OBJECTIVE Langerhans cell histiocytosis is an unusual disorder of unknown etiology with heterogeneous clinical behaviors and variable outcomes. It can involve one or more organs or systems, but to our best knowledge, parotid glands involvement in Langerhans cell histiocytosis is extremely rare in English literature. METHOD We report a 13-month-old girl who presented with bilateral parotid swe...

Familial hemophagocytic lymphohistiocytosis is a disorder which presents with fever, pancytopenia, liver dysfunction and also an increase in non-malignant histiocytes with prominent hemophagocytosis in various organs. It is usually difficult to distinguish from other hemophagocytic syndrome in the absence of family history. It rarely manifests in adults. Chemotherapy is usually indicated. Here,...

Introduction Germline mutations in SLC29A3 result in a range of clinically related, recessive syndromes: H syndrome, pigmented hypertrichosis with insulin-dependent diabetes mellitus (PHID) syndrome, Faisalabad histiocytosis (FHC), and sinus histiocytosis with massive lymphadenopathy (SHML). Main symptoms of these diseases are hyperpigmentation with hypertrichosis, sensorineural deafness, diabe...

A 54-year-old female presented with apparent isolated hypothalamic histiocytosis X associated with diabetes insipidus and Korsakoff's syndrome. Computed tomographic and magnetic resonance imaging demonstrated a single hypothalamic mass. A craniotomy for biopsy found granulation tissue of unknown cause. Further investigation discovered genital bleeding before admission. Biopsy of the cervix uter...

Progressive mucinous histiocytosis is a very rare, benign, non-Langerhans' cell histiocytosis limited to the skin. In total ten patients (all women) in four families and three sporadic cases have been reported. We report here the first published case of a male patient with progressive mucinous histiocytosis. The multiple red papules on the scalp and forearms were asymptomatic and had slowly inc...

Intracranial involvement with histiocytosis is usually limited to the hypothalamus and associated with extensive extracranial disease. We report the CT and MR findings in a patient with biopsy-proved histiocytosis presenting as multifocal intracranial involvement without any systemic disease. While extrahypothalamic CNS involvement by histiocytosis is well recognized (1-9], infiltrative brain i...

Langerhans cell neoplasms, which include histiocytosis and sarcoma, are tumors that originate from dendritic cells. sarcoma is defined as a high-grade neoplasm with overtly malignant cytological features the cell-like phenotype, generally has poorer prognosis more aggressive phenotype than histiocytosis. Insulin-like growth factor 2 messenger RNA-binding protein 3 (IGF2BP3 or IMP3) an oncofetal...

We have investigated the secretory function of cell suspensions from bone eosinophilic granulomas surgically collected in two patients with histiocytosis X. Unseparated cell preparations spontaneously produced interleukin 1 (IL-1) and prostaglandin E2 (PGE2). In order to ascertain that this secretion was due to the characteristic Langerhans cell-like histiocytosis X cells predominantly found in...

Clinical features, brain magnetic resonance imaging findings and EDSS scores of 11 patients with neurodegenerative central nervous system Langerhans cell histiocytosis were analyzed in Japan. All patients initially had multi-system-type Langerhans cell histiocytosis; 8 at 1-2 years of age and 3 at a later age. Neurodegenerative central nervous system Langerhans cell histiocytosis disease develo...

purpose : fine needle aspiration (fna) cytologic findings of langerhans cell histiocytosis (lch) have been well described, but using this method in the diagnosis of orbital lesions is a recent experience. we hereby report two cases of orbital bone lch diagnosed by fna that later was confirmed by routine h&e; histopathology and immunohistochemistry (ihc) methods. case reports : the first case wa...