Unique paraneoplastic syndrome associated with cll

Abstract Chronic lymphocytic leukemia is a hematologic malignancy characterized by overproduction of monoclonal B cells. Most people diagnosed with CLL do not immediately undergo treatment due to treatment guidelines. There are few cases of paraneoplastic syndrome associated CLL. This case is unique in that it is a paraneoplastic KLHL 11 antibody associated CLL. The patient is a 68-year-old female who originally presented to the hospital with abdominal pain and upon further imaging was found have retroperitoneal lymphadenopathy. After further studies including flow cytometry, she was diagnosed with CLL. She failed trials of Pembrolizumab, ibrutinib, and Obinutuzumab due to side effects. The patient then started experiencing excessive fatigue, bilateral temporal headache, nausea, vomiting, vertigo, tremor of bilateral upper extremities, and head bobbing. Her brain imaging was negative for acute abnormalities. Lumbar puncture was positive for CLL cells and the paraneoplastic panel came back positive with KLHL 11 antibodies. The patient’s symptoms continued to persist and her tremor and dysmetria continued to worsen. Due to the positive paraneoplastic panel, the patient was started on intravenous immunoglobulin but did not experience improvement. She was then trialed on intravenous methylprednisolone and still experienced no relief. She then underwent plasma exchange. Finally, she was given rituximab infusions with slight improvement. Her imaging remained negative for signs of leptomeningeal enhancement. Given the patient’s symptoms, negative brain imaging, and positive paraneoplastic panel, it was determined that the patient developed an irreversible paraneoplastic syndrome related to her CLL versus possible leptomeningeal disease. There are a few cases of paraneoplastic syndrome associated CLL involving paraneoplastic phemphigus, hypercalcemia, and nephrotic syndrome. This case is unique in that her symptoms presented with an ataxic syndrome with positive KLHL11 antibodies. It is important to be aware that some cases of CLL can present with unique paraneoplastic syndromes.