Potential advantage of Therapeutic Plasma Exchange over Intravenous Immunoglobulin in children with axonal variant of Guillain-Barré syndrome: A report of six paediatric cases - 21/11/24

Doi : 10.1016/j.tracli.2024.11.005

Joyisa Deb ^a , Gita Negi ^b , Aswin k Mohan ^c,^⁎ , Indar Kumar Sharawat ^d , Pradip Banerjee ^e , Deepali Chauhan ^b , Daljit Kaur ^b , Ashish Jain ^b
^a Department of Transfusion Medicine, All India Institute of Medical Sciences Guwahati, India
^b Department of Transfusion Medicine, All India Institute of Medical Sciences Rishikesh, India
^c Department of Transfusion Medicine, All India Institute of Medical Sciences Bibinagar, India
^d Department of Paediatrics, All India Institute of Medical Sciences Rishikesh, India
^e Department of Transfusion Medicine, Mahamana Pandit Madan Mohan Malviya Cancer Centre, Varanasi, India

^⁎Corresponding author at: Department of Transfusion Medicine, All India Institute of Medical Sciences Bibinagar, Telangana- 508126, IndiaDepartment of Transfusion MedicineAll India Institute of Medical Sciences BibinagarTelangana- 508126India

Sous presse. Manuscrit accepté. Disponible en ligne depuis le Thursday 21 November 2024

Highlights

•	Guillain-Barre Syndrome (GBS), a heterogeneous condition with several distinct variants of acute, immune-mediated polyneuropathies includes a rare variant, Acute motor axonal neuropathy (AMAN) which is associated with acute high mortality and up to 20% long term disability features.
•	AMAN variant of GBS with positive ganglioside antibodies are frequently associated with IVIG failure which is in stark contrast to the more common AIDP variant of GBS.
•	Therapeutic Plasma Exchange (TPE) is an established treatment option in GBS patients, and is used interchangeably with Intravenous Immunoglobulins (IVIG) as demonstrated in various Randomised Controlled Trials.
•	Since both the treatment options are equally efficacious, TPE may be considered an early option for the AMAN type of GBS, especially in resource constraint countries like India.

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Abstract

Guillain-Barre syndrome (GBS) is a disease entity described in literature since 1859. It is associated with various etiological, clinical and immunological factors with prognostic predictive value. Both Intravenous immunoglobulin (IVIG) and Therapeutic Plasma Exchange (TPE) have been regarded as the first-line treatment for GBS. Certain diagnostic tools help us in early identification of GBS subtypes that may aid clinical management. Here, we have discussed six paediatric cases of GBS of Acute Motor Axonal Neuropathy (AMAN) subtype that were considered for TPE. 5 out of 6 patients were eventually weaned from mechanical ventilation and discharged from the hospital. This study emphasizes the role of TPE in management of severe IVIG refractory GBS with axonal involvement that can be beneficial to the patient. TPE may be considered early in GBS cases with axonal involvement.

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Keywords : Guillain-Barré syndrome (GBS), Acute motor axonal neuropathy (AMAN), Therapeutic Plasma Exchange (TPE), Intravenous Immunoglobulins (IVIG), IVIG Resistance