Dataset Information

Immunophenotyping of peripheral blood cells allows to discriminate MIS-C and Kawasaki disease.

ABSTRACT:

Background

The pathogenesis of the novel described multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease (KD) is still debated as it is not clear if they are the same or different nosological entities. However, for both the diseases a rapid and unequivocal diagnosis is mandatory to start the therapy before the onset of severe complications. In this study, we aimed to evaluate the white cell populations in MIS-C and KD as potential markers to discriminate between the two diseases.

Methods

We studied white cell populations by flow cytometry in 46 MIS-C and 28 KD patients in comparison to 70 age-matched healthy children.

Results

MIS-C patients had a significant lymphopenia that involved both B and T populations while KD patients showed a significant neutrophilia and thrombocythemia. Granulocyte/lymphocyte ratio helped to diagnose both MIS-C and KD with a high diagnostic sensitivity, while a multivariate analysis of granulocyte and T lymphocyte number contributed to discriminate between the two diseases.

Conclusions

The relevant lymphopenia observed in MIS-C patients suggests that the disease would be a post-infectious sequel of COVID-19 immunologically amplified by a massive cytokine release, while the significant neutrophilia and thrombocythemia observed in KD confirmed that the disorder has the genesis of a systemic vasculitis. The analysis of a panel of circulating cells may help to early diagnose and to discriminate between the two diseases.

Supplementary information

The online version contains supplementary material available at 10.1186/s41231-022-00128-2.

SUBMITTER: Castaldo A

PROVIDER: S-EPMC9440857 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature

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Publications

Immunophenotyping of peripheral blood cells allows to discriminate MIS-C and Kawasaki disease.

Castaldo Alice A D'Anna Carolina C Gelzo Monica M Giannattasio Antonietta A Maglione Marco M Muzzica Stefania S Raia Maddalena M Scalia Giulia G Tripodi Lorella L Castaldo Giuseppe G Tipo Vincenzo V Grieco Domenico D Grieco Michela M

Translational medicine communications 20220904 1

<h4>Background</h4>The pathogenesis of the novel described multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease (KD) is still debated as it is not clear if they are the same or different nosological entities. However, for both the diseases a rapid and unequivocal diagnosis is mandatory to start the therapy before the onset of severe complications. In this study, we aimed to evaluate the white cell populations in MIS-C and KD as potential markers to discriminate between the ...[more]

PMID: 36093039

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Project description:PurposeKawasaki disease is a systemic vasculitis, and its etiology and pathogenesis are still not clear. Our study was undertaken to investigate the characteristics of the activation of B cells in the peripheral blood of Kawasaki disease (KD) patients and evidence of stimulation by superantigens.Materials and methodsBlood samples were obtained from three patients (2 males, 1 female) with KD, who were admitted to our Hospital, Seoul, Korea. The mean age was 1.2 years. Distribution of B cells was studied in the acute and subacute phases of KD patients. From the RNA of B cells, we obtained complementary DNA (cDNA) and performed polymerase chain reaction (PCR). To determine the oligoclonal expansion of immunoglobulin M (IgM) V(H) family, we cloned and sequenced the PCR products from each group and analyzed DNA.ResultsIn the peripheral blood of acute phase patients, T cells were significantly decreased (p < 0.05), whereas B cells were significantly increased (p < 0.05). When the first PCR was done on the B cell chains, V(H)1 to V(H)6 were all found to be expressed. The number of micro gene clones obtained from 3 patients was 312, and they belonged to V(H)3, V(H)4 and V(H)5 family. M99686 germ line was most frequently used and the next most frequently used, were X92224/J, L21967 and L21964. A similar order was seen in patients. Among the clones, 20 sets of clones showed the same base sequence and this was frequent between V(H)2 and V(H)5. There was one set, which showed almost the same base sequence between different patients, and the homology was 99.5%. Twenty sets of clones that had the same base sequence showed high similarity to the germ line (94 - 100%). Among these, the clones that utilized the M99686 germ line were 4 sets which were most frequent. The 3-dimensional structure of one of these clones showed typical beta, sheet structure of immunoglobulin chains.ConclusionThe IgM transcripts expressed by the B cells in the peripheral blood of KD patients in the acute phase of the disease clearly showed an oligoclonal expansion, suggesting that KD is caused not by stimulation of a superantigen, but rather by a conventional antigen.

Dataset Information

Immunophenotyping of peripheral blood cells allows to discriminate MIS-C and Kawasaki disease.

Background

Methods

Results

Conclusions

Supplementary information

Publications

Immunophenotyping of peripheral blood cells allows to discriminate MIS-C and Kawasaki disease.

Similar Datasets

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