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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Histiocytosis X developed in five patients (one woman and four men) when aged between 15 and 44 years. The initial sign in four of them was eosinophilic granuloma of the bone, in one it was pulmonary involvement. In three patients the disease remained confined to bone, while in two it involved the lungs and central nervous system, respectively. Osteolysis regressed spontaneously in one of the men, while in the woman there has been no recurrence 8 years after resection of the focus. In another man an osteolytic focus in a rib was noted after a 9-year recurrence-free interval. The man with pulmonary and bone involvement received chemotherapy with vinblastine and prednisone: dyspnoea and cough disappeared, vital capacity improved and the interstitial lung changes regressed. The osteolytic foci were repeatedly irradiated in the man with bone and CNS involvement. This brought about considerable reduction in pain but no significant radiological changes of the foci. Two courses of chemotherapy were given over 12 years, once with vincristine and prednisone, afterwards with cyclophosphamide. This arrested the progression of the osteolytic foci, but each time they recurred when the drugs were stopped.
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PMID:[Histiocytosis X. Clinical aspects and course in 5 patients]. 193 52

The authors report on 21 cases of "primary" xanthoma of bone. Twenty of the patients were older than 20 years old. The male-female ratio was 2:1. The presenting symptom was pain in 13 patients and neurologic symptoms in 2; in 6 patients, the lesion was an incidental finding. All but one of the lesions in this series were solitary, and the flat bones (pelvis, rib, skull) were the most frequently involved sites. Radiographically, a well-defined, sometimes expansile lytic lesion, with either a small area of surrounding reactive bone or a distinct sclerotic margin, was seen. Microscopically, foam cells, giant cells, cholesterol clefts, and fibrosis were present in varying degrees. In none of these cases was there an identifiable underlying lesion. The differential diagnosis includes Erdheim-Chester disease (a multisystemic granulomatosis) and bone involvement in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). More important is the differential diagnosis with metastatic clear cell carcinoma. Xanthoma of bone is a benign lesion, and complete or even partial removal is effective. Xanthomas may represent a "burnt-out" benign condition such as fibrous dysplasia or histiocytosis X.
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PMID:Xanthoma of bone. 314 Jun 52

61 cases of histiocytosis X of jaws were reported here. They occurred more often in man (72% of men) than in woman, with an average age of 27 years. The mandible was affected more frequently than the maxilla. Clinically, dental expulsion, gingival swelling, fractures or pain were the usual symptoms. Roentgenographic examination showed either central or peripheral (alveolar bone) osteolytic areas. The histologic diagnosis was easy when eosinophilic polymorphous leukocytes or histiocytes were preponderant. In the other cases, immunohistochemistry (positivity of S-100 protein) and electron microscopy (presence of Birbeck granules about paraffin embedded specimens) were of a great usefulness. The prognosis of the disease was mainly related to the diffusion of lesions to other skeletal portions or extraskeletal localizations (37% of our cases). It has been improved by combination of chemotherapy to surgery (despite of frequent recurrences), 40 upon 45 patients are well after a follow-up of 2 to 10 years after the last localization.
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PMID:[Histiocytosis X of the jaw. I. Anatomico-clinical study apropos of 61 cases]. 350 9

Evaluation of the response of histiocytosis X to various forms of treatment is difficult, because presentation patterns are protean and the disease can be self-limited. A retrospective evaluation was made of the healing of 42 histiocytosis X bone lesions, in 21 patients treated in variety of ways, using serial radiographs and a semiquantitative scoring system. Treatments included various combinations of surgery (simple biopsy or curettage), radiotherapy (200 to 1,200 centi-Gray [cGy]), chemotherapy (according to various protocols), and local steroid injection. Median times to a given degree of healing were similar across treatment groups and in untreated lesions. It was concluded that mode of treatment does not exert a strong influence on the rate of healing of histiocytosis X bone lesions. Some healing should be apparent 4 months after diagnosis, but complete healing may take many months. Treatment of histiocytosis X bone lesions is indicated only if intense pain or risk of fracture or deformity are present.
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PMID:Healing rates of treated and untreated bone lesions in histiocytosis X. 387 75

The present case is a 24-years-old man who complained painful attack with numbness in both bilateral shoulder regions and distal extremities. This pain was increased by the stimulations of sunlight and heat. On physical examinations, he had an acromegalic-like-appearance, thick mustache and beard. Malocclusion of the teeth which showed the broad space was found. And skin lesion was not found out. Hypesthesia in bilateral distal extremities was revealed and no other abnormal neurological findings were observed. He was made diagnosis of Fabry's disease by laboratory examinations which were alpha-galactosidase deficiency analysed in leukocytes, increased ceramide trihexosides demonstrated in urinary sediment and electron microscopic findings in the biopsy of the skin and sural nerve. His mother had and decreased alpha-galactosidase activity which levels showed between normal and patient and was speculated to be a carrier. On an electron microscopy, "Zebra body" was observed in fibroblasts, capillary endothelial cells, their pericytes, prineural and Schwann cells. Many of them had a distinct limiting membrane and laminal structure with irregular alterations of light and dark zone. In the cytoplasma of Schwann cells, there were many rough endoplasmic reticulums which were located parallel with alignment and seemed to show the loosed laminal structure with the unclear limited membrane. Occasionally, fusions between irregular laminal structure and rough endoplasmic reticulum were also observed. These findings could be indicated that the formation of Zebra body is related to rough endoplasmic reticulum, because laminal structure which described above is corresponded to pre-Zebra body. In the cytoplasma of the prineural cells, recket-like structure which was seen in histiocytosis X were occasionally observed.
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PMID:[Fabry's disease with malocclusion and acromegalic-appearance: clinical and electromicroscopic studies (author's transl)]. 624 68

