Hypereosinophilic syndrome with elevated IgG4 and T-cell clonality: A report of two cases
In some cases, higher IgG4 levels are accompanied by increased circulating IgE, higher eosinophil counts, and various autoantibodies. Among these cases, IgG4-related disease (IgG4-RD) is one of the most frequently diagnosed conditions. This study reported two particularly complex and rare cases of h...
Saved in:
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
De Gruyter
2025-07-01
|
| Series: | Open Life Sciences |
| Subjects: | |
| Online Access: | https://doi.org/10.1515/biol-2025-1138 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1850065746416107520 |
|---|---|
| author | Huang Meichun Wang Limin Li Xiuxiu Lu Ying |
| author_facet | Huang Meichun Wang Limin Li Xiuxiu Lu Ying |
| author_sort | Huang Meichun |
| collection | DOAJ |
| description | In some cases, higher IgG4 levels are accompanied by increased circulating IgE, higher eosinophil counts, and various autoantibodies. Among these cases, IgG4-related disease (IgG4-RD) is one of the most frequently diagnosed conditions. This study reported two particularly complex and rare cases of hypereosinophilic syndrome (HES) associated with elevated IgG4 and T-cell clonality. The first case involved T-cell clonality complicated by HES and IgG4-RD, presenting with clinical features resembling eosinophilic granulomatosis with polyangiitis (EGPA). Laboratory findings showed a serum IgG4 concentration of 8.74 g/L, an IgG4/IgG ratio of 40.58%, and positive results for P-anti-neutrophil cytoplasmic antibodies (ANCA) and myeloperoxidase-ANCA. Renal biopsy findings were consistent with IgG4-related interstitial nephritis. However, routine hematological testing revealed a markedly elevated eosinophil count of 14.75 × 109/L and eosinophilic infiltration in both lymph nodes and kidney tissue. Furthermore, monoclonal rearrangements of the T-cell receptor gamma and delta genes were identified. The second patient was ultimately diagnosed with HES with elevated IgG4 and T-cell clonality, with an elevated IgG4 concentration of 2.458 g/L and an eosinophil count of 14.75 × 109/L. In conclusion, in cases presenting with elevated IgG4 levels and hypereosinophilia, further pathological and genetic evaluations may be essential to guide appropriate and timely treatment, improving patient prognosis. |
| format | Article |
| id | doaj-art-a059d840155746d1a6ee1ed6bb6e0d1d |
| institution | DOAJ |
| issn | 2391-5412 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | De Gruyter |
| record_format | Article |
| series | Open Life Sciences |
| spelling | doaj-art-a059d840155746d1a6ee1ed6bb6e0d1d2025-08-20T02:48:55ZengDe GruyterOpen Life Sciences2391-54122025-07-0120112720842210.1515/biol-2025-1138Hypereosinophilic syndrome with elevated IgG4 and T-cell clonality: A report of two casesHuang Meichun0Wang Limin1Li Xiuxiu2Lu Ying3Renal Department, Tongde Hospital of Zhejiang Province, No. 234 Gucui Road, Hangzhou, 310012, ChinaRenal Department, Tongde Hospital of Zhejiang Province, No. 234 Gucui Road, Hangzhou, 310012, ChinaRenal Department, Tongde Hospital of Zhejiang Province, No. 234 Gucui Road, Hangzhou, 310012, ChinaRenal Department, Tongde Hospital of Zhejiang Province, No. 234 Gucui Road, Hangzhou, 310012, ChinaIn some cases, higher IgG4 levels are accompanied by increased circulating IgE, higher eosinophil counts, and various autoantibodies. Among these cases, IgG4-related disease (IgG4-RD) is one of the most frequently diagnosed conditions. This study reported two particularly complex and rare cases of hypereosinophilic syndrome (HES) associated with elevated IgG4 and T-cell clonality. The first case involved T-cell clonality complicated by HES and IgG4-RD, presenting with clinical features resembling eosinophilic granulomatosis with polyangiitis (EGPA). Laboratory findings showed a serum IgG4 concentration of 8.74 g/L, an IgG4/IgG ratio of 40.58%, and positive results for P-anti-neutrophil cytoplasmic antibodies (ANCA) and myeloperoxidase-ANCA. Renal biopsy findings were consistent with IgG4-related interstitial nephritis. However, routine hematological testing revealed a markedly elevated eosinophil count of 14.75 × 109/L and eosinophilic infiltration in both lymph nodes and kidney tissue. Furthermore, monoclonal rearrangements of the T-cell receptor gamma and delta genes were identified. The second patient was ultimately diagnosed with HES with elevated IgG4 and T-cell clonality, with an elevated IgG4 concentration of 2.458 g/L and an eosinophil count of 14.75 × 109/L. In conclusion, in cases presenting with elevated IgG4 levels and hypereosinophilia, further pathological and genetic evaluations may be essential to guide appropriate and timely treatment, improving patient prognosis.https://doi.org/10.1515/biol-2025-1138lymphocyte variant hypereosinophilic syndromeigg4igg4-related diseaseshypereosinophiliaacute kidney injury |
| spellingShingle | Huang Meichun Wang Limin Li Xiuxiu Lu Ying Hypereosinophilic syndrome with elevated IgG4 and T-cell clonality: A report of two cases Open Life Sciences lymphocyte variant hypereosinophilic syndrome igg4 igg4-related diseases hypereosinophilia acute kidney injury |
| title | Hypereosinophilic syndrome with elevated IgG4 and T-cell clonality: A report of two cases |
| title_full | Hypereosinophilic syndrome with elevated IgG4 and T-cell clonality: A report of two cases |
| title_fullStr | Hypereosinophilic syndrome with elevated IgG4 and T-cell clonality: A report of two cases |
| title_full_unstemmed | Hypereosinophilic syndrome with elevated IgG4 and T-cell clonality: A report of two cases |
| title_short | Hypereosinophilic syndrome with elevated IgG4 and T-cell clonality: A report of two cases |
| title_sort | hypereosinophilic syndrome with elevated igg4 and t cell clonality a report of two cases |
| topic | lymphocyte variant hypereosinophilic syndrome igg4 igg4-related diseases hypereosinophilia acute kidney injury |
| url | https://doi.org/10.1515/biol-2025-1138 |
| work_keys_str_mv | AT huangmeichun hypereosinophilicsyndromewithelevatedigg4andtcellclonalityareportoftwocases AT wanglimin hypereosinophilicsyndromewithelevatedigg4andtcellclonalityareportoftwocases AT lixiuxiu hypereosinophilicsyndromewithelevatedigg4andtcellclonalityareportoftwocases AT luying hypereosinophilicsyndromewithelevatedigg4andtcellclonalityareportoftwocases |