Brain Diseases ; pathology ; Histiocytosis, Sinus ; pathology ; Humans ; Skull ; pathology

Histiocytosis, Langerhans-Cell ; pathology ; Humans ; Intestinal Diseases ; pathology

Adult ; Female ; Histiocytosis, Sinus ; pathology ; surgery ; Humans ; Skull ; pathology

Female ; Histiocytosis, Langerhans-Cell ; pathology ; Humans ; Infant, Newborn

Histiocytosis, Langerhans-Cell ; Humans ; Infant ; Male ; Temporal Bone ; pathology

Although Rosai-Dorfman disease is a rare disorder characterized by proliferation of distinctive histiocytes within lymph node sinuses and lymphatics, sometimes involving extranodal sites, the nasal cavity and pharyngeal cavity are rarely involved additional. In this report, a 22-year old man with multiple subcutaneous lumps and submucosal lumps in bilateral nasal cavity and pharyngeal cavity is presented, which was diagnosed by nasopharyngeal endoscope,computed tomography scan and biopsy. The subcutaneous lumps were ablated and prednisone was accepted for three months as preventive therapy.
Histiocytosis, Sinus ; pathology ; Humans ; Male ; Nasal Cavity ; pathology ; Pharynx ; pathology ; Subcutaneous Tissue ; pathology ; Young Adult

Encephalocele ; pathology ; Granulomatosis with Polyangiitis ; pathology ; Histiocytosis, Sinus ; pathology ; Humans ; Otorhinolaryngologic Diseases ; pathology ; Pathology ; trends ; Precancerous Conditions ; pathology ; Tuberculosis, Laryngeal ; pathology

By using the method of clonal analysis the evidence to prove that Hemophagocytic syndrome (HPS) is reactive or malignant was investigated to probe into the pathogenesis of HPS and its relations with clinical prognosis. The macrophages abnormally proliferated in bone marrow were isolated. Electrophoresis analysis was made after DNA extraction, enzyme restriction of human ardrogen receptor (HUMARA) genetic locus, and PCR amplification. In the 9 specimens, clonal proliferation was found in 2 cases and nonclonal proliferation in 7. Among the 7 cases of nonclonal proliferation, 3 were voluntarily discharged without clinical outcome, 2 cases fully recovered after 2-3 week treatment of large dose gamma globulin intravenous drip and hormone therapy, 1 case died at the 43th day after the hormone and anti-infection therapy, and one case was found to have granular leukoblast in peripheral blood after 3 weeks and diagnosed as having M2a after bone puncture. For the two patients with clonal proliferation, one obtained remission after chemotherapy and the other was died after 32 days without chemotherapy. It was concluded that there do exist clonal or malignant proliferation in HPS, so not every case is reactive.
Clone Cells ; Histiocytosis, Non-Langerhans-Cell/*blood ; Histiocytosis, Non-Langerhans-Cell/therapy ; Macrophages/*pathology ; gamma-Globulins/therapeutic use

Diagnosis, Differential ; Histiocytoma, Benign Fibrous ; metabolism ; pathology ; Histiocytosis, Langerhans-Cell ; metabolism ; pathology ; Histiocytosis, Sinus ; metabolism ; pathology ; surgery ; Humans ; Infant ; Male ; S100 Proteins ; metabolism ; Xanthogranuloma, Juvenile ; metabolism ; pathology

Rosai-Dorfman disease in nasal cavity and nasal sinuses is known as an idiopathic proliferative disease of histiocytes with a distinct morphologic feature and is very rare. It is easy to relapse and be misdiagnosed. In on case, the infiltrating histiocytes had large round or oval nuclei. The cytoplasm was abundant, granular or vacuolated and foamy. Immunohistochemical studies showed that the histiocytes were strongly reactive with antibodies against S100 and CD68. The diagnosis of extranodal RDD in the paranasal sinuses could be differentiated with rhinoscleroma, eosinophilic granuloma, plasmacytoma, or fibrous histiocytoma. It showed that the identification of the distinctive histiocytes which are typically immunoreactive to S100 and CD68 can be quite helpful for correct diagnosis.
Adult ; Histiocytosis, Sinus ; pathology ; Humans ; Male ; Nasal Cavity ; pathology ; Paranasal Sinuses ; pathology