[Eosinophilic granuloma of the parietal bone of an adult patient with BRAF mutation]. / Éozinofil'naia granulema temennoi kosti s mutatsiei gena BRAF u vzroslogo patsienta.

The paper describes a case of eosinophilic granuloma of the parietal bone in a 32-year-old man. Histological examination revealed a large number of bean-shaped Langerhans cell histiocytes with lobed nuclei and nuclear grooves. The histiocytes alternated with the foci of obvious eosinophilic infiltration and with eosinophilic microabscesses. There were osteoclast-like multinucleated giant cells, bone resorption, and numerous bone rods covered with osteoblast chains. The histiocytes expressed CD1α, langerin, CD68, S100, and p53 (in 90.0% of the tumor cells). The Ki-67 proliferation index was 18.0%. A molecular genetic study identified BRAFV600E mutation (nucleotide substitution s.1799 T>A (p.V600E) in the heterozygous state). Clinical and morphological data and the results of molecular genetic studies led to the conclusion that there was eosinophilic granuloma of the right parietal bone (the unifocal form of Langerhans cell histiocytosis (LCH), type I, group A1, with the monoossal nature of lesion and with BRAFV600E mutation). In adults, this disease is extremely rare (2-5 cases of LCH per million people, bone loss in the fourth decade of life in 2.5% of the patients).

Rare case of eosinophilic granulomatosis with polyangiitis in two patients with α-1-antitrypsin deficiency (PiSZ).

We present two cases of eosinophilic granulomatosis with polyangiitis occurring with α-1-antitrypsin deficiency, both PiSZ phenotype. The simultaneous occurrence of these two conditions has seldom been described in the literature, despite evidence of an association between α-1-antitrypsin deficiency and other forms of vasculitis. Both patients had pulmonary involvement and reported intermittent exacerbations of vasculitic symptoms. Both patients were managed on low-dose oral steroids and azathioprine remaining well with occasional exacerbations. It is important to consider whether there is an association between eosinophilic granulomatosis with polyangiitis and α-1-antitrypsin deficiency, as this may lead to more severe pulmonary symptoms during exacerbations. If a genetic association between the two conditions is found, clinicians should be aware of the possible need to screen for α-1-antitrypsin deficiency in appropriate patients.

Tumor-associated eosinophilia.

The review provides information on current literature on structural and functional features of eosinophilic granulocytes and their role in the pathogenesis of cancer. There are examined data of clinical and experimental studies about an influence of hemic and tissue eosinophilia on the course and prognosis of malignant tumors. Molecular mechanisms of action of eosinophils in tumor pathology are discussed.

[Inflammatory fibroid polyp: from Vanek's "submucosal granuloma" to the concept of submucosal mesenchymal neoplasia]. / Inflammatorischer fibroider Polyp: Von Vaneks "submukösem Granulom" zum Konzept einer submukösen mesenchymalen Neoplasie.

Inflammatory fibroid polyps (IFP) were described by Vanek 60 years ago as "submucosal granuloma with eosinophilic infiltration". IFP represent polypous proliferations of spindle cells in the submucosa and mucosa of the stomach, small bowel and colon with inflammatory infiltration. The lesions have been regarded as inflammatory and reactive. Recent data show that the spindle cells express PDGFRA, and the majority of IFP harbour activating PDGFRA mutations. Therefore, IFP represent true benign mesenchymal tumors of the gastrointestinal tract.

Traumatic ulcerative granuloma with stromal eosinophilia: a reactive lesion of the oral mucosa.

Traumatic ulcerative granuloma with stromal eosinophilia (TUGSE) is a benign lesion of the oral mucosa of an unclear pathogenesis. We analyzed the profile of the inflammatory infiltrate in 12 cases of TUGSE by using immunohistochemical analysis and polymerase chain reaction-based repertoire analysis to detect T- and B-cell receptor gene rearrangements. The inflammatory infiltrate consisted in most cases of B and T lymphocytes, macrophages, abundant eosinophils, and large atypical cells. In 5 cases, CD30+ cells were found. Spectratyping analysis displayed a polyclonal rearrangement of the T-cell receptor g gene in 6 cases and oligoclonality in 5 cases. Monoclonality was observed in 1 case that also fulfilled histologic criteria for lymphoma. Healing was uneventful in all cases, including the one suspected of being lymphoma, with no recurrences in more than 2 years'follow-up. TUGSE can be regarded reactive. Some cases, however, may harbor a dominant clonal T-cell population; in these cases, long-term follow-up is mandatory.

