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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 7  |  Issue : 1  |  Page : 96-98

Therapeutic Plasma Exchange using Continuous Flow Cell Separator Machine in a Neuromyelitis Optica Patient Outcome and Analysis


Department of Transfusion Medicine, Apollo Multispeciality Hospitals Ltd, Kolkata, India

Date of Submission29-Dec-2021
Date of Decision28-Jan-2022
Date of Acceptance27-Feb-2022
Date of Web Publication29-Apr-2022

Correspondence Address:
Dr. Sourav Mukherjee
Department of Transfusion Medicine, Apollo Multispeciality Hospitals Ltd, Kolkata
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/gjtm.gjtm_106_21

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  Abstract 


Neuromyelitis optica (NMO) is an inflammatory central nervous system syndrome, associated with serum aquaporin-4 immunoglobulin G antibodies. Autoantibodies against aquaporin-4 (AQP4-IgG), the principal water channel on astrocyte foot processes at the blood brain barrier, are pathogenic in NMO. Acute attacks are managed by high-dose intravenous methylprednisolone,if symptoms fail to resolve, plasma exchange is considered. Here , we present a case of NMO spectrum disorder in a 20 year old lady, failed to pharmacotherapy , was managed by plasma exchange using Spectra Optia Cell Separator machine . Detailed plasma exchange protocol, disease progression and outcome was analysed in this study.

Keywords: Anti-aquaporin-4 IgG, Neuromyelitis optica, plasma exchange


How to cite this article:
Mukherjee S, Das SS, Sen S. Therapeutic Plasma Exchange using Continuous Flow Cell Separator Machine in a Neuromyelitis Optica Patient Outcome and Analysis. Glob J Transfus Med 2022;7:96-8

How to cite this URL:
Mukherjee S, Das SS, Sen S. Therapeutic Plasma Exchange using Continuous Flow Cell Separator Machine in a Neuromyelitis Optica Patient Outcome and Analysis. Glob J Transfus Med [serial online] 2022 [cited 2022 May 20];7:96-8. Available from: https://www.gjtmonline.com/text.asp?2022/7/1/96/344326




  Introduction Top


Therapeutic apheresis is an extracorporeal therapy used in the treatment and management of various diseases. The main goal of therapeutic apheresis is (1) to remove pathologic cellular and/or humoral elements from patient's blood, (2) to replace a deficient substance, (3) to modulate cellular functionality, and (4) to collect various autologous nonpathogenic cellular elements for further manipulations.[1] Therapeutic plasma exchange (TPE) is the most commonly performed clinical apheresis procedure where auto and alloantibodies, pathological substances, antibody-mediated disorders, immune complexes, drugs, toxins, etc., that are circulating in the blood are removed and replaced by suitable fluids (plasma, albumin, saline, etc). (The American Society for Apheresis [ASFA]) has published evidence-based clinical guidelines categorizing treatment indications.[2]

Neuromyelitis optica (NMO or Devic's disease) is an inflammatory central nervous system syndrome, associated with serum aquaporin-4 immunoglobulin G antibodies (AQP4-IgG). Acute attacks are usually managed by high-dose intravenous methylprednisolone, 1 g daily for 3–5 days, followed by oral taper and/or intravenous immunoglobulin (IVIG), if symptoms fail to resolve, TPE is considered. NMO falls under category II of ASFA guidelines which indicates that apheresis should be considered as second-line therapy, either as standalone or in conjunction with other modes of treatment.[2]

Here, we present a case of NMO spectrum disorder in a 20-year-old female, nonresponsive to steroids and IVIG therapy, managed by TPE using Spectra Optia Cell Separator machine (Terumo BCT, Inc. Lakewood, Colorado, United States) highlighting apheresis protocol and disease outcome.


  Case Report Top


A 20-year-old female was admitted to local hospital with chief complaints of multiple episodes of vomiting, altered sensorium, poor Glasgow Coma Scale score. The patient was referred to our hospital in an unconscious state, not responding to verbal commands. After clinical evaluation, she was found to have medullary demyelinating lesion, bulbar palsy, longitudinally extensive spinal cord involvement seen in magnetic resonance imaging, hemiparesis noted more on the right side, and lower respiratory tract infection due to aspiration pneumonia along with Type II respiratory failure. High-dose methylprednisolone was started on provisional diagnosis of myelitis and medullary demyelinating lesion. No ocular manifestation was noted but her anti-AQP4 antibody tested positive. Tracheostomy was done and she was put on mechanical ventilation. Based on her clinical condition and seropositivity, NMO spectrum disorder diagnosis was made. Initial treatment started with intravenous steroid, later IVIG was added but she later developed both lower limb weakness along with the right upper limb weakness which did not improve with pharmacotherapy. TPE was planned as lifesaving measure.

Therapeutic plasma exchange protocol

Spectra Optia Cell Separator machine uses continuous flow centrifugation and optical detection technology and one of the most advanced multipurpose cell separator systems used nowadays. Total five procedures were planned on every alternate day. At the initiation of the procedure, power was 4/5 in the right upper limb, 5/5 in the left upper limb, 3/5 in both lower limbs. The patient's body weight was 55 kg and height 156 cm. Her plasma volume (PV) was calculated by using formula PV = total blood volume (TBV) × (1-hematocrit). The PV calculation is usually performed on the assumption that the patient's TBV is 70 mL/kg body weight for males and 65 mL/kg body weight for females.[3]

The first TPE procedure was done by removing 1 PV. Replacement fluids used were 20% of human albumin infused with normal saline and fresh frozen plasma (FFP). Intravenous 10% of calcium gluconate was infused simultaneously to counteract citrate toxicity due to anticoagulant use. The first procedure was limited to 1 PV due to poor health condition of the patient.

