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VDJ-Seq: Deep Sequencing Analysis of Rearranged Immunoglobulin Heavy Chain Gene to Reveal Clonal Evolution Patterns of B Cell Lymphoma
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VDJ-Seq: Deep Sequencing Analysis of Rearranged Immunoglobulin Heavy Chain Gene to Reveal Clonal Evolution Patterns of B Cell Lymphoma

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Single clonal origin of neoplastic B-cells with different immunoglobulin light chains in a patient with Richter's

K Miyamura1, H Osada, T Yamauchi

  • 1Department of Internal Medicine, Japanese Red Cross Nagoya First Hospital.

Cancer
|July 1, 1990
PubMed

Insights

Richter's syndrome, a simultaneous diagnosis of chronic lymphocytic leukemia (CLL) and diffuse large cell lymphoma (DLC), may originate from a single clone. This finding challenges the assumption of biclonality based solely on differing surface immunoglobulins in these lymphoid malignancies.

Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • Richter's syndrome is a rare but serious complication of chronic lymphocytic leukemia (CLL).
  • It involves the transformation of CLL into a more aggressive lymphoma, typically diffuse large B-cell lymphoma (DLBCL).
  • The clonal origin of DLBCL in Richter's syndrome remains a subject of debate, with theories suggesting either a single clone evolving or two independent clones arising.

Observation:

  • A 71-year-old male patient presented with simultaneous chronic lymphocytic leukemia (CLL) and diffuse large cell lymphoma (DLC).
  • The CLL exhibited mu lambda surface immunoglobulin (sIg), while the DLC showed mu kappa sIg.
  • Despite differing sIg, both malignancies displayed identical immunoglobulin heavy chain (IgH) gene rearrangement patterns and shared surface markers (CD5+, CD19+, CD20+).

Findings:

  • The identical IgH gene rearrangement and surface marker profiles strongly suggest a single clonal origin for both the CLL and DLC in this patient.
  • The differing sIg (mu lambda vs. mu kappa) between the CLL and DLC components does not necessarily indicate biclonality.
  • This case highlights the complexity in determining clonality in Richter's syndrome.

Implications:

  • The findings challenge the conventional understanding of Richter's syndrome potentially arising from biclonal origins.
  • Further research involving nucleotide sequencing of rearranged Ig genes in more Richter's syndrome cases is crucial for definitive conclusions on clonality.
  • This study underscores the need for advanced molecular techniques to accurately diagnose and understand the pathogenesis of Richter's syndrome.

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