1125 - Harlequin Syndrome in Metastatic Non-Hodgkin Lymphoma: A Case Report

Harlequin Syndrome in the setting of B-cell lymphoma.

Case Description: This case describes a 67-year-old woman who presented with progressive back pain. MRI spine found a large, left-sided paraspinal mass extending from the C7-T1 levels and T4-T5 posterior vertebral body involvement. Imaging also revealed T5 compression fracture, T4/5 epidural extension and cord compression, and secondary thoracic levoscoliosis. Biopsy of the paraspinal mass confirmed metastatic diffuse large B-cell lymphoma (DLBCL). She received systemic chemotherapy and intrathecal methotrexate. Concurrently, she was referred to cancer rehabilitation medicine for evaluation of her back pain, which improved with bracing and physical therapy. However, she also reported intermittent exertional flushing unilaterally on the right face with absence contralaterally. This was attributed to Harlequin Syndrome (HS). Though she had resolution of her back pain, made excellent functional gains, and has no evidence of cancer recurrence or progression, she continues to have intermittent HS symptoms.

Discussions: This case highlights a unique neurologic manifestation of HS in lymphoma. HS is precipitated by interruption of sympathetic chain fibers in the low-cervical or thoracic spine, leading to an abnormal lack of sympathetic tone on the affected side. Multiple factors may cause this patient’s sympathetic chain impairment including the left-sided mass, T5 compression fracture, T4-T5 epidural extension, or thoracic levoscoliosis. Her persistent HS may be due to delayed, or relatively slow, healing of the nerves in the sympathetic chain following cancer treatment. Though HS is not life-threatening, it can cause significant psychosocial distress to the patient. Recognition of this process can direct rehabilitation management and patient conversations, providing reassurance. It can direct the rehab provider to seek out other neurological compromise in this patient population.

Conclusions: Neurological symptoms can occur in patients with lymphoma. HS is a very rare manifestation of lymphoma metastasis and should be recognized as a possibility when evaluating these patients.