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Lymphoproliferative disorders associated with congenital immunodeficiencies

K S Elenitoba-Johnson1, E S Jaffe

  • 1Laboratory of Pathology, National Cancer Institute, Bethesda, MD 20892-1500, USA.

Seminars in Diagnostic Pathology
|February 1, 1997
PubMed

Insights

Congenital immunodeficiencies increase the risk of lymphoproliferative disorders (LPD), often influenced by Epstein-Barr virus (EBV). Evaluation requires integrating clinical, histopathologic, and molecular data for accurate diagnosis and prognosis.

Area of Science:

  • Hematopathology
  • Immunology
  • Molecular Biology

Background:

  • Congenital immunodeficiencies (CIDs) are frequently associated with lymphoproliferative disorders (LPD).
  • Key CIDs linked to LPD include Wiskott-Aldrich syndrome (WAS), common variable immunodeficiency (CVID), ataxia telangiectasia (AT), severe combined immunodeficiency (SCID), X-linked lymphoproliferative disorder (XLP), and hyper-IgM syndrome.
  • Epstein-Barr virus (EBV) significantly contributes to LPD development in several CID types.

Purpose of the Study:

  • To review clinicopathologic, immunologic, and molecular features of CIDs and associated LPD.
  • To present cases from the Third Slide Workshop of the Society of Hematopathology.
  • To highlight the interplay between specific immunodeficiencies, risk factors, and LPD patterns.

Main Methods:

  • Review of clinicopathologic, immunologic, and molecular biological data.
  • Analysis of cases presented at a specialized workshop.
  • Integration of clinical, histopathologic, immunophenotypic, and molecular findings.

Main Results:

  • Ataxia telangiectasia (AT) involves DNA repair defects, with lymphomas/leukemias appearing earlier than in sporadic cases.
  • EBV is a critical factor in LPD associated with WAS, CVID, SCID, and XLP.
  • Monoclonality in LPD does not always predict an aggressive clinical course, as seen in some WAS cases.

Conclusions:

  • A comprehensive approach combining clinical, histopathologic, and molecular data is essential for evaluating LPD in patients with CIDs.
  • Understanding the specific risk factors and viral associations for each CID is crucial for managing LPD.
  • Immunophenotypic and molecular clonality findings require careful interpretation in the context of clinical behavior.

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