Reversible Restrictive Cardiomyopathy Due to Light-Chain Deposition Disease

Nakamura, Motoyuki; Satoh, Mamoru; Kowada, Shugo; Satoh, Hidetoshi; Sato, Fumitoshi; Masuda, Tomoyuki; Hiramori, Katsuhiko

doi:10.1016/s0025-6196(11)62336-7

Cited by 27 publications

(7 citation statements)

References 11 publications

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“…In addition to LCDD, 17% of patient had concurrent light chain amyloid (AL) and 13% had cast nephropathy (CN). Of interest, biopsy proven cardiac LCDD was found in 33% of patients, exceeding frequencies of 2‐20% previously reported …”

Section: Resultsmentioning

confidence: 67%

Clinical characteristics and prognostic factors in multiple myeloma patients with light chain deposition disease

et al. 2017

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Light chain deposition disease (LCDD) is characterized by monotypic immunoglobulin depositions which will eventually lead to loss of organ function if left untreated. While the kidney is almost always affected, the presence and degree of LCDD in other organs vary. Ten to thirty percent of LCDD patients have underlying Multiple Myeloma (MM), yet outcome and prognostic markers in this particular patient group are still lacking. Here, we analyzed 69 patients with MM and biopsy proven LCDD and report on renal and extra-renal involvement and its impact on prognosis as well as renal response depending on hematologic response. Coexisting light chain diseases such as AL amyloid and cast nephropathy were found in 30% of patients; those with LCDD and concurrent amyloid tended to have shorter survival. Cardiac involvement by LCDD was seen in one-third of our patients and was associated with shorter overall survival; such patients also had a significantly higher risk of treatment-related mortality (TRM) after stem cell transplant (SCT) compared to LCDD patients without cardiac involvement. This study highlights that MM patients with LCDD present with different clinical features compared to previously reported LCDD cohorts. Rapid initiation of treatment is necessary to prevent progressive renal disease and worse outcome. Coexisting light chain diseases and cardiac involvement are more common than previously reported and confer worse clinical outcome, emphasizing the need for careful patient careful patient evaluation and treatment selection.

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Section: Resultsmentioning

confidence: 67%

Clinical characteristics and prognostic factors in multiple myeloma patients with light chain deposition disease

et al. 2017

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“…6 Affected organs can be the kidneys, liver, lungs, and heart. [7][8][9] The histopathological hallmark of LCDD is light chain deposition that can be detected by immunohistochemistry or electron microscopy. 10 In LHCDD, deposition of light and heavy chains is observed.…”

Section: Sporadic Late Onset Nemaline Myopathy (Slonm)mentioning

confidence: 99%

Sporadic late onset nemaline myopathy and immunoglobulin deposition disease

et al. 2013

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“…Cardiac involvement is very rare and occurs about 20% of LCDD [ 11 ]. Outcome of CIDD and its response to treatment are not well known, with some reports suggesting reversibility [ 12 ] and others showing aggressive disease with no response to treatment [ 13 ].…”

Section: Discussionmentioning

confidence: 99%

A Case of Cardiac Light Chain Deposition Disease in a Patient with Solitary Plasmacytoma

Mohan

Gökden

et al. 2016

Am J Case Rep

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Patient: Male, 31Final Diagnosis: Light chain depsotion diseaseSymptoms: —Medication: —Clinical Procedure: NoneSpecialty: HematologyObjective:Rare co-existance of disease or pathologyBackground:Light chain deposition disease is a systemic disease characterized by deposition of immunoglobin light chains in various organs. Cardiac involvement of light chain deposition disease, also known as cardiac nonamyloidotic immunoglobin deposition disease (CIDD), is a rare clinical entity, where clinical outcome is very variable and best treatment approaches are not well known.Case Report:We present the case of a 31-year-old man with a solitary thoracic plasmacytoma and cardiac light chain deposition disease with evidence of congestive heart failure by echocardiography and cardiac markers. The patient underwent surgical resection of the plasmacytoma followed by systemic therapy with 50% VDT-PACE and then VRD with near-normalization of his heart function. A melphalan-based stem cell transplant is planned in this young patient to achieve the best possible long-term remission.Conclusions:CIDD is a very rare disease, with previous reports showing diverse manifestations with variable outcome. A high level of clinical suspicion should be maintained in such cases and early intervention, as in our patient, can restore cardiac function. There is very little literature on the optimal management of these patients. A combination of surgery and chemotherapy were pursued in our patient with very good results.

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Reversible Restrictive Cardiomyopathy Due to Light-Chain Deposition Disease

Cited by 27 publications

References 11 publications

Clinical characteristics and prognostic factors in multiple myeloma patients with light chain deposition disease

Clinical characteristics and prognostic factors in multiple myeloma patients with light chain deposition disease

Sporadic late onset nemaline myopathy and immunoglobulin deposition disease

A Case of Cardiac Light Chain Deposition Disease in a Patient with Solitary Plasmacytoma

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