, and his vision worsened. He then developed right eye ptosis, restricted eye movements, nausea, vomiting, headache, dysphagia, tongue deviation, and slurred speech. MRI showed diffuse cranial nerve enhancement. He was found to be positive for HIV and Hepatitis A with CD4 count of 41. Lumbar puncture showed WBC 83 (94% lymphocytes), RBC 1460, glucose 62, and protein 195, with Epstein-Barr virus (EBV) positivity and negative cytology. Gd1a antibody was positive (72). He underwent empiric treatment with IV solumedrol for possible VKHD exacerbation, followed by empiric intravenous immune globulin (IVIG) for possible acute inflammatory demyelinating polyneuropathy (AIDP). He subsequently developed diffuse limb weakness and loss of reflexes, and he was treated with plasma exchange (PLEX). He demonstrated minimal response to treatment. Electromyography (EMG) was unrevealing, and the MRI of the cervical and lumbar spine showed diffuse nerve root thickening and enhancement. He underwent an esophagogastroduodenoscopy (EGD) for continued dysphagia, and the biopsy was positive for an aggressive B-cell lymphoma strongly favoring Burkitt’s lymphoma. VKHD is a rare condition diagnosed based on retinal exam findings. Few cases of lymphoma report findings suggestive of VKHD. This is a rare case of lymphoma initially presenting with these retinal findings. Understanding this potential presentation of lymphoma is essential for early diagnosis and treatment and for optimizing patient outcomes.
Highlights
Vogt-Koyanagi-Harada disease (VKHD) is a systemic granulomatous autoimmune disease process that preferentially invades tissues rich in melanocytes and is characterized by panuveitis, often with associated neurological and cutaneous manifestations
There have been a few cases reported in the literature describing concurrent diagnoses of VKHD and lymphoma, with some skepticism raised regarding the accuracy of the VKHD diagnosis
Hashida et al described a case of a 69-year-old male with prior history of gastric diffuse intermediate B-cell large-type malignant lymphoma in remission following chemotherapy and total gastrectomy, presenting with sudden bilateral loss of visual acuity [7]. He was found to have moderate anterior chamber cells with granulomatous keratic precipitates on the retrocornea in both eyes and a circumferential posterior synechia in the left eye as well as bilateral papilledema and serous retinal detachment. He had elevated levels of soluble IL-2 receptor (1954 U/mL), which has been identified as a tumor marker for malignant lymphoma
Summary
Vogt-Koyanagi-Harada disease (VKHD) is a systemic granulomatous autoimmune disease process that preferentially invades tissues rich in melanocytes and is characterized by panuveitis, often with associated neurological and cutaneous manifestations. A month later, he was seen by a retinal specialist and underwent fluorescein angiography that revealed bilateral serous retinal detachments He was diagnosed with VKHD and was started on prednisone 60 mg daily, with a plan to taper by 10 mg over the course of four weeks. He was initially compliant with his steroid regimen, but due to symptoms of anxiety and jitteriness, he stopped taking it after three weeks His vision worsened, and a few days later, he developed right eye ptosis, nausea, vomiting, headache, and difficulty swallowing liquids. Serum and CSF paraneoplastic panel including LGI1, CASPR2, AMPAR 1/2, GABAR, and NMDAR were negative He was started on sulfamethoxazole/trimethoprim, fluconazole, and azithromycin prophylaxis for his low CD4 count. His course was complicated by febrile neutropenia, septic shock, and appendicitis requiring appendectomy, and a decision was made to withdraw care and transfer to hospice
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