Guilain-Barre' Syndrome (GBS) Post Adult Cord Blood Transplantation in a Patient with Chronic Lymphocytic Leukemia (CLL)

Immune mediated demyelinating disease after allogeneic stem cell transplantation is a rare entity with unclear etiology. Acute inflammatory demyelinating polyneuropathy (AIDP) has been reported post related and unrelated allogeneic stem cell transplantation but no such case has been reported post unrelated cord blood transplantation. We hereby present the first case of GBS post double umbilical cord blood transplantation (DUCBT).

A 55 year old male with relapsed refractory CLL received DUCBT with two 5/6 matched cord units, with fludarabine, cytoxan and total body irradiation based intensity conditioning regimen.GVHD prophylaxis was with cyclosporine and mycophenolate. Patient developed grade 4 acute GVHD of the gut with a complete resolution with steroid therapy.7 months post transplantation, patient presented with skin rash and tingling in both feet that progressed rapidly to lower extremity paralysis over the course of 2 days. Physical exam showed maculo-papular rash affecting his upper extremities, upper chest and back area. Neurologic exam was significant for motor weakness in lower extremities 2/5, plantar flexion and knee flexion 3/5. He had loss of deep tendon reflexes in both lower extremities (Achilles and Patellar) and upper extremities (biceps and triceps).Workup revealed normal blood counts, organ function, vitamin B12, folate, TSH level, free cortisol. SPEP and immunfixation were also normal. Magnetic resonance imaging of the CNS was normal. Serology for Lyme disease, Epstein Bar virus (EBV), syphilis, cytomegalo virus (CMV), Hepatitis, HIV, toxoplasma, enterovirus and human herpes virus 6 was negative. Blood tests for autoimmune markers including ANA, acetylcholine esterase and volted calcium channel antibodies were normal. A lumbar puncture showed high protein level of 67mg/dl, 1 nucleated cell/mm3 and normal glucose. CSF was negative for oligoclonal bands, West Nile virus, cryptosporidium, HHV6, HSV 1 and 2, gram stain and cultures. Nerve conduction studies and needle electormyegraphy were suggestive of AIDP.

Based on the above workup, he was diagnosed with GBS and started on therapy with intravenous immunoglobulin at 0.5gm/kg for 4 days and prednisone 1mg/kg daily for the treatment of GVHD. Etiology of GBS was presumed to be related to GVHD as his workup was negative for campylobacter, HIV and CMV. He became ambulatory without assistance in 4 weeks but his weakness symptoms relapsed with prednisone was taper. Prednisone was increased again to 1mg/kg and sirolimus was started. Patient was successfully tapered of prednisone and remains fully ambulatory without assistance or evidence of GVHD on single agent sirolimus 16 months post DUCBT.

This is the first case of autoimmune demyelinating polyneuropathy post DUCBT with association of GVHD that was managed successfully with a combination of intravenous immunoglobulins , steroids and sirolimus.