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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 non-Langerhans' cell histiocytosis that may present with pulmonary symptoms. The condition seems to be nonfamilial and typically affects middle-aged adults. Radiographic and pathologic changes in the long bones are diagnostic, but patients often present with extraskeletal manifestations. Advanced pulmonary lesions are associated with extensive fibrosis that may lead to cardiorespiratory failure. The clinical, radiologic, and pathologic features of six patients with ECD with lung involvement are presented. The patients were three men and three women (mean age, 57). Five presented with progressive dyspnea, and one presented with diabetes insipidus. Open-lung biopsies showed histiocytic infiltrates in a lymphangitic pattern with associated fibrosis and lymphoplasmacytic inflammatory infiltrates. The histiocytes did not stain with periodic acid-Schiff. Immunoperoxidase studies performed on specimens from five of six patients showed that the histiocytes were positive for CD68 and Factor XIIIa and negative for CD1a. Specimens from two patients exhibited immunoreactivity for S-100 protein. Electron microscopy studies performed on specimens from two patients showed phagocytic lysosomes but no Birbeck granules. Clinical follow-up of up to 16 years was available. At the end of that time, five patients were dead of complications related to their disease; one patient remains alive 4 years after diagnosis but with severe respiratory compromise. ECD is a rare non-Langerhans' cell histiocytosis that may present as interstitial lung disease and resemble other pulmonary conditions, particularly usual interstitial pneumonitis and pulmonary Langerhans' cell histiocytosis. Recognition of this entity will allow better assessment of its true incidence, therapeutic options, and prognosis.
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PMID:Pulmonary pathology of Erdheim-Chester disease. 1091 34

Langerhans cell histiocytosis (LCH) represents a poorly defined pathologic entity characterized by diverse clinical appearence and falling into two major categories namely a restricted and an extensive disease. Since the outcome and the course of the disease is variable, we postulated that this might be reflected by the phenotype of the Langerhans cells. We have selected 11 adult restricted cases and 10 extensive childhood cases and compared the phenotype of LCH cells by immunohistochemistry on paraffin sections. Morphometric analysis indicated a significantly higher expression of histiocytic (CD68, S-100, lysozyme) markers in the adult restricted cases compared to the extensive form of the disease. Both groups were equally positive for LCH marker CD1a and negative for T cell marker CD4. On the other hand, HLA-DR expression was significantly higher in LCH cells of the extensive childhood cases suggesting higher activation. These data suggest that LCH cells have a different phenotype in the extensive childhood and restricted adult LCH where the latter is characterized by a more differentiated histiocytic phenotype.
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PMID:Differential Expression of Markers in Extensive and Restricted Langerhans Cell Histiocytosis (LCH). 1117 5

Langerhans cell histiocytosis (LCH) consists of lesions composed of cells with a dendritic Langerhans cell (LC) phenotype. The clinical course of LCH ranges from spontaneous resolution to a chronic and sometimes lethal disease. We studied 25 patients with various clinical forms of the disease. In bone and chronic lesions, LCH cells had immature phenotype and function. They coexpressed LC antigens CD1a and Langerin together with monocyte antigens CD68 and CD14. Class II antigens were intracellular and LCH cells almost never expressed CD83 or CD86 or dendritic cell (DC)-Lamp, despite their CD40 expression. Consistently, LCH cells sorted from bone lesions (eosinophilic granuloma) poorly stimulated allogeneic T-cell proliferation in vitro. Strikingly, however, in vitro treatment with CD40L induced the expression of membrane class II and CD86 and strongly increased LCH cell allostimulatory activity to a level similar to that of mature DCs. Numerous interleukin-10-positive (IL-10(+)), Langerin(-), and CD68(+) macrophages were found within bone and lymph node lesions. In patients with self-healing and/or isolated cutaneous disease, LCH cells had a more mature phenotype. LCH cells were frequently CD14(-) and CD86(+), and macrophages were rare or absent, as were IL-10-expressing cells. We conclude that LCH cells in the bone and/or chronic forms of the disease accumulate within the tissues in an immature state and that most probably result from extrinsic signals and may be induced to differentiate toward mature DCs after CD40 triggering. Drugs that enhance the in vivo maturation of these immature DCs, or that induce their death, may be of therapeutic benefit.
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PMID:Differentiation of Langerhans cells in Langerhans cell histiocytosis. 1156 38

