Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Erdheim-Chester disease (ECD) is a rare disorder with fewer than 80 cases reported in the world. It consists of a non-Langerhans' cell histiocytosis that usually presents as pain due to bone involvement; however, the prognosis is marked by extraskeletal involvement. Although the final diagnosis needs an anatomophatologic study (normally through a bone biopsy), radiologic and scintigraphic findings are quasi pathognomonic. In this work, we report 2 ECD cases and their respective bone scans showing typical findings described in the literature. We found bilateral and symmetrical increased uptake of diaphyses and metaphyses of long bones, mainly in lower limbs. The mid-diaphyses and the epiphyses (partially in the first case) as well as the axial skeleton are spared.
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PMID:[Bone scintigraphy in Erdheim-Chester disease]. 1284 51

Eosinophilic granulomas of long tubular bones, a form of Langerhans cell histiocytosis, occurs in metaphyses and diaphyses with equal frequency. Epiphyseal location is unusual, with only 13 cases previously reported in the literature. The present case involves the epiphysis of the upper end of the left femur in a 12-year-old boy with pain and limp in the affected area. Radiographic findings were an oval, radiolucent rarefaction with nonsclerotic border, measuring approximately 3.5 x 3.0 cm. Microscopic examination identified aggregates of histiocytes, multinucleated giant cells, scattered eosinophils, and few plasma cells and lymphocytes. Ultrastructural studies demonstrated Birbeck granules within cytoplasm of histiocytes diagnostic of Langerhans cell histiocytosis. Whether Langerhans cell histiocytosis is a neoplastic disorder or a reactive process remains controversial. The true nature of the Langerhans cell, the proliferating cells and hallmark of this disease, is likewise under scrutiny.
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PMID:Langerhans cell histiocytosis involving epiphysis of a long bone. 1506 Aug 87

Langerhans' cell histiocytosis (LCH) is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often solitary bone lesion that occurs predominantly in the pediatric age group. The hallmark of LCH is the proliferation and accumulation of a specific histiocyte: the Langerhans' cell. In bone this may cause pain and adjacent soft-tissue swelling, but some lesions are asymptomatic. LCH can involve any bone, but most lesions occur in the skull (especially the calvarium and temporal bones), the pelvis, spine, mandible, ribs, and tubular bones. Imaging diagnosis of the disease in bone is first based on the plain radiographic appearance, which is usually a central destructive, aggressive-looking lesion. In the skull, the lesions develop in the diploic space, are lytic, and their edges may be beveled, scalloped or confluent (geographic), or show a "button sequestrum." Vertebral body involvement usually causes collapse, resulting in vertebra plana. With significant recent improvements in the quality of gamma cameras, imaging techniques, and in studying children, bone scintigraphy at diagnosis and on follow-up usually reveals the sites of active disease, especially when the involvement is polyostotic. CT and MR imaging are very useful in providing detailed cross-sectional anatomic detail of the involved bone, including the bone marrow and the adjacent soft tissues. CT is better suited for demonstrating bone detail and MR imaging for bone marrow and soft-tissue involvement.
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PMID:Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement. 1528 42

Langerhans' cell histiocytosis (LCH) can involve virtually any site and organ of the body, either as an isolated lesion or as a widespread systemic disease. Bone can be involved as a part of generalised disease or as a separate entity, formerly known as eosinophilic granuloma. The clinical cause of localised LCH (LLCH) to bone is generally benign. Solitary bone lesions may resolve spontaneously after diagnostic biopsy in a period of months to years. If treatment is indicated, as in larger lesions with pain, swelling and risk for spontaneous fracture, the disease can be controlled by chemotherapy or local measures such as surgical curettage, low-dose radiotherapy and intralesional injection of corticosteroids. There are no controlled studies in the literature comparing outcomes of these treatment modalities because of low incidence of the disease and general tendency to regress. Three patients (female, 28 months; male, 9 years; male, 15 years) with LLCH of the mandible were treated in an one stage procedure with intralesional injection of 80, 40 and 80 mg methylpredisolone succinate, respectively, as the primary form of treatment. Patients were seen for clinical and radiological evolution 1, 3, 6, 9 and 12 months after treatment, and yearly thereafter. The overall outcome was excellent. The lesions showed clinically and radiologically complete remission approximately 6 months after treatment. There were no complications nor morbidity of the treatment. After a follow-up period of 12 months, the patients are well with no evidence of residual disease on radiographs. Local injection of corticosteroids should be the initial choice of treatment of LLCH of the mandible, because of the favourable treatment outcome in this disease and possible complications and lasting effects of surgery, radiotherapy and chemotherapy.
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PMID:Intralesional infiltration of corticosteroids in the treatment of localised langerhans' cell histiocytosis of the mandible Report of known cases and three new cases. 1605 80

