Pediatr Allergy Respir Dis.  2002 Dec;12(4):299-314.

Clonal Expansion of Peripheral B Cells in Kawasaki Disease

• Lee JS
• Park IH
• Shin JS
• Kim DS

Abstract

PURPOSE
The etiology and pathogenesis of Kawasaki disease(KD) remains unknown and KD is a potentially fatal acute systemic vasculitis of childhood. Recently, expansions of T cells expressing TCR(T cell receptor) Vbeta2 and TCR Vbeta8 chanins have been reported, and this suggests the involvement of a superantigen in the pathogenesis of KD. With the stimulation with superantigen, polyclonal B cell activation can be observed. However, there was no report on the polyclonal or oligoclonal B cell activation in the peripheral blood of the KD. We investigated clonal expansion to search for the evidence of clonality of the peripheral B cell in KD and for the evidence of the superantigen involvement in KD.
METHODS
Peripheral mononuclear cells of acute and subacute phase of KD were stained for immunophenotype. Total RNA was extracted from peripheral mononuclear cells of the patients and cDNA was prepared. Primary PCR and second round PCR were done for analysing the CDR3 region. Cloning and sequencing were done, and VH3 family gene sequences were compared with immunoglobulin germline sequence.
RESULTS
The percentage of B cells in KD increased significantly(P<0.05) in both acute and subacute phases compared to those of normal controls. Random utilization of diverse VH family genes was observed in the acute phase, and no specific expansion of VH family was detected. An increase in B cells expressing the VH3 family was seen during acute phase. Analysis of CDR3 size profile showed that various prominent bands appeared in acute phase, and some disappearing in the convalescent, while other new bands were developed in convalescent phase. IgM VH transcripts showed that DH6 and JH4 were used most commonly which showed the evidence of oligoclonality. However, DNA sequences of CDR3s of VH3 showed no dominant clones.
CONCLUSION
These data suggest KD may be caused by conventional antigens rather than superantigen. DNA sequences of all 6 VH families should be searched further.

Keyword

Kawasaki disease; B cell; Clonal expansion; Superantigen

MeSH Terms

B-Lymphocytes*
Base Sequence
Clone Cells
Cloning, Organism
DNA, Complementary
Humans
Immunoglobulin M
Immunoglobulins
Mucocutaneous Lymph Node Syndrome*
Polymerase Chain Reaction
RNA
Systemic Vasculitis
T-Lymphocytes
DNA, Complementary
Immunoglobulin M
Immunoglobulins
RNA