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Skeletal and Cardiac Myopathy with Acquired Factor X Deficiency in a Patient with Monoclonal Gammopathy of Clinical Significance: A Rare Case Report

delete2026-04-01
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PRE
AI
P
Pragya Garg
S
S Meghana
N
Neeraj Jain
D
Duggal, Lalit *
DOI：10.7860/JCDR/2026/84925.23148delete
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Abstract

Abstract

En 中文
Monoclonal gammopathies include several types of plasma cell proliferative disorders, which might be benign to malignant. Monoclonal Gammopathy of Clinical Significance (MGCS) refers to small plasma cell clones that cause organ damage without meeting criteria for multiple myeloma. The common MGCS syndromes include renal, neurologic and cutaneous, while there is evidence of haematological and multi-organ involvement. MGCS-associated myopathy is a rare presentation, including amyloid light chain amyloidosis and sporadic late-onset nemaline myopathy. MGCS presenting solely with myopathy may lead to misdiagnosis with other common causes of myopathies, delaying management. The condition being rare, it lacks standardised guidelines on management, while data from certain reports suggest treating monoclonal gammopathy for optimal results. This article presents a case of a 57-year-old man with progressive thigh pain, ecchymoses, and proximal muscle weakness for four months. MRI showed muscle oedema, and autoantibody positivity initially suggested immune-mediated myositis. However, recurrent bleeding with deranged coagulation revealed acquired factor X deficiency. Further evaluation demonstrated elevated lambda light chains, abnormal kappa/lambda ratio, and 5% clonal plasma cells on bone marrow biopsy. Cardiac imaging revealed infiltrative cardiomyopathy without amyloidosis. These findings confirmed MGCS with multisystem involvement (myopathy, cardiomyopathy, and coagulopathy). The patient improved on daratumumab, bortezomib, cyclophosphamide, and dexamethasone. This case illustrates the diagnostic challenge of MGCS, which may mimic autoimmune myositis. Awareness of such atypical presentations is crucial, as therapy is guided by organ injury rather than tumour burden. Early recognition and clone-directed treatment are essential to preserve organ function.
Keywords:
Cardiomyopathy
Global longitudinal strain
Monoclonal gammopathy
Myositis-specific antibody

Journal

J
Journal of Clinical and Diagnostic Research
IF:
0.2
Papers:
1.1K
Citations:
1.1W

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