Concurrent eosinophilia and IgG4‑related disease in a child: A case report and review of the literature

Abstract

The current study presents the case of a 9-year-old Chinese boy who presented with eosinophilia and elevated serum levels of immunoglobulin G4 (IgG4). A bone marrow puncture identified an elevated eosinophil rate of 23% (normal range, <5%), which indicated eosinophilia. However, gene analysis, fluorescent in situ hybridization and other examinations, including bone marrow aspiration, blood routine, auto-antibody tests and parasitic and allergens screening, contradicted a diagnosis of secondary or clonal eosinophilia. Furthermore, the patient exhibited multiple lymph node swelling and a lymph biopsy strongly indicted a pathological diagnosis of IgG4-related disease (IgG4-RD). His peripheral blood flow cytometry confirmed an elevated count of plasmablasts, which is specific to IgG4-RD. The patient responded well to therapy with prednisone and remained healthy in all follow-ups. By taking all these factors into consideration, the boy was diagnosed with IgG4-RD. It is difficult to distinguish IgG4-RD from hypereosinophilic syndrome and the potential association between the two remains unclear. However, the present case study serves as a reminder that IgG4-RD may occur in children and medical professionals should not neglect this possibility.

Computerized tomography features and clinicopathological analysis of Kimura disease in head and neck

“…There are a few reports of Kimura disease cases involving the groin, limbs, eyelid, tongue, auricle, hard palate or throat (17,18). The onset age (middle aged, 35-50 years), sex (>90% males) and site of pathological changes, with a majority in the head and neck regions poten-in the head and neck regions poten-the head and neck regions potentially affecting major salivary glands and lymph nodes, in the current study were consistent with the literature (12,13).…”

“…(11). Kimura disease may occur at any age, although the peak age is 20-40 years; the majority of cases occur in middle-aged males (12,13). The male to female ratio has been reported to be as high as 14:1 (14).…”

Chronic myelomonocytic leukemia-associated pulmonary alveolar proteinosis: A case report and review of literature

“…As sPAP secondary to MDS is very rare, only a limited number of case reports and small case series have been published to date. In total, 65 such cases have been reported to date as noted in Table 1 [ 9 , 20 - 30 ] with ages ranging from 18–75-years-old and with a male/female ratio of 27/38. Of the eleven cases of PAP diagnosed prior to MDS, only four, including the present case, were diagnosed as PAP coinciding with MDS.…”

“…Histological analyses exhibited the thickening of the alveolar septum with fiber hyperplasia, vasodilation, congestion and the infiltration of histiocytes and inflammatory cells. Samples were positive for periodic acid-Schiff staining, and flow cytometry analyses were performed as previously described[ 9 ], revealing an elevated frequency of monocytes in the BALF samples (12.4%) with CD33+ CD14+ CD300e+ mature monocytes accounting for 4% of nucleated cells and CD33+ CD14- CD300e+ partially immature monocytes accounting for 8.4% of nucleated cells (Figure 1A ).…”

“…CMML molecular studies revealed the presence of synonymous ASXL and SRSF mutations without any corresponding TET2 mutations. These examinations were performed as previously described[ 9 ]. Karyotyping revealed the inversion of chromosome 6.…”