We describe the outcome of a 20-month-old female and a 6-year-old male, both of whom had acutely developed severe respiratory distress with tachypnea, cyanosis and, in Patient 2, thoracic pain. Chest X-ray and CT scan showed interstitial pulmonary involvement and a bullous process with bilateral pneumothoraces for both children. Pulmonary biopsy confirmed the diagnosis of Langerhans cell histiocytosis (LCH). Laboratory testing and skeletal radiography did not reveal any other involvement of LCH. The patients received chemotherapy (prednisone, vinblastine, 6-mercaptopurine). They had recurrent episodes of pneumothorax during follow-up and placement of chest tubes was the treatment chosen. They were asymptomatic, with regression of bullae and disappearance of pneumothorax at 58 and 63 months of follow-up, respectively. Pulmonary function tests done during follow-up were normal in both patients. Despite severe pulmonary involvement, conservative surgical treatment and moderate chemotherapy produced good results in these two rare cases.
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PMID:Isolated pulmonary Langerhans cell histiocytosis presenting with recurrent pneumothorax. 1620 6

We report a case of multisystem Langerhans cell histiocytosis (LCH) with lung, bone and pituitary involvement. A 20-year-old man developed thirst, polydipsia and polyuria in 1983. He had right femur pain from 1988 and osseous LCH was diagnosed based on the operated specimen in 1989. From July 1990, he had right chest pain on coughing and dyspnea and was admitted in November 1990. LCH involving the lungs was diagnosed by CT images and diabetes insipidus was also detected. Steroid therapy was started from 1991, but he discontinued it in 1998. Though he stopped smoking, his clinical symptoms worsened and he experienced bilateral pneumothoraces in 2002 and since then he has been receiving home oxygen therapy. Pulmonary LCH is thought to have a good prognosis, but in recent studies, its survival rate appears low. We report a case of multisystem LCH with lung deterioration over about 15 years.
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PMID:[A case of multisystem Langerhans' cell histiocytosis with lung deterioration over 15 years]. 1684 15

Langerhans' cell histiocytosis (LCH) is a rare condition of children and young adults in which Langerhans' cells proliferate. The clinical spectrum ranges from solitary or few focal lesions to multisystem involvement mimicking vasculitis or hematological malignancy. Focal bone lesions, known as eosinophilic granulomas, are the most common manifestations. Eosinophilic granuloma usually presents with a variable combination of pain, swelling, fracture, and fever. Facial bone involvement may manifest as an ear discharge, hearing loss, or exophthalmos. Nerve root pain is rarely reported, even in patients with lesions in the axial skeleton. We report four cases of nerve root pain caused by LCH. Two male patients aged 25 and 34 years, respectively, presented with truncated femoral neuralgia related to acetabular granulomas. A 25-year-old woman with involvement of the L5 vertebral body and a 41-year-old man with a sacral lesion presented with sciatica.
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PMID:Radiculopathy as a manifestation of Langerhans' cell histiocytosis. 1734 85

We report an unusual case of spinal Langerhans' cell histiocytosis of the cervicothoracic junction in a young adult man. A 17-year-old male was referred to our institution with a 3-week history of cervicothoracic pain associated to a weakness of his right upper limb. Computed tomography and magnetic resonance imaging showed a collapsed T1 vertebral body with epidural soft tissue showing mass effect on spinal cord. The patient underwent a classic anterior cervicotomy. Complete removal of the lesion could be achieved, but the soft consistency of C7 and T2 body precluded a solid anterior fixation and an extended resection of C7 and T2 body had to be performed. Then a C6 - T3 stabilisation using an anterior plate fixation and cyanomethylacrylate graft was performed. Postoperative course was uneventful. At 2 years follow-up, the patient was asymptomatic and radiological workup showed a perfect stability of anterior fixation system. Aggressive surgical management of eosinophilic granuloma should be considered in some selected cases particularly when spinal instability or neurological deficit occurs. In this young patient a modified anterior cervicotomy allowed a comfortable approach to the anterior aspect of T3 vertebra for spinal fixation.
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PMID:Spinal Langerhans' cell histiocytosis in a young adult: case report and therapeutic considerations. 1745 94

A 13-year-old Ethiopian female patient who initially presented with localized pain and minimal soft tissue swelling later developed pathological fracture of the left femnur. The patient was initially treated as a case of osteomyelitis until the diagnosis of Langerhans cell histiocytosis (histiocytosis X, eosiniphilic granuloma) was made by bone biopsy. The clinical presentation of the case and brief review of the literature is discussed.
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PMID:Unifocal langerhans cell histiocytosis presenting with pathological fracture. 1764 63

Eosinophilic granuloma is the most common expression of Langerhans cell histiocytosis and corresponds with typical bone lesions. This disease is of importance to dental professionals because early clinical signs can occur in the jaw and can cause extensive destruction of the periodontal tissues. Pathological fracture is an unusual finding. The purpose of this case report is to describe a case of eosinophilic granuloma in a 28-year-old black man who initially appeared with intense pain from a pathological mandibular fracture.
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PMID:Eosinophilic granuloma in the jaws. 1794 36


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