Stanford School of Medicine

Surgical Pathology Criteria
http://surgpathcriteria.stanford.edu/

 use browser back button to return

Monoclonal Light Chain and Heavy Chain Deposition Diseases

Definition

  • Plasma cell neoplasm that secretes immunoglobulin that deposits in tissues and causes tissue damage but does not form an amyloid-type beta-pleated sheet structure

Alternate/Historical Names

  • Randall disease

Diagnostic Criteria

Dita Gratzinger MD PhD

Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Initial posting/updates : 9/1/07, 4/18/10

Supplemental studies

Microscopy

  • Congo Red negative
  • Electron microscopy shows granular, non-fibrillar deposits
  • Immnohistochemistry, in situ hybridization or flow cytometry of bone marrow often demonstrates clonal plasma cells
    • Not demonstrable at site of tissue deposition
  • Immunostaining of renal biopsy may demonstrate kappa light chains

Useful Laboratory Tests

  • Serum or urine protein electrophoresis, immunofixation, light chain quantification
  • Quantitation and typing of monoclonal immunoglobulin / light chain
  • Serum free light chain analysis may be required to demonstrate clonal light chains
  • These studies may be used to
    • Establish presence of a monoclonal plasma cell population
    • Quantitation helps subtype the plasma cell dyscrasia (i.e. >3g/dL serum monoclonal protein is a major criterion for myeloma)
    • Track disease burden over time

Differential Diagnosis

Amyloidoses and Deposition Diseases

Primary (AL) Secondary (AA) Familial (AF) Hemodialysis Associated (β2) Light/Heavy Chain Deposition Disease
M component in 80% (usually lambda) No M component No M component No M component M component often present (usually kappa)
Clonal plasma cells No clonal population No clonal population No clonal population Clonal plasma cells may be demonstrable in marrow
Clonal light chain in deposits may be demonstrable No clonal light chain deposits No clonal light chain deposits No clonal light chain deposits Clonal light/heavy chain deposits may be demonstrable
Congo Red positive Congo Red positive Congo Red positive Congo Red negative Congo Red negative
Heavy chain deposition disease is very rare, so the following must be ruled out
  • Lymphoplasmacytic lymphoma with IgG deposition
  • SLL/CLL witih IgM deposition
  • Extranodal marginal zone lymphoma with IgA deposition (IPSID)
  • Clinical

    • Usually associated with MGUS or overt plasma cell myeloma
    • 85% have monoclonal serum immunoglobulin (M component)
      • Usually kappa
    • Symptomatic deposition frequently occurs in
      • Kidneys
      • Liver
      • Heart
      • Joints
    • Poor prognosis

    Grading / Staging / Report

    • Grading is not applicable
    • Staging is applicable only if myeloma is present

    The pathology report should contain the following information:

    • Results of supplementary studies if performed

     

    Lists

    Plasma cell neoplasms / Immunosecretory disorders (WHO 2008)

    Bibliography

    • Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW . WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, International Agency for Research on Cancer, Lyon, 2008
    • Warnke RA, Weiss LM, Chan JKC, Cleary ML, Dorfman RF . Tumors of the Lymph Nodes and Spleen, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 14, 1995
    • Ansari-Lari MA, Ali SZ Fine-needle aspiration of abdominal fat pad for amyloid detection: a clinically useful test? Diagn Cytopathol. 2004 Mar;30(3):178-81.
    • Giorgadze TA, Shiina N, Baloch ZW, Tomaszewski JE, Gupta PK. Improved detection of amyloid in fat pad aspiration: an evaluation of Congo red stain by fluorescent microscopy.  Diagn Cytopathol. 2004 Nov;31(5):300-6.
    • Halloush RA, Lavrovskaya E, Mody DR, Lager D, Truong L. Diagnosis and typing of systemic amyloidosis: The role of abdominal fat pad fine needle aspiration biopsy. Cytojournal. 2010 Jan 15;6:24.
    Printed from Surgical Pathology Criteria: http://surgpathcriteria.stanford.edu/
    © 2005  Stanford University School of Medicine