1/94. Visual loss with Langerhans cell histiocytosis: multifocal central nervous system involvement.A 42-year-old woman with a 6-year history of diabetes insipidus and progressive hypersomnolence presented with visual loss. neuroimaging showed infiltration in the hypothalamus, the optic nerve, and the chiasm, as well as multiple lesions in other areas of the brain parenchyma. biopsy showed Langerhans cell histiocytosis. This is an unusual presentation of Langerhans cell histiocytosis, involving the visual pathways without manifestations outside of the central nervous system. The differential diagnosis and the magnetic resonance imaging findings will be discussed.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/94. Labyrinthine involvement in Langerhans' cell histiocytosis.BACKGROUND: Langerhans' cell histiocytosis, a rare condition caused by the proliferation of abnormal Langerhans' cells ('LCH cells') and an accompanying granulomatous infiltrate, can affect several organs including the ear. External and middle ear involvement are common with a reported incidence as high as 61%. The bony labyrinth is resistant to erosion by the granulation tissue, thereby protecting the cochlea and vestibular structures. Probably for this reason, involvement of the inner ear is rare, with few case reports in the literature. patients: We report two girls, one with bilateral and the other with unilateral mastoid involvement, in whom there was invasion of the labyrinth. The first girl had 'single system' LCH affecting only bone and developed an acute hearing loss due to invasion of the cochlea, while the second had both bone and skin involvement and labyrinthine involvement was diagnosed on imaging prior to the onset of labyrinthine symptoms. CONCLUSION: Inner ear involvement can lead to permanent deafness, which may be prevented by early institution of treatment. Threatened inner ear involvement requires urgent systemic medical therapy with steroids, possibly combined with chemotherapy.- - - - - - - - - - ranking = 53.984206540278keywords = organ (Clic here for more details about this article) |
3/94. Langerhans' cells histiocytosis.Langerhans cell histiocytosis (LCH) is a rare disorder of unknown cause, characterized by the proliferation of histiocytic cells in various tissues and organs. The role of the otolaryngologist is important in the early and accurate evaluation, staging and diagnosis of LCH, because it may mimic more common diseases such as otitis externa and acute mastoiditis. We discuss a case report of bilateral mastoid involvement in a child with a history of otalgia unresponsive to medical therapy.- - - - - - - - - - ranking = 53.984206540278keywords = organ (Clic here for more details about this article) |
4/94. Expression of Fas ligand in Langerhans' cell histiocytosis: A case report of a boy with multisystem involvement.Previous reports of patients with Langerhans' cell histiocytosis have shown characteristics of osteolytic lesion, visceral involvement and organ dysfunction. We report a 2-year-old boy who was diagnosed as Langerhans' cell histiocytosis with a prominent hepatomegaly. X-Radiogram, computed tomography and magnetic resonance imaging revealed the osteolysis of the right iliac bone, the absence of the left eighth rib as well as the right mandible, and an enhancing mass in the left cerebellum. The data of radiography were highly suggestive of Langerhans' cell lineage. The presence of large CD1a-positive mononuclear cells associated with inflammatory cells in peripheral blood smear and bone marrow aspirate further confirmed the diagnosis. In addition, expressions of S100, CD25, CD68, CD80, CD86, and Fas ligand were identified on these cells by immunocytochemical staining. The results indicate that although these cells are activated Langerhans' cells, progression of the disease and the bone destruction could be mediated by the overt FasL expression of the cells.- - - - - - - - - - ranking = 53.984206540278keywords = organ (Clic here for more details about this article) |
5/94. Neuropsychologic deficits in children with Langerhans cell histiocytosis.BACKGROUND: Manifestations of Langerhans cell histiocytosis (LCH) in children range from only a rash, to bony lesions accompanied by pain, to major organ disease. When the central nervous system (CNS) is affected, the LCH patient may exhibit signs and symptoms of hypothalamic and pituitary dysfunction (most often resulting in diabetes insipidus or other endocrinopathies) or more global neurologic and neuropsychologic sequelae. Surprisingly, researchers have only recently begun to examine the neuropsychologic manifestations of the disease, but early findings suggest that they may, in fact, be significant in a small percentage of children with LCH. PROCEDURE: We evaluated two CNS-positive patients with LCH and long-term intermittent treatments, using extensive neuropsychologic assessments, including intellectual functioning, memory, visual-motor functioning, attention and concentration, sensory and motor performance, and gross academic achievement. Objective measures of behavior were obtained through parental report. Neuroradiologic imaging was obtained concurrently with the neuropsychologic evaluations. RESULTS: The neuropsychologic assessments indicated significant deficits in a number of the measured areas of functioning. Global cognitive deficiencies in full-scale IQ were identified, as were deficits in memory, attention/concentration, and perceptual-organizational capabilities. Similarities were noted in the patterns of deficits obtained with both patients, despite differences in the pathophysiology of their disease. Behavioral functioning in both children had suffered, presumably in relation to the neuropsychologic deficits. There were radiologic findings of gross cerebellar white matter damage in one patient, in addition to focal (e.g., hypothalamic) lesions in the other. CONCLUSIONS: LCH has an adverse impact on cognitive functions in some children with evidence of CNS involvement, and further study into the etiology, incidence, and means of remedial intervention is needed.- - - - - - - - - - ranking = 107.96841308056keywords = organ (Clic here for more details about this article) |
