Langerhans' cell histiocytosis (LCH) of the liver is uncommon. When seen, it is part of multifocal disease and can present as biliary obstruction. We present a case of sclerosing biliary disease with a solitary LCH lesion and no evidence of systemic disease. We postulate that the LCH is a secondary phenomenon, arising against a background of a complex, familial liver disease. This case also rai...

Etiological classification of diabetes insipidus is as follows. 1. Central Diabetes Insipidus (CDI) A. Idiopathic B. Familial C. Structural Causes i. Congenital: Septo-optic dysplasia Tuber cinereum Hamartoma ii. Traumatic: Iatrogenic, head trauma iii. Inflammatory: Tuberculous Meningitis Sarcoidosis Wegener’s granulomatosis Lymphocytic Hypophysitis iv. Neoplastic: a. Pediatric: Hypothalamic gl...

Sinus histiocytosis massive lymphadenopathy (SHML) syndrome with hepatic involvement, occurring in a seven-year-old black female, is reported. Morphologic characterization of the hepatic lesion is accomplished utilizing conventional light, fluorescent and electron microscopy, and histochemical techniques and by comparing and contrasting the findings with those in cases of familial erythrophagoc...

Langerhans cell histiocytosis (LCH) as a term encompasses eosinophilic granuloma and two clinical syndromes: Letterer-Siwe disease and Hand-Schüller-Christian. All these syndromes seem to represent similar processes in which the proliferating cells have the structural and functional features of Langerhans cells. In reviewing the literature found that this disease does indeed occur in some famil...

langerhans cell histiocytosis (lch) is a rare disorder described as three different entities including eosinophilic granulomaof bone, the hand-schuller-christian syndrome, and letterer-siwe disease. lch is currently classified into singlesystem lch, and multisystem lch. patients with single system lch have an excellent prognosis, and are mostlytreated with local therapy. multisystem lch is subd...

BACKGROUND Some uncommon lymphoproliferative and histiocytic disorders may present with an aggressive course and require hematopoietic stem cell transplantation (HSCT) as part of the therapeutic approach. METHODS Published research on the use of HSCT for the treatment of these disorders was reviewed and summarized. RESULTS Allogeneic HSCT may be indicated in patients with blastic plasmacyto...

Histiocytosis, according to Histiocyte Society2, is traditionally classifi ed into three main groups: Class I: (Langerhans cell histiocytosis), formerly called X histiocytosis Class II: Histiocytosis of other mononuclear phagocytes different from Langerhans cells Class III: Histiocytic malignancies.1,3,4 Nevertheless, Langerhans cell Histiocytosis is the currently preferred term. It is classifi...