Vol 9, No 3 (2018)
Case report
Published online: 2018-10-17

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Fluctuating Guillain-Barré syndrome in AL systemic amyloidosis as a progression indicator?

Anna Suska12, Jacek Matyszkiewicz, Artur Jurczyszyn13
Hematologia 2018;9(3):239-244.

Abstract

Primary systemic light-chain amyloidosis (AL) that accounts for 4/5 of all cases can be manifested as a neuropathy. In some cases, neuropathy is a prodromal symptom, present even before the diagnosis or preceding the progression of the disease. Typically, it is symmetrical and progressive, involving the sensitive rather than motor function in the distal parts of the extremities. It can be accompanied by the paresis and autonomic dysfunction. Some atypical presentations of amyloid neuropathy are also mentioned in the literature including asymmetric polyradiculopathy, cranial nerve (III, V, VII) palsies or multiple mononeuropathies. We present a case study of a 58-year-old male patient with AL kappa-light chain amyloidosis of the kidneys, bone marrow and heart, treated in frontline with VD (bortezmib, dexamethasone) and CyBorD (cyclophosphamide, bortezomib, dexamethasone) and with high-dose melphalan followed by autologous hematopoietic stem cell transplantation (auto-HSCT), who developed Guillain-Barré syndrome not responding to standard treatment (intravenous immunoglobulin and plasmapheresis). Severe symptoms of axonal demyelination proceeded the progression of amyloidosis. The improvement in neurological status was obtained by high dose intravenously corticosteroid therapy. Currently, the patient takes dexamethasone as monotherapy – until now without both hematological and neurological progression. Considering the overall clinical picture, the fluctuating Guillain-Barré syndrome may be a prodromal symptom of the amyloidosis progression.

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Hematology in Clinical Practice