No abstract available.
Histiocytosis, Non-Langerhans-Cell ; Xanthomatosis

No abstract available.
Histiocytosis ; Histiocytosis, Non-Langerhans-Cell ; Stomach* ; Xanthomatosis*

Adult ; Histiocytosis, Non-Langerhans-Cell ; Humans ; Male

Xanthoma disseminatum (XD) is one of cutaneous nonhistiocytosis X, and characterized by multiple, widely distributed red, brown, yellowish papules and nodules that tend to coalesce. We present a case of XD with characteristic clinical findings and treated with the combination therapy of COz laser vaporization and high dose steroid.
Histiocytosis, Non-Langerhans-Cell* ; Laser Therapy ; Lasers, Gas* ; Volatilization* ; Xanthomatosis*

Reticulohistiocytosis represents a rare spectrum of non-Langerhans cell histiocytosis: the solitary cutaneous form (reticulohistiocytoma), the diffuse cutaneous form without systemic involvement, and multicentric reticulohistiocytosis with systemic involvement. We report a case of solitary cutaneous reticulohistiocytosis in a 46-years-old male, who presented with an asymptomatic firm, dome-shaped nodule on the right vestibule of nose. The lesion was completely excised and no evidence of recurrence was observed.
Histiocytosis, Non-Langerhans-Cell ; Humans ; Male ; Nose ; Recurrence

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

Reticulohistiocytosis represents a rare spectrum of non-Langerhans cell histiocytosis: the solitary cutaneous form (reticulohistiocytoma), the diffuse or multiple cutaneous form without systemic involvement, and multicentric reticulohistiocytosis with systemic involvement. To the best of our knowledge, there have been relatively few reports in the literature of solitary cutaneous reticulohistiocytoma found on the lip. We report here on a case of solitary cutaneous reticulohistiocytoma in a 58-year-old male, who presented with an asymptomatic dome-shaped nodule adjacent to the upper lip. The histopathologic examination revealed a dense dermal infiltrate, that was composed predominantly of large oncocytic histiocytes and multinucleated giant cells with abundant granular eosinophilic cytoplasms, which had a "ground-glass" appearance.
Cytoplasm ; Eosinophils ; Giant Cells ; Histiocytes ; Histiocytosis ; Histiocytosis, Non-Langerhans-Cell* ; Humans ; Lip* ; Male ; Middle Aged

Familial hemophagocytic lymphohistiocytosis (FHL) is a rapidly fatal illness, usually encountered in infancy, characterized by fever, hepatosplenomegaly, pancytopenia, and central nervous system involvement. Microscopic examination of tissue shows a non-malignant lymphohistiocytic infiltrate, with prominent erythrophagocytosis. FHL is an autosomal recessive hereditary disorder but may develop secondarily to other conditions such as immunosuppression, malignancies, fat overload and certain infections. We recently experienced a case of siblings developing FHL, which may be associated with EBV infection.
Case Report ; Child, Preschool ; Female ; Herpesviridae Infections/complications* ; Herpesvirus 4, Human* ; Histiocytosis, Non-Langerhans-Cell/virology* ; Histiocytosis, Non-Langerhans-Cell/genetics* ; Human ; Infant ; Male ; Tumor Virus Infections/complications*

Blood Cell Count ; Child ; Fever ; Histiocytosis, Non-Langerhans-Cell ; diagnosis ; Humans ; Male ; Prognosis

Xanthoma disseminatum (XD) is a rare, potentially progressive non-Langerhans cell histiocytosis, which preferentially affects males in their childhood or young adulthood. It is characterized by the insidious onset of small, yellowish-red to brown papules and nodules that are discrete and disseminated. They characteristically involve the eyelids and flexural areas of the axillary and inguinal folds, and the antecubital and popliteal fossae. Systemic disease frequently occurs, the most common manifestation being diabetes insipidus (DI), which occurs in about 40% of cases and is due to deposition of xanthoma cells in the hypothalamic-pituitary axis. We present a case of XD with DI, which was treated with the combination therapy of CO2 laser vaporization, cyclophosphamide, electrocauterization and synthetic anti-diuretic hormone.
Axis, Cervical Vertebra ; Cyclophosphamide ; Diabetes Insipidus ; Eyelids ; Histiocytosis ; Histiocytosis, Non-Langerhans-Cell ; Humans ; Lasers, Gas ; Male ; Volatilization ; Xanthomatosis