We describe the clinical and pathologic features of four cases of nodular histiocytic proliferation in the endometrium. We have been able to find only one brief reference to this lesion in the literature. The lesion in each case was a detached nodule composed of aggregates of histiocytes within a biopsy or curettage specimen. The constituent cells differed from foamy histiocytes of the endometrium in that they had either lobulated or ovoid, vesicular nuclei, distinctive cytoplasmic margins, and a moderate amount of amphophilic cytoplasm. Mitoses were frequent (up to 11 per 10 high-power fields) in one case but were absent in the remaining cases. On immunohistochemical staining, CD68 and lysozyme were strongly expressed in the cytoplasm. Neither estrogen receptor nor progesterone receptor was expressed in contrast to the background endometrium. The cells were also negative for S-100 and cytokeratin. Each patient's postcurettage course was uneventful. The cause of nodular histiocytic proliferation of the endometrium is currently unknown, although response to intracavitary debris has been suggested. The lesion should not be confused with a variety of reactive, inflammatory, or neoplastic conditions, such as xanthogranulomatous endometritis, malakoplakia, histiocytic granuloma, hormonal changes of the endometrial stroma, Langerhans' cell histiocytosis, morular metaplasia, extravillous trophoblast, or exaggerated placental site reaction.
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PMID:Nodular histiocytic hyperplasia of the endometrium. 1191 23

A 29-year-old woman had a 2-month history of an enlarging lesion over her left frontal bone following minor trauma. CT scan showed an osteolytic lesion with an overlying soft tissue mass, thought to be an unhealed skull fracture with pseudomeningocele. Left frontal craniotomy revealed a soft tissue mass, which was resected. Histologic examination revealed multinucleated giant cells mixed with Langerhan's cells that showed the characteristic "coffee bean nuclei." Eosinophils were scant. Immunostaining for CD1a and S100 revealed strong positive staining primarily in the Langerhans' cells while giant cells and inflammatory cells were negative. Immunostaining for CD68, in contrast, stained the osteoclast-like giant cells and macrophages. Electron microscopy confirmed the presence Birbeck granules. The final diagnosis was Langerhans' cell histiocytosis (histiocytosis X) of the skull.
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PMID:February 2002: 29-year-old woman with a skull mass for 2 months. 1214 8

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis. Herein we describe documented skeletal and pericardial involvement by ECD producing cardiac tamponade in a 30-year-old woman. The diagnosis of ECD was established by histopathology, immunocytochemistry, and by radiologic studies demonstrating diffuse, bilateral, symmetrical osteosclerosis of the long bones, sparing the epiphyses and axial skeleton. Scintography using methyl diphosphonate showed increased uptake in involved bone. The patient presented with jaundice and hepatic congestion produced by cardiac tamponade. Pericardial biopsy revealed xanthogranulomatous lesions comprised of foamy and lipid-laden macrophages, multinucleated giant cells, monocytes, and lymphocytes in a mesh of fibrosis. Immunohistochemical staining was positive for CD68 and negative for CD1a, consistent with ECD rather than with the much more common Langerhans cell form of histiocytosis.
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PMID:Erdheim-Chester disease with prominent pericardial involvement: clinical, radiologic, and histologic findings. 1218 13

The May 2002 COM. A 38-year-old man presented with new onset seizures and a 69-year-old woman presented with bilateral headaches and episodes of syncope. Both were found to have extra-axial masses that were contrast-enhancing and thought to be meningiomas. Both had complete resection. Microscopic examination revealed an inflammatory lesion composed of plasma cells, scattered lymphocytes and numerous large histocytic cells, which exhibited emperi polesis and were CD1 a negative, but positive for CD68 and S100. The diagnosis of Destombes-Rosai-Dorfman Disease (DRDD) was rendered. Both cases had good long-term outcome. The differential diagnosis of inflammatory masses in the dura (plasmacytoma, lymphomas, plasma cell fibroma, angiofollicular hyperplasia [Castleman's-disease] and Langerhan's cell histiocytosis) are discussed.
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PMID:May 2002: 38-year-old man and 69-year-old woman with dural based masses. 1240 40

