IgD Multiple Myeloma Paraproteinemia as a Cause of Myositis

A 48-years old man was diagnosed an IgD-k multiple myeloma (MM) at age 38 years for which he successfully underwent chemotherapy and bone marrow transplant. He then developed a graft-versus-host disease (GVHD) whose manifestations included, three years later, a polymyositis, diagnosed at muscle biop...

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Main Authors: I. Colombo, M. E. Fruguglietti, L. Napoli, M. Sciacco, E. Tagliaferri, A. Della Volpe, V. Crugnola, N. Bresolin, M. Moggio, A. Prelle
Format: Article
Language:English
Published: Wiley 2010-01-01
Series:Neurology Research International
Online Access:http://dx.doi.org/10.1155/2010/808474
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author I. Colombo
M. E. Fruguglietti
L. Napoli
M. Sciacco
E. Tagliaferri
A. Della Volpe
V. Crugnola
N. Bresolin
M. Moggio
A. Prelle
author_facet I. Colombo
M. E. Fruguglietti
L. Napoli
M. Sciacco
E. Tagliaferri
A. Della Volpe
V. Crugnola
N. Bresolin
M. Moggio
A. Prelle
author_sort I. Colombo
collection DOAJ
description A 48-years old man was diagnosed an IgD-k multiple myeloma (MM) at age 38 years for which he successfully underwent chemotherapy and bone marrow transplant. He then developed a graft-versus-host disease (GVHD) whose manifestations included, three years later, a polymyositis, diagnosed at muscle biopsy and successfully treated with steroids. Few months after polymyositis remission, myeloma relapsed and the patient was treated with thalidomide for six years with good remission. Soon after thalidomide suspension, MM relapsed again and the patient came to our observation for a new onset of neuromuscular symptoms. He underwent both muscle and peripheral nerve biopsy to discriminate between myositis (paraproteinemia versus GVHD), amyloidosis, and thalidomide toxicity. The first muscle biopsy showed an inflammatory pattern with necrotic fibres, macrophagical invasion (CD68 positive), rare interstitial cellular infiltrates (CD8 positive and CD4 negative), widespread anti-HLA positivity and negative antiMAC. The second muscle biopsy showed the same inflammatory pattern plus an involvement of blood vessels. Direct immunofluorescence for IgD showed diffuse positivity along the sarcolemmal in both muscle biopsies. Sural nerve biopsy demonstrated both demyelinating and axonal aspects with no inflammatory infiltrates, but positivity for HLA and MAC. Congo Red was negative in both skeletal muscle and peripheral nerve.
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spelling doaj-art-a64cdf038d8d4228bc09ca461e0573ad2025-02-03T01:00:54ZengWileyNeurology Research International2090-18522090-18602010-01-01201010.1155/2010/808474808474IgD Multiple Myeloma Paraproteinemia as a Cause of MyositisI. Colombo0M. E. Fruguglietti1L. Napoli2M. Sciacco3E. Tagliaferri4A. Della Volpe5V. Crugnola6N. Bresolin7M. Moggio8A. Prelle9Centro Dino Ferrari, Fondazione IRCCS Cá Granda Ospedale Maggiore Policlinico, 20122 Milano, ItalyDipartimento di Neuroscienze, Azienda Ospedaliera Niguarda Cá Granda, 20122 Milano, ItalyCentro Dino Ferrari, Fondazione IRCCS Cá Granda Ospedale Maggiore Policlinico, 20122 Milano, ItalyCentro Dino Ferrari, Fondazione IRCCS Cá Granda Ospedale Maggiore Policlinico, 20122 Milano, ItalyEmatologia, Fondazione IRCCS Cá Granda Ospedale Maggiore Policlinico, 20122 Milano, ItalyEmatologia, Fondazione IRCCS Cá Granda Ospedale Maggiore Policlinico, 20122 Milano, ItalyCentro Dino Ferrari, Fondazione IRCCS Cá Granda Ospedale Maggiore Policlinico, 20122 Milano, ItalyCentro Dino Ferrari, Fondazione IRCCS Cá Granda Ospedale Maggiore Policlinico, 20122 Milano, ItalyCentro Dino Ferrari, Fondazione IRCCS Cá Granda Ospedale Maggiore Policlinico, 20122 Milano, ItalyDipartimento di Neuroscienze, Azienda Ospedaliera Fatebenefratelli e Oftalmico, 20122 Milano, ItalyA 48-years old man was diagnosed an IgD-k multiple myeloma (MM) at age 38 years for which he successfully underwent chemotherapy and bone marrow transplant. He then developed a graft-versus-host disease (GVHD) whose manifestations included, three years later, a polymyositis, diagnosed at muscle biopsy and successfully treated with steroids. Few months after polymyositis remission, myeloma relapsed and the patient was treated with thalidomide for six years with good remission. Soon after thalidomide suspension, MM relapsed again and the patient came to our observation for a new onset of neuromuscular symptoms. He underwent both muscle and peripheral nerve biopsy to discriminate between myositis (paraproteinemia versus GVHD), amyloidosis, and thalidomide toxicity. The first muscle biopsy showed an inflammatory pattern with necrotic fibres, macrophagical invasion (CD68 positive), rare interstitial cellular infiltrates (CD8 positive and CD4 negative), widespread anti-HLA positivity and negative antiMAC. The second muscle biopsy showed the same inflammatory pattern plus an involvement of blood vessels. Direct immunofluorescence for IgD showed diffuse positivity along the sarcolemmal in both muscle biopsies. Sural nerve biopsy demonstrated both demyelinating and axonal aspects with no inflammatory infiltrates, but positivity for HLA and MAC. Congo Red was negative in both skeletal muscle and peripheral nerve.http://dx.doi.org/10.1155/2010/808474
spellingShingle I. Colombo
M. E. Fruguglietti
L. Napoli
M. Sciacco
E. Tagliaferri
A. Della Volpe
V. Crugnola
N. Bresolin
M. Moggio
A. Prelle
IgD Multiple Myeloma Paraproteinemia as a Cause of Myositis
Neurology Research International
title IgD Multiple Myeloma Paraproteinemia as a Cause of Myositis
title_full IgD Multiple Myeloma Paraproteinemia as a Cause of Myositis
title_fullStr IgD Multiple Myeloma Paraproteinemia as a Cause of Myositis
title_full_unstemmed IgD Multiple Myeloma Paraproteinemia as a Cause of Myositis
title_short IgD Multiple Myeloma Paraproteinemia as a Cause of Myositis
title_sort igd multiple myeloma paraproteinemia as a cause of myositis
url http://dx.doi.org/10.1155/2010/808474
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