Langerhans' cell granulomatosis (LCG) (histiocytosis X) of bone is generally considered a childhood disease. A series of 61 adult patients who presented with histologically confirmed skeletal disease was studied. The male-to-female ratio was 2.1, and 36 patients were more than 30 years old, the oldest being 71 years. Pain was the most frequent symptom. Forty-six patients has solitary lesions, six had two or three, and nine had more than three. The time interval between the development of lesions, although usually less than 2 years, ranged up to 32 years. Rib, skull, and pelvis were the most frequently involved sites. Eight patients had extraskeletal disease. Radiologically, a well-defined lytic lesion, either with no surrounding reaction or with sclerotic margins, was seen most often. The rare, poorly defined lesions tended to occur in the clavicle. Microscopically, typical Langerhans' cells were present in large numbers. Eosinophils were usually prominent. Benign giant cells, mitotic figures, and necrosis also were observed. There was no histologic difference between solitary and multiple lesions. Surgery, radiation therapy, or a combination of both usually provided adequate treatment. Only two of the solitary lesions recurred. Eleven patients suffered from chronic, progressive disease, which inconsistently responded to treatment. None of the patients in our series died of the disease.
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PMID:Langerhans' cell granulomatosis (histiocytosis X) of bone in adults. 698 5

A 4-year-old girl presented with pain associated with a mobile mandibular left permanent first molar. The mandibular left primary second molar was also found to be mobile and the gingiva buccal to these teeth was inflamed. A well-defined radioluceny of the left posterior mandible was detected radiographically. Langerhans' cell histiocytosis had been diagnosed 10 months previously when she had presented with an isolated tibial lesion. Curettage of the mandibular lesion and extraction of four adjacent teeth was performed. Post-operatively, radiographic skeletal screening revealed osteolytic lesions in the right parietal bone and right eighth rib. A 6-month course of chemotherapy was instigated which resulted in bony healing of all the osteolytic lesions.
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PMID:Multifocal Langerhans' cell histiocytosis in a 4-year-old child: a case report. 774 47

Hospital records of seventeen patients (11 males & 6 females) with Langerhans cell histiocytosis, confirmed by electron microscopic demonstration of Birbeck granules, were studied retrospectively from October 1982 to October 1992 at Taichung Veterans General Hospital. The ages at presentation ranged from 5 months to 17 years (a median of 6 years). The clinical features were protean and included fever, pain, bony lesions, lung lesions, abnormal dentition, diabetes insipidus, oral ulcer, otorrhea, dermatitis, anemia, thrombocytopenia, lymphadenopathy, and hepatosplenomegaly. Skull and femur were the most common sites of bony lesions. The main therapeutic modalities were excision, radiotherapy, and chemotherapy with vincristine and prednisolone. The young age at presentation, several involved organ systems, presence of organ dysfunction, and clinical diagnosis with Letterer-Siwe disease were poor prognostic factors. Although Langerhans cell histiocytosis is not a rare disease, the cause is still unknown. It needs further research to disclose the mystery.
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PMID:Langerhans cell histiocytosis: a 10-year review. 794 24

In 1973, a 10 year old boy presented with numerous bilateral lung nodules, diagnosed as histiocytosis X by open lung biopsy. The patient was treated with prednisone until 1984. In 1993, he developed severe pain in the neck. A biopsy of the spine revealed the same tumour morphology as was seen in the lung in 1973. Immunohistological examination of the former and present biopsy led to the definitive diagnosis of epithelioid haemangioendothelioma of the lung with metastases to spine and liver. Epithelioid haemangioendothelioma of the lung is a rare soft tissue tumour of vascular origin, readily mistaken for carcinoma or, as in this case, histiocytosis. The tumour has an intermediate malignant potential. Although metastases of epithelioid haemangioendothelioma of the lung are well-known, metastatic spread to bones, as in our case, has not previously been mentioned in the literature.
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PMID:Epithelioid haemangioendothelioma of the lung: clinical and pathological pitfalls. 857 93

Otological involvement in Histiocytosis X, although infrequent, may be present in any of the forms of this entity. The otologist must keep Histiocytosis X in mind in the differential diagnosis of cases presenting with post-aural swelling, non purulent otorrhea and absence of fever and pain in children under three-years old. Some aspects of clinical presentation and treatment are discussed.
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PMID:Eosinophilic granuloma of the temporal bone. 863 50


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