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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 is a rare non-Langerhans' cell histiocytosis with characteristic radiological and histological features. This entity is defined by a mononuclear infiltrate consisting of lipid laden, foamy histiocytes that stain positively for CD68. About half of those affected have extraskeletal manifestations, including involvement of the hypothalamus-pituitary axis, lung, heart, retroperitoneum, skin, liver, kidneys, spleen, and orbit. This report describes the case of a 50 year old white man who presented with hypogonadism and diabetes insipidus. At necropsy, extensive organ involvement was found, including the testes, thyroid, and lymph nodes. This is the first report of thyroid and lymph node infiltration in this disease. Because of the endocrinological symptoms, neurosarcoidosis and hypophysitis are important diseases in the differential diagnosis. This report also includes a review of the literature concerning rare organ manifestations and patients presenting primarily with similar symptoms.
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PMID:Erdheim-Chester disease: case report with multisystemic manifestations including testes, thyroid, and lymph nodes, and a review of literature. 1550 91

Hemophagocytosis (HP), a feature seen in malignant histiocytosis and infection- and lymphoma-associated disorders, has not been previously emphasized in Erdheim-Chester disease (ECD). Generally, ECD is recognized as a rare, systemic, non-Langerhans cell histiocytosis with a variable clinical course. Herein, we describe a unique case of multisystem non-Langerhans cell histiocytic proliferation with a fulminant clinical course (death occurred within 3 months of presentation) that showed prominent HP and extensive involvement of multiple organs, including the lungs, resulting in respiratory failure. Hemophagocytosis led to severe anemia that required transfusion and thrombocytopenia. Antemortem lung and bone marrow biopsy specimens revealed involvement by a histiocytic infiltrate with features highly suggestive of ECD and HP. Furthermore, the autopsy documented the presence of HP and the histiocytic infiltrate in multiple other organs. This case is best categorized as a variant form of ECD. Recognizing this variant has the following important implications: (1) HP may be a marker for fulminant clinical course in ECD, (2) the presence of HP does not exclude a diagnosis of ECD, and (3) ECD should be considered in the differential diagnosis of HP.
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PMID:Fulminant multisystem non-langerhans cell histiocytic proliferation with hemophagocytosis: a variant form of Erdheim-Chester disease. 1567 46

Erdheim-Chester disease (ECD) is a rare, progressive, non-Langerhans cell histiocytosis that portends a poor prognosis. Misdiagnosis is common until adequate tissue analysis reveals the infiltrative histiocytic process. The disease process affects various organ systems, and effective treatment options are limited. Knowledge of the constellation of signs and symptoms associated with ECD is important to avoid misdiagnosis. We describe a patient who underwent laparoscopic bilateral ureterolysis and laparoscopic biopsy for presumed retroperitoneal fibrosis confirmed previously by percutaneous needle biopsy findings. The final pathologic diagnosis based on laparoscopic biopsy results was ECD. As evidenced by this case, ureterolysis offers little benefit to patients with ECD.
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PMID:Laparoscopic biopsy and ureterolysis in Erdheim-Chester disease. 1581 94

Erdheim-Chester disease is a rare systemic non-Langerhans cell histiocytosis. We report the first case of surgical treatment of severe compression of renal parenchyma by retroperitoneal masses in a 61-year-old male patient with progressing renal failure. After 3 years of follow-up, we have concluded that the open surgical approach is an option in the management of renal complications in Erdheim-Chester disease.
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PMID:Compression of kidneys in Erdheim-Chester disease of retroperitoneum: Open surgical approach. 1583 40

Erdheim-Chester disease is a disseminated non-Langerhans' cell histiocytosis involving multiple organs with characteristic sclerotic musculoskeletal lesions. This is the report of the case of a 53-year-old woman with extensive and progressive pulmonary disease. Computed tomography scans revealed diffuse infiltrative lung disease. Thoracoscopic lung biopsy and a biopsy of the right femur lesion were performed. The histopathology revealed that she had non-Langerhans' cell histiocytosis; Erdheim-Chester disease. The characteristic lesions of Erdheim-Chester disease, including involvement of the orbit, pericardium, periaorta, and bone were detected. This helped to further confirm that the patient had Erdheim-Chester disease with associated pulmonary involvement. As Erdheim-Chester disease is a rare non-Langerhans' cell histiocytosis that may be misdiagnosed as interstitial lung disease or other pulmonary disorders, this diagnosis should be considered in the differential diagnosis of such lung lesions.
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PMID:Pulmonary involvement in Erdheim-Chester disease. 1595 55

