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Light Chain Deposition Disease: A Morphological Case Report.

Hristo Popov¹, George S Stoyanov^2,1, Peter Ghenev¹

¹General and Clinical Pathology/Forensic Medicine and Deontology, Medical University of Varna, Varna, BGR.

|July 29, 2022

Summary

Light chain deposition disease (LCDD) involves kidney damage from abnormal protein buildup. This case highlights LCDD in a patient with multiple myeloma, leading to severe complications and death.

Keywords:

case report kidney light chain deposition disease monoclonal immunoglobulin deposition disease multiple myeloma nephropathology

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Area of Science:

Nephrology
Hematology
Pathology

Background:

Light chain deposition disease (LCDD) is a rare plasma cell dyscrasia characterized by the deposition of monoclonal immunoglobulin light chains.
Kidney involvement is universal in LCDD, often presenting as renal failure, nephrotic syndrome, hematuria, and proteinuria.
LCDD is frequently associated with lymphoproliferative disorders, particularly multiple myeloma, although idiopathic cases exist.

Observation:

A 72-year-old female presented with symptoms consistent with LCDD.
Kidney biopsy revealed nodular sclerosis with specific staining characteristics (PAS-positive, Congo red-negative, silver weakly-positive, Masson's trichrome-positive) and ribbon-like changes.
Light chain deposits were identified in the tubular basement membranes.

Findings:

The patient was diagnosed with LCDD and concurrently with multiple myeloma of the thoracic vertebrae.
Despite initiating myeloma treatment, the patient developed a severe lower limb infection requiring amputation.
The patient ultimately succumbed to sepsis following the amputation.

Implications:

This case underscores the severe renal and systemic manifestations of LCDD, particularly when associated with multiple myeloma.
The diagnostic findings from kidney biopsy are crucial for identifying LCDD.
The case illustrates the potential for rapid disease progression and fatal outcomes despite treatment, emphasizing the need for early diagnosis and management of both LCDD and associated plasma cell neoplasms.