Large Volume Leukapheresis in a Patient With Meniere's Disease

Abstract

Meniere's Disease (MD) is a progressive, debilitating disease of the inner ear, characterized by aural pressure, tinnitus, hearing loss and vertigo. Meniere's disease has a late onset, with most cases occurring after 40 years of age [1]. MD is caused by endolymphatic hydrops of the inner ear and is typically managed conservatively with a low sodium diet (1500–2300 mg Na++ per day), diuretics, and abundant fluid intake to lower both endolymphatic pressure and plasma antidiuretic hormone levels. Antihistamines (meclizine, betahistamine, and diphenhydramine) are often prescribed to reduce the motion sickness associated with vertigo [1]. Patients with severe refractory symptoms may require intratympanic injections (steroids and gentamicin) or surgery [1]. We recently encountered a patient with MD and multiple myeloma who was referred for autologous peripheral blood hematopoietic progenitor cell collection (AHPCC). Per institutional IRB, informed consent is waived.

The patient was diagnosed with vertigo and MD nearly 20 years ago. He was managed conservatively with a low sodium diet (1500 mg Na++/day) and diuretics thrice weekly (triamterene-hydrochlorothiazide 25 mg, MWF). His symptoms were well controlled and limited to intermittent episodes of vertigo, aural fullness, and “feeling off” usually associated with high dietary sodium intake.

The patient's MD history raised concerns for large volume leukapheresis (LVL) due to the volume of acid citrate dextrose anticoagulant (ACDA) administered over the course of AHPCC. ACDA contains trisodium citrate dihydrate (C₆H₅Na₃O₇:2H₂0), which averages 510 mg Na++ per 100 mL of ACDA [2]. Adult APHCC patients undergoing a standard 3 blood volume LVL may receive 1200 to 1600 mL ACDA and > 6000 mg Na++. In addition, we prophylactically administer CaCl₂ in saline (0.1 mEq Ca++/mL saline) for citrate prophylaxis, further adding to the sodium burden. We discussed using heparin-ACDA for anticoagulation, which would decrease both the ACDA administered and minimize citrate toxicity with lower CaCl₂ infusion [3]. Unfortunately, heparin-ACDA for LVL using the Spectra Optia (TerumoBCT, Lakewood, CO) is not validated at our institution and was not considered a viable option by the apheresis service.

In a literature review, we found no cases discussing LVL or apheresis in patients with MD. After informal consultation with bone marrow transplant and otolaryngology, a plan was formulated for AHPCC and his subsequent admission for HPC transplant. The patient was mobilized with G-CSF and plerixafor. During leukapheresis, he was prescribed prophylactic meclizine (25 mg, BID) as well as a benzodiazepine (lorazepam 0.5 mg, as needed), which can also reduce vertigo by increasing the inhibitory neurotransmitter GABA (gamma aminobutyric acid) [1, 4]. In addition, his diuretic was increased to twice daily starting 3 days prior to apheresis and through his transplant.

On his first HPCC, he received 1203 mL ACDA (6350 mg Na++) and 323 mL saline, for a total of 7550 mg Na++. Because he only collected 2.4 × 10⁶ CD34/kg, he returned the next day for a second procedure. On day 2, he reported aural fullness and tinnitus in the left ear but no vertigo or nausea. Due to falling peripheral CD34 counts (17 CD34/μL), the process time was increased to four donor blood volumes. He received 1652 mL ACDA (8530 mg Na++) and 552 mL saline, for a total of 13 500 mg Na++. The patient collected 2 × 10⁶ CD34/kg, for a final yield of 4.4 × 10⁶ CD34/kg. The patient did not experience any worsening of MD symptoms after his second procedure and was admitted for transplant 1 week later.

A review of his transplant course was unremarkable. During the first week of transplant, he continued twice daily diuretics, restricted sodium intake, and meclizine as needed. His diuretics were subsequently discontinued due to hypokalemia (3.2 mmol/L) and hyponatremia (129 mmol/L). He experienced chemotherapy-associated nausea but no MD-related tinnitus or vertigo. He was discharged on transplant day +15 and continues to do well.

To our knowledge, this is the first report on the challenges and management of LVL in patients with MD. This required coordination between apheresis, bone marrow transplant, and otolaryngology to construct a therapy plan to minimize a flare of MD symptoms using antihistamines and diuretics. In addition to MD, the large sodium loads associated with ACDA may impact other sensitive patient populations with renal disease, heart, and/or liver failure [5-7].

The author declares no conflicts of interest.