The next four procedures were done on every alternate day with 1.3 volume plasma removal and replaced by FFP and human albumin. FFP was used considering its low cost compared to albumin. The blood was sent for investigating serum calcium, total protein, albumin, and complete hemogram after each procedure. All the procedures were completed uneventfully.

The patient showed signs of improvement after two procedures. There were progressive improvements in muscle power of all the four limbs. Her general condition and sensorium had improved gradually. She weaned off from ventilation after the fifth procedure and started walking 1 week after the procedure. On the 16th day of admission, she was discharged and advised to visit neurology outdoor after 2 weeks. On her first visit, she was found clinically stable with normal laboratory parameters. Clinical outcome of the patient is summarized in [Table 1].
Table 1: Clinical outcome of the patient

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  Discussion Top


NMO, also called Devic's syndrome, is the diagnosis applied to a group of patients with attacks of CNS demyelination that affect the optic nerves and/or the spinal cord, often severely. Anti-AQP4 appears to be a useful diagnostic marker for NMO, in particular, it may be useful in distinguishing NMO from other neurological disorders with ocular and spinal involvement at presentation.[4] Autoantibodies against AQP4-IgG, the principal water channel on astrocyte foot processes at the blood‒brain barrier, are pathognomonic of the disease. IgG binding to AQP4 leads to complement-dependent astrocyte cytotoxicity, leukocyte infiltration, cytokine release, and blood‒brain barrier disruption, resulting in oligodendrocyte death, myelin loss, and neuron death.[5] AQP4-IgG is found in approximately 70% of patients. Acute attacks are managed by high-dose intravenous corticosteroid followed by oral taper and if symptoms fail to resolve, TPE is added.[6]

Here, in this case, the female presented with the first episode of acute attack with no past history, intravenous steroids, and IVIG started after diagnosis but failed to show any improvement after 4 days of pharmacotherapy. The previous studies had suggested that five or six plasma exchange sessions are required to substantially reduce the blood antibody level of IgG by 85%.[7] TPE was performed using a single-volume plasma exchange with continuous flow cell separator (Spectra Optia) machines by femoral or central line access using 12-French double-lumen dialysis catheter. Anticoagulation with citrate was used systematically. Hemogram, serum electrolytes, total protein, and albumin were monitored daily.

We evaluated the effect of TPE on the degree of recovery from NMO. Both subjective and objective clinical responses to TPE were noted. Steroids have only immunomodulatory effect, and they do not remove the antibodies; hence, significant improvement after steroid may not be noticed in all patients.[8],[9] TPE is found to be more effective method in removing circulating antibody and better clinical outcome.


  Conclusion Top


This case study suggests that TPE can be useful in acute phase of NMO spectrum disorder. TPE can be performed safely in children and has been shown to be effective. Careful assessment of the patients and expertise in TPE is essential to optimize therapy and minimize adverse consequences. Our results showed that TPE is a safe and effective alternative to IVIG in patients who cannot afford it.

Patient consent

Authors certify that they have patient consent for the study and that the study has been conducted in line with declaration of Helsinki and cleared by the institutional review board. The documents are attached with patient case file.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Andrzejewski Jr C, Devenport R.D. Technical manual, American Association of Blood Banks. Therapeutic apheresis. Vol.25. Bethesda. USA: 2017. P641.  Back to cited text no. 1
    
2.
Padmanabhan A, Connelly-Smith L, Aqui N, Balogun RA, Klingel R, Meyer E, et al. Guidelines on the use of therapeutic apheresis in clinical practice – Evidence-based approach from the writing committee of the American society for apheresis: The eighth special issue. J Clin Apher 2019;34:171-354.  Back to cited text no. 2
    
3.
Nadler SB, Hidalgo JH, Bloch T. Prediction of blood volume in normal human adults. Surgery 1962;51:224-32.  Back to cited text no. 3
    
4.
Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG. The spectrum of neuromyelitis optica. Lancet Neurol 2007;6:805-15.  Back to cited text no. 4
    
5.
Sherman E, Han MH. Acute and chronic management of neuromyelitis optica spectrum disorder. Curr Treat Options Neurol 2015;17:48.  Back to cited text no. 5
    
6.
Watanabe S, Nakashima I, Misu T, Miyazawa I, Shiga Y, Fujihara K, et al. Therapeutic efficacy of plasma exchange in NMO-IgG-positive patients with neuromyelitis optica. Mult Scler 2007;13:128-32.  Back to cited text no. 6
    
7.
Kim SH, Kim W, Huh SY, Lee KY, Jung IJ, Kim HJ. Clinical efficacy of plasmapheresis in patients with neuromyelitis optica spectrum disorder and effects on circulating anti-aquaporin-4 antibody levels. J Clin Neurol 2013;9:36-42.  Back to cited text no. 7
    
8.
Khatri BO, Kramer J, Dukic M, Palencia M, Verre W. Maintenance plasma exchange therapy for steroid-refractory neuromyelitis optica. J Clin Apher 2012;27:183-92.  Back to cited text no. 8
    
9.
Abboud H, Petrak A, Mealy M, Sasidharan S, Siddique L, Levy M, et al. Treatment of acute relapses in neuromyelitisoptica: Steroids alone versus steroids plus plasma exchange. Mult Scler 2016;22:185-92.  Back to cited text no. 9
    



 
 
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