AN AUTOPSY CASE OF DIFFUSE MYELOMATOSIS ASSOCIATED WITH SYSTEMIC KAPPA LIGHT CHAIN DEPOSITION DISEASE (LCDD) A Patho‐anatomical, Immunohistochemical and Immunobiochemical Study

Toyoda, Mitsuyasu; Kajita, Akira; Kita, Shigeo; Osamura, Yoshiyuki; Shinoda, Tomotaka

doi:10.1111/j.1440-1827.1988.tb02321.x

Cited by 11 publications

(4 citation statements)

References 8 publications

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“…LCDD has been described surrounding parenchymal cells in numerous organs, most commonly liver, heart, and kidney, but also adrenal glands, spleen, pancreas, muscle, choroid plexus, and nerve 4, 16–19. In LCDD, the light chain is more commonly kappa, while in AL amyloidosis, it is more frequently lambda 4, 5, 18, 20. In both their own series of 13 patients and a review of 43 published cases of amyloid myopathy, Spuler et al8 found that the light chain dysproteinemia in amyloid myopathy is most commonly lambda.…”

Section: Discussionmentioning

confidence: 99%

Expanding the spectrum of monoclonal light chain deposition disease in muscle

Ostrow¹,

Corse²,

Morrison³

et al. 2012

Muscle and Nerve

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Introduction The diagnosis of amyloid myopathy is delayed when monoclonal gammopathies are not detected on initial testing and muscle biopsies are nondiagnostic, and the EMG and symptoms can mimic an inflammatory myopathy. Methods Case report of a patient presenting with severe progressive muscle weakness of unclear etiology despite an extensive workup including two nondiagnostic muscle biopsies. Results Directed by MRI, a third biopsy revealed amyloid angiopathy and noncongophilic kappa light chain deposition in scattered subsarcolemmal rings and perimysial regions. A serum free light chain (FLC) assay revealed a kappa monoclonal gammopathy, which was not detected by multiple immunofixations. Conclusions The spectrum of immunoglobulin deposition in muscle is similar to other organs. It comprises a continuum that includes parenchymal amyloid deposition, amyloid angiopathy, and noncongophilic Light Chain Deposition Disease (LCDD). We recommend including the FLC assay in the routine investigation for monoclonal gammopathies. This case also highlights the value of MRI-guided muscle biopsy.

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Section: Discussionmentioning

confidence: 99%

Expanding the spectrum of monoclonal light chain deposition disease in muscle

Ostrow¹,

Corse²,

Morrison³

et al. 2012

Muscle and Nerve

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“…Although several reports have shown myocardial deposition of immunoglobulin light chains in postmortem examinations, 10,11 the clinical features of LCCM and patient responses to treatment have yet to be elucidated. Left ventricular morphology and transmitral inflow pattern determined by Doppler echocardiography are consistent with diastolic ventricular dysfunction due to restrictive cardiomyopathy.…”

Section: Discussionmentioning

confidence: 99%

Reversible Restrictive Cardiomyopathy Due to Light-Chain Deposition Disease

Nakamura

Satoh

Kowada

et al. 2002

Mayo Clinic Proceedings

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Systemic light-chain deposition due to plasma cell dyscrasias manifests as a form of restrictive cardiomyopathy with diastolic ventricular dysfunction. Although these manifestations are likely to be cardiac amyloidosis, whether these pathological conditions are reversible after treatment of the underlying plasma cell disorders is unknown. To our knowledge, we describe the first patient with cardiac light-chain deposition due to multiple myeloma in whom echocardiographic and biochemical LCCM = light-chain cardiomyopathy; MM = multiple myeloma factors of cardiac function were ameliorated dramatically after remission of this disorder. We emphasize that restrictive cardiomyopathy due to light-chain deposition may be reversible and have a relatively better prognosis after remission of plasma cell dyscrasias.

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“…This disease is characterized by the widespread tissue de position of amorphous non-amyloid material con taining monoclonal light chains. These gammaglobu lin fragments are produced in excess by monoclonal plasma cells that are either neoplastic (multiple mye loma) or hyperplastic (benign monoclonal gammopathy) [12][13][14][15][16]. Light-chain nephropathy should be distinguished from the so-called 'myeloma kidney' due to obstruction of tubules by abnormal casts.…”

Section: Discussionmentioning

confidence: 99%

Pseudo-Nonsecretory Multiple Myeloma with Light Chain Deposition Disease

Matsuzaki

Yoshida²,

Akahoshi³

et al. 1991

Acta Haematol

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A diagnosis of nonsecretory myeloma was established in two patients with anemia and proteinuria on the basis of the suppression of polyclonal immunoglobulins and the increase of plasma cells in the bone marrow. No paraprotein was detected in the serum or concentrated urine of these patients. However, a plaque-forming assay of bone marrow cells showed the secretion of monoclonal immunoglobulin by the myeloma cells. Moreover, renal biopsies from both patients indicated the deposition of monoclonal light chains in the glomerular mesangium and basement membrane, as well as in the tubular basement membrane, a pattern consistent with light-chain deposition disease. These observations suggested that the secreted paraprotein disappeared rapidly as a result of enhanced catabolism or deposition in organs such as the kidney, producing severe proteinuria and chronic renal failure. The plaque-forming assay is a useful technique for the demonstration of this type of nonsecretory myeloma, pseudo-nonsecretory myeloma.

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AN AUTOPSY CASE OF DIFFUSE MYELOMATOSIS ASSOCIATED WITH SYSTEMIC KAPPA LIGHT CHAIN DEPOSITION DISEASE (LCDD) A Patho‐anatomical, Immunohistochemical and Immunobiochemical Study

Cited by 11 publications

References 8 publications

Expanding the spectrum of monoclonal light chain deposition disease in muscle

Expanding the spectrum of monoclonal light chain deposition disease in muscle

Reversible Restrictive Cardiomyopathy Due to Light-Chain Deposition Disease

Pseudo-Nonsecretory Multiple Myeloma with Light Chain Deposition Disease

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