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Interstitial, Diffuse, and Inhalational Lung Disease
Metabolic Diseases and Miscellaneous Conditions
Light-Chain Deposition Disease
Light-Chain Deposition Disease
Takeshi Johkoh, MD, PhD
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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
Light-chain deposition disease (LCDD)
Definitions
Rare condition characterized by systemic extracellular accumulation of immunoglobulin light chains commonly involving kidneys, liver, and heart
Deposition of amorphous nonfibrillary material; does not contain amyloid fibrils
Does not have β-pleated sheet configuration and consequently does not bind Congo red, unlike amyloidosis
IMAGING
Radiographic Findings
CT Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Sheard S et al: Pulmonary light-chain deposition disease: CT and pathology findings in nine patients. Clin Radiol. 70(5):515-22, 2015
Rho L et al: Pulmonary manifestations of light chain deposition disease. Respirology. 14(5):767-70, 2009
Colombat M et al: Pathomechanisms of cyst formation in pulmonary light chain deposition disease. Eur Respir J. 32(5):1399-403, 2008
Bhargava P et al: Pulmonary light chain deposition disease: report of five cases and review of the literature. Am J Surg Pathol. 31(2):267-76, 2007
Colombat M et al: Pulmonary cystic disorder related to light chain deposition disease. Am J Respir Crit Care Med. 173(7):777-80, 2006
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
Light-chain deposition disease (LCDD)
Definitions
Rare condition characterized by systemic extracellular accumulation of immunoglobulin light chains commonly involving kidneys, liver, and heart
Deposition of amorphous nonfibrillary material; does not contain amyloid fibrils
Does not have β-pleated sheet configuration and consequently does not bind Congo red, unlike amyloidosis
IMAGING
Radiographic Findings
CT Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Sheard S et al: Pulmonary light-chain deposition disease: CT and pathology findings in nine patients. Clin Radiol. 70(5):515-22, 2015
Rho L et al: Pulmonary manifestations of light chain deposition disease. Respirology. 14(5):767-70, 2009
Colombat M et al: Pathomechanisms of cyst formation in pulmonary light chain deposition disease. Eur Respir J. 32(5):1399-403, 2008
Bhargava P et al: Pulmonary light chain deposition disease: report of five cases and review of the literature. Am J Surg Pathol. 31(2):267-76, 2007
Colombat M et al: Pulmonary cystic disorder related to light chain deposition disease. Am J Respir Crit Care Med. 173(7):777-80, 2006
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