Guillain Barre Syndrome: A Late Complication of Autologous Stem Cell Transplant?.

Abstract A 54 year old female with a history of multiple myeloma (MM) complained of progressive ascending weakness starting in her lower extremities. She received an autologous stem cell transplant (ASCT) 7 months prior and engrafted from a 2nd ASCT 39 days prior. Now she noted progressive bilateral ascending lower extremity weakness and paresthesias which began several weeks after an upper respiratory viral syndrome. Her MM was in remission. MRI of the brain and spinal cord ruled out any compressive lesions. Neurology consultants demonstrated hyporeflexia and impaired strength, greater in lower extremities than in upper. Forced vital capacity (FVC) was impaired. CBC and metabolic panel were normal. Blood and urine cultures were negative and CSF unremarkable. Viral studies for Epstein-Barr virus (EBV), West Nile Virus (WNV), herpes simplex virus (HSV), human herpes virus 6 (HHV6), cytomegalovirus (CMV), rhinovirus, adenovirus, influenza, HIV, and hepatitis B and C were all negative. The Guillain Barre Syndrome (GBS) was diagnosed. The patient received intravenous immunoglobulin (IVIG) at a dose of 0.5 g/kg per day for 5 days. Her strength, reflexes, and FVC drastically improved. She was discharged within 2 weeks of admission. GBS is an acute idiopathic demyelinating polyneuropathy characterized by progressive muscle weakness and areflexia. It is a diagnosis of exclusion and the majority of patients have antecedent respiratory or gastrointestinal infection. Rapid diagnosis and treatment are crucial as 30% of patients may have acute respiratory decompensation. GBS has been associated with infectious agents like campylobacter, EBV, HSV, WNV, and CMV. GBS is relatively rare, with less than 3 cases per 100,000 in the general population. To the authors' knowledge, there are 6 published cases of GBS as a complication after ASCT. 5 of the 6 patients presented within 31 days of ASCT, while one patient presented at 47 days. 5 of the 6 patients recovered with treatment of GBS, although one patient decompensated and expired despite intervention. This data suggests that most patients with GBS after ASCT will present early (within 1 month) and have a reasonable response rate to treatment. Our patient presented with GBS more than 7 months after her 1st ASCT and greater than 1 month after her 2nd ASCT, and responded well to IVIG. It has been proposed that T-lymphocyte dysfunction or humoral autoimmune mechanisms may be involved in the development of GBS after ASCT. Immunosuppression and the conditioning regimen are probably less significant in ASCT than in allogeneic transplant. Regardless of the underlying mechanism, clinicians should be aware of the possibility of GBS and the appropriate treatment with IVIG or plasmapheresis. Our data at the Cell and Gene Therapy Center reports 1 case of GBS after 351 ASCTs, a higher incidence than the general population. According to the Center for International Blood and Marrow Transplant, there were 6 cases of GBS among 12,695 patients who underwent ASCT between 1994 and 1998, also a higher incidence than the general population. More data at other centers should be collected and further investigation of the underlying mechanism of GBS may advance our understanding of immunologic complications of ASCT.