Non-pruritic granuloma in Norwegian forest cats.

The eosinophilic granuloma complex is a group of skin disorders common in cats. This paper describes the clinical, haematological and histopathological features of 17 related Norwegian forest cats, six of which had a linear granuloma on the caudal thigh, three of which also had a granuloma on the lower lip, and one of which had a granuloma in combination with an indolent ulcer. The high prevalence of the disease in this population is suggestive of a genetic background.

Eosinophil-rich CD30+ lymphoproliferative disorder of the oral mucosa. A form of "traumatic eosinophilic granuloma".

We describe 3 patients who had oral mucosal lesions with features of traumatic eosinophilic granuloma (TEG) and containing CD30+ atypical cells. In 1 patient, the oral lesion was followed by skin nodules. All lesions were evaluated histologically, by immunohistochemical analysis, and by polymerase chain reaction (PCR) analysis of the T-cell receptor (TCR) gamma chain gene. All oral lesions were characterized by a dense and deeply infiltrative lymphoproliferation, showing epitheliotropism and massive eosinophilia. They contained atypical large lymphoid cells, which expressed T-cell markers and CD30. PCR analysis showed a monoclonal rearrangement of the TCR gamma chain gene in all lesions and, in 1 patient, the same rearrangement in the oral and cutaneous specimens. The lesions in these patients seem to be the oral counterpart of the spectrum of primary cutaneous CD30+ lymphoproliferative disorders and should be recognized as such to avoid a diagnosis of large T-cell lymphoma and possible consequent overtreatment. However, they represent only a subset among several others within the complex and heterogeneous category of disorders referred to as TEG.

Schistosome infection of transgenic mice defines distinct and contrasting pathogenic roles for IL-4 and IL-13: IL-13 is a profibrotic agent.

Experimental Schistosoma mansoni infections of mice lead to a dynamic type 2 cytokine-mediated pathological process. We have used IL-4-deficient, IL-13-deficient, and IL-4/13-deficient mice to dissect the role of these cytokines in the development of immune response and pathology following S. mansoni infection. We demonstrate that while both of these cytokines are necessary to develop a robust Th2 cell-driven, eosinophil-rich granuloma response, they also perform disparate functions that identify novel sites for therapeutic intervention. IL-13-deficient mice demonstrated significantly enhanced survival following infection, which correlated with reduced hepatic fibrosis. In contrast, increased mortality was manifest in IL-4-deficient and IL-4/13-deficient mice, and this correlated with hepatocyte damage and intestinal pathology. Therefore, we demonstrate that during a dynamic type 2 cytokine disease process IL-13 is detrimental to survival following infection, whereas IL-4 is beneficial.

Cytogenetic study of twenty-two intracranial tumors.

G-banded chromosomal analysis was performed on primary cultures of 22 intracranial tumors, including eight astrocytomas, nine meningiomas, two dermoid cysts, one acoustic neuroma, one pineal teratoma and one eosinophilic granuloma. One or more chromosomally abnormal clones were observed in 6 (75%) gliomas and 5 (56%) meningiomas. There was no chromosomal abnormality found in one of the dermoid cysts, the acoustic neuroma or the eosinophilic granuloma. A teratoma and a grade IV glioma had heterogeneous hyperdiploid karyotypes. Furthermore, astrocytomas displayed nonrandom loss of chromosomes #19, #21, #22 and Y. In meningiomas, characteristic changes involving chromosome 22 were found in 5 tumors. One meningioma had a ring chromosome in addition to chromosomal loss. With our culture and harvesting techniques, cytogenetic studies can be successfully performed on nearly all intracranial tumor explants, including those derived from small biopsy specimens. Also, in our study, specific nonrandom chromosomal anomalies were found.