Juvenile xanthogranulomas (JXG) is a histiocytic disorder, primarily but not exclusively seen throughout the first two decades of life and principally as a solitary cutaneous lesion. This study is a retrospective clinical and pathologic review of 174 cases documenting the cutaneous and extracutaneous manifestations in patients presenting from the neonatal period to 20 years of age (mean 3.3 years; median 1 year). There was a male predominance (99 male:75 female) in all categories of clinical presentation, but especially notable in the group with multiple cutaneous lesions (12 male:1 female). A solitary cutaneous lesion accounted for 67% of all cases, followed by a solitary subcutaneous or deep soft tissue mass (28 cases, 16%), multiple cutaneous lesions (13 cases, 7%), a solitary extracutaneous, nonsoft tissue lesion (9 cases, 5%), and multiple cutaneous and visceral-systemic lesions (8 cases, 5%). The recorded deaths due to disease included two neonates with systemic JXG who developed hepatic failure and thrombocytopenia and at autopsy had giant cell-neonatal hepatitis in addition to JXG in the liver and other visceral sites. A third death in a 3-month-old boy with a retroperitoneal-pelvic JXG occurred after failure to control severe hypercalcemia. The characteristic Touton giant cell in variable numbers was a consistent feature of the cutaneous lesions; however, these cells were either absent or present in reduced numbers in the various extracutaneous lesions when compared with JXG in the skin. Spindle cells intermingled among the mononuclear cells or forming short fascicles were seen in both cutaneous and extracutaneous lesions. Immunohistochemistry was performed on all extracutaneous lesions, and the constituent cells, regardless of their individual morphologic features, were uniformly positive for vimentin, CD68, and factor XIIIa and negative for S-100 protein and CD1a. It is widely held that JXG is a proliferative disorder of dendrocytes, possibly dermal dendrocytes; thus, its clinical and pathologic similarities to Langerhans cell histiocytosis are not entirely unexpected in light of the most recently proposed international classification of histiocytic disorders, which includes JXG and Langerhans cell histiocytosis together as "dendritic cell-related" histiocytoses.
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PMID:Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. 1271 44

Langerhans cell histiocytosis (LCH) is a neoplastic proliferation of Langerhans cells that occurs in a range of nodal and extranodal sites. Scattered reports of LCH within the thymus exist, typically among children within the setting of multifocal, multisystem disease. Rare cases of isolated LCH involving the thymus have occurred in adult patients with myasthenia gravis. We report a case of unifocal LCH involving the thymus in a middle-aged woman with a history of a resected leiomyosarcoma but no evidence of myasthenia gravis. Computed tomographic scans revealed an anterior mediastinal mass, which was excised and measured 9.0 cm. Histologic and immunophenotypic findings (CD1a, S100, and Fascin positive and CD68 negative) were consistent with LCH. To our knowledge, this is the first example of LCH occurring in a patient with a history of soft tissue sarcoma and one of the rare reported examples of LCH presenting as a large isolated lesion in the thymus of a nonmyasthenic adult.
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PMID:Langerhans cell histiocytosis involving the thymus. A case report and review of the literature. 1282 60

We make a retrospective evaluation of clinical and radiologic features, treatment, and outcome of Erdheim-Chester disease, a rare non-Langerhans cell histiocytosis. We report a case of Erdheim-Chester disease and review 60 cases from the literature. These cases are consider to have Erdheim-Chester disease when they have either typical bone radiographs (symmetrical long bones osteosclerosis) and/or histologic criteria disclosing histiocytic infiltration with distinctive immunohistochemical phenotype of the non-Langerhans cell histiocytes with positive staining for CD68 and negative staining for S-100 protein and CD1a. Our patient undergoes chemiotherapy according to the LCH-II stratification and therapy plan (Vinblastine, Etoposide and Prednisone) and thereafter receives Carboplatin and Etoposide, and Somatostatin. She is alive and clinically well 33 months after onset of symptoms and the lesions don't appear to progress at imaging examinations. In conclusion, Erdheim-Chester disease may be confused with Langerhans cell histiocytosis as it sometimes shares the same clinical (exophthalmos, diabetes insipidus) or radiologic (osteolytic lesions) findings. However, the characteristics radiological pattern of Erdheim-Chester disease together the immunohistochemical phenotype of hystiocytic infiltration supports the theory that Erdheim-Chester disease is a unique disease entity distinct.
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PMID:[Erdheim-Chester disease: a non-Langerhans cell histiocytosis. A clinical-case and review of the literature]. 1534 69


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