Elsevier

Human Pathology

Volume 34, Issue 3, March 2003, Pages 270-277
Human Pathology

Original Contributions
Light chain crystal deposition as a manifestation of plasma cell dyscrasias: The role of immunoelectron microscopy☆

https://doi.org/10.1053/hupa.2003.27Get rights and content

Abstract

Light chain crystal deposition disease is a rare and poorly characterized entity that can be confused with a number of different conditions, depending on where the disease process is manifested. The present study explored the role of ultrastructural immunogold labeling in the diagnosis of this condition. Seven cases of light chain crystal deposition (kappa light chain-related) are reported. Immunohistochemistry and immunofluorescence techniques play a rather limited role in the evaluation of these cases, as a result of the inability to detect monoclonal kappa light chains in association with the crystalline structures or high background staining. Ultrastructural labeling is the method of choice to fully characterize these cases. However, surgical pathologists must learn to recognize the findings associated with this condition to avoid misdiagnosis. If the diagnosis is at least suspected, then a complete hematologic workup may identify the underlying plasma cell dyscrasia. It must be emphasized that in some patients the plasma cell dyscrasia does not become clinically manifested until years after the diagnosis of light chain crystal deposition. Hum Pathol 34:270-277. Copyright 2003 Elsevier Inc. All rights reserved.

Section snippets

Materials and methods

Seven cases with tissue crystal deposition were obtained from the Louisiana University Health Sciences Center and Baylor College of Medicine. Surgical specimens from theses patients were submitted for pathological diagnosis. All specimens contained histologically identifiable crystals identifiable on hematoxylin and eosin-stained sections, albeit difficult to detect in 2 of the cases (Table 1).

. Summary of Clinical Findings of the 7 Cases With Crystalline Inclusions

CaseAge (years)SexInitial

Clinical findings

The 7 patients reported on herein included 4 men and 3 women, with a mean age at diagnosis of 57 years (range, 30 to 67 years). The initial clinical presentations in these patients included nonnephrotic-range proteinuria (cases 1, 5, and 7), Fanconi's syndrome (cases 5 and 7), pleural effusion (case 6), eyelid swelling and orbital mass (cases 2 and 3), and neck mass (case 4). Biopsy of the pertinent tissue revealed crystal deposition in each case, albeit in different locations. In 1 case (case

Discussion

We have presented 7 cases of kappa light chain-related crystal formation in patients with plasma cell dyscrasias, including 6 cases of multiple myeloma and 1 case of Waldenström's macroglobulinemia. Crystal formation occurred in either histiocytes in soft tissues or parenchymal cells, including renal proximal tubular, thyroid follicular, or gallbladder surface epithelial cells. The initial clinical presentation depended on the site of crystal formation. In 1 case it was an incidental finding.

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