Muscle involvement with pseudohypertrophy in systemic light chain amyloidosis

Autor: Draghici, Mirela, Jercan, Andreea, Badelita, Sorina Nicoleta, Irimia, Ruxandra Maria, Bastian, Alexandra Eugenia, Dobrea, Camelia, Popescu, Monica, Coriu, Daniel
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Zdroj: Medicine
ISSN: 1536-5964
0025-7974
Popis: Rationale: Muscle pseudohypertrophy is a rare manifestation of light chain amyloidosis (AL) amyloidosis. Patient concerns: A 63-year-old woman presented with a 2-year history of progressive asthenia, macroglossia, dysphonia, cachexia, hypotension, paresthesia, and lower limb muscle hypertrophy. Diagnosis: Free serum lambda light chains were increased, and fat pad biopsy demonstrated Congo red-positive deposits. Additionally, electromyography showed a myopathic pattern, whereas muscle biopsy revealed amyloid deposits. A diagnosis of λAL with cardiac, renal, nervous system, and skeletal muscle involvement was established. Interventions and outcomes: The patient received 3 subsequent lines of therapy over the following 23 months, with very slow hematological remission followed by resolution of organ dysfunction. Lessons: Despite its rarity, muscle involvement should be considered in patients diagnosed with AL amyloidosis associated with unexplained muscle hypertrophy or weakness associated with macroglossia or elevated troponin T levels in the absence of clear cardiac involvement.
Databáze: OpenAIRE