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 (antigen level HLA-A, HLA-B and allele level HLA-DRB1) with fludarabine, cytoxan and total body irradiation based reduced intensity conditioning regimen. Graft versus host disease (GVHD) prophylaxis was with cyclosporine and mycophenolate. Early post transplant course was complicated by grade 4 acute GVHD of the gut with a complete resolution with steroid therapy and successful taper of all immunosuppression by day 180. 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 macula-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. Serum electrophoresis and immunfixation was also normal. Magnetic resonance imaging of the central nervous system showed mild neural foramina narrowing at the L4-L5 level. 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 (anti-nuclear antibody)ANA, acetylcholine esterase and volted calcium channel antibodies were normal. A lumbar puncture was performed and showed a high protein level of 67mg/dl, 1 nucleated cell/mm3 and normal glucose level. Cerebrospinal fluid was negative for oligoclonal bands, West Nile virus, cryptosporidium, HHV6, herpes virus 1 and 2, gram stain and cultures. Nerve conduction studies and needle electormyegraphy was suggestive of acute demyelinating polyneuropathy.

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 improved significantly over the next 4 weeks and became ambulatory without assistance but his weakness symptoms relapsed as his prednisone was tapered. 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 13 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.