Erdheim-Chester disease (ECD) is a rare xanthomatous non-Langerhans cell histiocytosis which involves the marrow space of the long bones. Extraosseous sites most commonly affected include the eyes, lungs, pituitary glands, and kidneys. We report the case of a 49-year-old woman who presented with palpable breast nodules, followed by progressive soft tissue and subcutaneous disease, and involvement of the long bones, dysarthria, and dysphagia. The histopathologic features and skeletal radiography findings are consistent with ECD. This case represents an unusual presentation, which led to delayed diagnosis, as ECD of the breast has been rarely reported. ECD should be considered in the differential diagnosis of histiocytoid breast lesions, including fat necrosis and histiocytoid invasive mammary carcinoma.
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PMID:Erdheim-Chester disease of the breast: a case report and review of the literature. 1629 93

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by tissue infiltration of lipid-laden macrophages, multinucleated giant cells, and inflammatory infiltrate of lymphocytes and histiocytes. The disease typically involves long bone, but may also affect the central nervous system, the orbit, retroperitoneal organs, and the lungs. Patients with visceral involvement tend to have poorer outcome. There is no proven effective treatment for ECD to date. However, recent data suggested the potential use of bisphosphonates for the treatment of this rare disease. Here we report a case of biopsy-proven skeletal ECD, who received treatment with zoledronic acid, an aminobisphosphonate, with remarkable clinical improvement. We also discuss possible mechanisms of action of bisphosphonates in this disorder, especially their roles in inhibition of inflammatory cytokines and macrophage infiltration.
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PMID:Treatment of skeletal Erdheim-Chester disease with zoledronic acid: case report and proposed mechanisms of action. 1693 56

Erdheim-Chester disease is a rare disorder, belonging to the group of histiocytoses, in which diffuse infiltration of histiocytes affects various organs and systems. Bone involvement in Erdheim-Chester disease manifests as generalized sclerosis of the bone marrow and cortex of the long bones, and this peculiar radiologic characteristic differentiates it from other histiocytoses. Diagnostic suspicion of the disease derives from the pulmonary and bone radiologic findings as well as from the clinical findings. Histological study reveals histiocyte infiltration affecting the soft tissues, musculoskeletal system, and central nervous system. The definitive diagnosis is reached by immunohistochemistry. Like other histiocytoses, such as Langerhans cell histiocytosis, immunohistochemical techniques reveal lipid-laden histiocytes; however, unlike the other types, Erdheim-Chester histiocytes stain negatively for S 100 protein and do not contain Birbeck granules.
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PMID:[Radiologic diagnosis of Erdheim-Chester disease. A case report]. 1716 44

Erdheim-Chester disease is a non-Langerhans cell histiocytosis that is progressive and may lead on to multi-organ involvement. Pulmonary involvement is rare, its presentation is nonspecific, and it carries an adverse outcome. Several radiological features, when considered together, may point to the diagnosis. This condition should be considered in the differential diagnosis of interstitial lung disease. We describe a 39-year-old woman who presented with dry cough, malaise and progressive dyspnoea. She was diagnosed to have late stage interstitial lung disease due to Erdheim-Chester disease.
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PMID:Erdheim-Chester disease: a rare cause of interstitial lung disease. 1730 81

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis with a characteristic long bone lesion. Although about half of the patients have extraosseous organs, pulmonary involvement is very rare. We report a 55-year-old man with ECD who complained of severe dyspnea despite home oxygen therapy with noninvasive positive pressure ventilation. Continuous PGI2 administration was not very effective, but administration of cyclophosphamide and prednisolone induced rapid improvement of respiratory failure and the effect for six months on arterial blood gas analysis and stability of the disease state persisted.
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PMID:[A case of Erdheim-Chester disease effectively treated by cyclophosphamide and prednisolone]. 1731 26


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