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The case of a two-year-old girl with generalized histiocytosis, probably induced by phenobarbital, is reported. Symptoms, including intermittent fever, systemic lymphadenopathy, maculopapular skin eruption and hepatosplenomegaly, suggested Langerhans cell histiocytosis. Laboratory examinations
Langerhans cell histiocytosis (LCH), previously referred to as histiocytosis X, is a dendritic cell histiocytic tumor that demonstrates a variable spectrum of organ involvement. Clinical syndromes within this entity include eosinophilic granuloma, Hand-Schuller-Christian disease, Abt-Letterer-Siwe
Sinus histiocytosis with massive lymphadenopathy (SHML) was originally defined as a relatively specific benign pseudolymphomatous disorder. Although the etiology remains unknown, the spectrum of SHML has been expanded to include predominance of extranodal disease in some patients, clinically
Non-Langerhans cell histiocytosis in the absence of cutaneous or other organ involvement is very rare. A Caucasian boy age 3 years 11 months presented with episodes of recurrent right-side seizures over 2 weeks. Brain CT and MR imaging showed a single enhancing left frontal lobe lesion. Stereotactic
A 37 year old man presented with recurrent secondarily generalized seizures from right partial onset since December 1999, MRI scan of brain (contrast study) revealed multiple enhancing lesions predominantly involving frontal, parietal and temporal regions. Left frontotemporal lesion was biopsied and
A 37-year-old man presented with recurrent secondary generalised seizures from right focal onset. An MRI scan of the brain revealed multiple contrast enhancing lesions mainly involving the frontal, parietal and temporal regions. The left frontotemporal lesion was biopsied and histopathology
A retrospective analysis of 40 cases with histiocytosis X was undertaken to find out the course of primarily localized disease, and the prognosis of children with initially disseminated disease. Bone lesions recurred in nine of 23 children with localized histiocytosis X. In eleven cases other organ
An 8-year-old male Tibetan Terrier showed prolonged astasia, complete paralysis, ticlike signs, and seizure and died 2 months after the onset of symptoms. Histopathologically, there was moderate to severe infiltration of pleomorphic histiocytic mononuclear cells bilaterally in the basiarachnoidal
We report two cases of Langerhans' cell histiocytosis with unusual central nervous system (CNS) involvement. The first patient had behavioural disturbances, memory loss and diabetes insipidus. His response to a range of treatments was poor. The second patient presented with seizures and headaches
A cerebrospinal fluid sample collected from the cerebellomedullary cistern of a 10-year-old Shetland Sheepdog with a recent history of seizures was submitted for fluid analysis and cytologic examination. Key findings included a total nucleated cell count of 520/microL (reference interval 0-5
Fourteen children, 4 males and 10 females, with malignant histiocytosis (MH) were treated between July 1980 and June 1986. None of them had an affected sibling with a similar disorder. Septic-type fever was the most prominent symptom in all cases. Hepatosplenomegaly was present in 13 cases,
We report a case of Langerhans cell histiocytosis in a 64-year-old male who presented with symptoms and signs of brain involvement, including seizures and hypopituitarism. The diagnosis was confirmed with a biopsy of a lytic skull lesion. The disease affecting the bone showed no sign of progression
Ten children, four males and six females, with malignant histiocytosis were treated from July 1980 to July 1984. None of them had an affected sibling with a similar disorder. Septic-type fever, hepatosplenomegaly, lymphadenopathy, pulmonary infiltration, and disseminated intravascular coagulation
Progressive mucinous histiocytosis is a rare, benign, non-Langerhans' cell histiocytosis limited to the skin. Ten cases--all women--in four families and one sporadic case have been described in the literature. The disorder usually begins in childhood and progresses slowly. We report two sporadic
BACKGROUND
Because prostaglandin (PG) E2 has been identified in the bone lesions of Langerhans cell histiocytosis (LCH), we speculated that indomethacin, a potent PG inhibitor, may be useful in patients with symptomatic LCH involving the bony skeleton.
METHODS
We used indomethacin to treat patients