6/94. histiocytosis X of the cauda equina.laminectomy, performed on a 46-year-old woman because of weakness and paresthesias of the lower limbs, showed multiple discrete yellow nodules on the nerve roots of the cauda equina. One of these lesions was excised and proved to be a granuloma consistent with the diagnosis of histiocytosis X.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
7/94. Ocular and cerebral involvement in familial lymphohistiocytosis.A 5-month-old infant girl died of familial lymphohistiocytosis. The central nervous system showed widespread perivascular infiltration of the cerebral pia and arachnoid, the cerebral white matter and deep gray matter, the cerebellum, and brain stem by lymphocytes, benign appearing histiocytes, and macrophages with erythro-and lymphophagocytosis. The eyes had mild infiltration of the anterior uveal tract, moderate involvement of the inner retina, and marked infiltration of the optic nerves by identical cells.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
8/94. Langerhans cell histiocytosis of the thyroid gland. A case report.BACKGROUND: The variant of Langerhans histiocytosis commonly encountered in adults is the benign, localized form (eosinophilic granuloma). The more-aggressive or diffuse type (Letterer-Siwe disease) is rare in adults. CASE: A 28-year-old woman presented with enlargement of the thyroid gland three years after she had been diagnosed with and placed on treatment for diabetes insipidus. thyroidectomy was performed following an initial fine needle aspiration cytology report of either papillary thyroid carcinoma or Langerhans cell histiocytosis. The latter diagnosis was confirmed on histopathology and immunohistochemical staining for S-100 protein. Intracellular Birbeck granules were also demonstrated by electron microscopy. The disease progressed over a 10-week period to involve the kidneys, resulting in renal dysfunction. CONCLUSION: Synchronous or metachronous involvement of the hypothalamus and thyroid gland by Langerhans histiocytosis could not be excluded in the present case, with subsequent progression to involve other organs. This was an unusual presentation of the disease in an adult.- - - - - - - - - - ranking = 53.984206540278keywords = organ (Clic here for more details about this article) |
9/94. Concurrent Langerhans cell histiocytosis and myelodysplasia in children.BACKGROUND: Langerhans cell histiocytosis (LCH) is characterized by the proliferation of abnormal histiocytes (langerhans cells), whose origin as a reactive process or a neoplastic disorder is still poorly understood. Although LCH has been recorded as being associated with malignant neoplasms, concurrence of LCH and myelodysplastic syndrome has not been reported so far. PROCEDURE: We report on four children aged 23, 25, 26, and 53 months with multisystem LCH with organ dysfunction (bone marrow and liver) whose bone marrow pictures, taken at diagnosis, revealed the presence of myelodysplastic abnormalities (RA, RAEB, RAEB-t). RESULTS: We suggest that the commonly used expression of "organ dysfunction," which refers to clinical and functional alterations, could be explained by a myelodysplastic-like disorder. CONCLUSIONS: The contemporary presence of both events may provide a better understanding of the pathogenesis of LCH, especially in young children with multisystem disease and organ dysfunction, who are known to have a very poor outcome.- - - - - - - - - - ranking = 161.95261962083keywords = organ (Clic here for more details about this article) |
10/94. Langerhans' cell histiocytosis of the thymus associated with multilocular thymic cyst.Though Langerhans' cells are a normal component of the thymus, the pathologic entity of Langerhans' cell histiocytosis (LCH) occurs very uncommonly in this organ. The few reports of thymic LCH are nearly all in children or young adults and may or may not be associated with extrathymic clinical disease. Although radiographic descriptions of cyst formation in the thymus in association with LCH have been reported, no examples to our knowledge of thymic LCH have been reported in association with multilocular thymic cyst (MTC). We report a case of thymic LCH in association with an asymptomatic multilocular thymic cyst which was discovered as part of cardiothoracic surgery in a middle-aged man. To our knowledge, this is the first example of MTC occurring in association with thymic LCH. HUM PATHOL 31:1532-1535.- - - - - - - - - - ranking = 53.984206540278keywords = organ (Clic here for more details about this article) |
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