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

An unusual case of Langerhans cell histiocytosis in a 7-year-old female is presented. She had ultrastructural evidence of desmosomal biogenesis and formation of gland-like structures by lesional cells; their apical plasma membranes were folded into large numbers of microvilli. Despite the presence of these structures characteristic of epithelial cells, an infiltrated plaque on the abdominal skin of this patient was interpreted as cutaneous involvement of multiple system Langerhans cell histiocytosis because the immunohistochemical staining of the lesional cells for CD1a, S100, PNA, CD4, EN-4, and HLA-DR was positive, and numerous Birbeck granules were ultrastructurally identified in some lesional cells. Other clinical data included the presence of scaly erythematous skin lesions on the forehead and lytic osseous lesions in the maxilla, which were also histologically diagnosed as Langerhans cell histiocytosis. The absence of any internal malignancy in this patient readily ruled out the other diagnostic possibility of a metastatic adenocarcinoma showing glandular differentiation with brush border morphogenesis. The possibility that the desmosome-linked lesional epithelioid cells were actually cells of sweat glands entrapped in the histiocytic proliferation was also ruled out. The functional significance of the desmosomes and microvillous structures in the present case of Langerhans cell histiocytosis remains to be clarified. Awareness of this variant of Langerhans cell histiocytosis will be important for averting potential misdiagnosis in favor of epithelial tumors, especially metastatic adenocarcinomas.
 ...
PMID:Langerhans cell histiocytosis (histiocytosis X) with morphologic expression of desmosomes and microvillous structures. 924 66

We report on two children with malignancy who showed fungemia despite the antifungal treatment with fluconazole. Case 1 was a 7-year-old girl with a recurrence of stage IV neuroblastoma. She had profound neutropenia and fungemia developed after a month-long treatment with fluconazole. Her peripheral blood smear showed phagocytosis in the neutrophils and they were identified as fungi by immunofluorescence method (Fungi flora Y). She died two days after the diagnosis of fungemia. Rhodotorula rubra was isolated after her death. Case 2 was a 2-year-old boy with disseminated Langerhans cell histiocytosis. He had profound neuropenia and fungemia developed after treatment with fluconazole for 6 months. His peripheral blood smear also showed phagocytosis in the neutrophils and they were identified as fungi by Fungi flora Y. He was treated with intravenously administered amphotericin-B. However, he died 13 days after the diagnosis of fungemia. Candida guilliermondii was isolated after his death. Careful observation of the peripheral blood smear is important for early detection of fungi and Fungi flora Y is a quick and useful method to identify fungi. Fluconazole-resistant fungus should be considered when patients with neutorpenia are treated prophylactically with fluconazole for a long time.
 ...
PMID:[Phagocytosis of fungi in the peripheral blood neutrophils of two children with cancer during treatment with fluconazole]. 931 Dec 73

We present the case of a 19-year-old male who suffered from Langerhans cell histiocytosis (LCH) 12 months after having been treated for recurrent Hodgkin's disease (HD). Immunophenotypic characterization and electron microscopic analysis were useful for the exclusion of a bone relapse of Hodgkin's disease or any other differential diagnosis. The association of LCH with HD or other malignancies is rare but more frequent than previously believed. The significance of such an association and the pathophysiology of LCH are still open questions.
 ...
PMID:Langerhans cell histiocytosis arising after Hodgkin's disease. 938 12

We present the cytogenetic investigations of five histiocytic tumour lesions from children. In four cases there was a confirmed diagnosis of Langerhans cell histiocytosis (LCH) and one case of histiocytosis that did not fulfil all the criteria for true LCH. All five cases showed cytogenetic abnormalities, including the first report of an abnormal clone in LCH. The clone showed a t(7;12)(q11.2;p13) translocation and was detected in only a small percentage of cells. This case and a further three also contained non-clonal abnormalities and an increase in chromosome breakage. The fifth case, the only one in which no acquired abnormalities were seen, had a constitutional paracentric inversion of chromosome 13q.
Br J Cancer 1998 Feb
PMID:Cytogenetic abnormalities in Langerhans cell histiocytosis. 948 10

Langerhans cell histiocytosis in the adult is rare, but it is important to recognize its occurrence, as it must be differentiated from lymphoma, myeloma, and a variety of skin conditions and endocrinopathies. It has been reported in patients up to the ninth decade of life, and occurs equally in men and women. Local disease has a good prognosis, but associated diseases--particularly malignancy--may be the cause of death in some adults. The optimal treatment is not known. Coordinated investigation of the epidemiology and therapy of this disease is needed.
 ...
PMID:Langerhans cell histiocytosis in adults. 956 99

The frequency of Langerhans cell histiocytosis (LCH) and a malignant neoplasm occurring in the same individual appears to be greater than previously recognized. To define the occurrence and the pattern of these events, a Study Group of the Histiocyte Society initiated a registry of patients in whom this association occurred synchronously or asynchronously. Evaluation of 54 patients detected two patterns of associations between LCH and other disorders. First, it is possible that therapy of LCH promotes a secondary malignancy. Second, it is possible that a genetic predisposition, with or without the immunosuppression associated therapy for the malignancy, plays a role in the development and expression of disseminated LCH. Data collected by the LCH-Malignancy Study Group may provide insights into the etiology and pathophysiology of LCH.
 ...
PMID:The relation of Langerhans cell histiocytosis to acute leukemia, lymphomas, and other solid tumors. The LCH-Malignancy Study Group of the Histiocyte Society. 956 6

The development of therapy-related acute myeloid leukemia (t-AML) has become a growing concern over the past decade, because of the increase in the percentage of long-term survivors of primary malignancy. We reviewed 17 cases with etoposide-related acute promyelocytic leukemia (APL) reported in the literature. The close association between treatment with etoposide for Langerhans cell histiocytosis (LCH) and the development of etoposide-related APL was demonstrated among Japanese and Italians. Our data on the breakpoints (b/ps) of the PML and RARalpha genes are presented. It is suggested that chromatin structure might be more important than specific consensus sequence in the distribution of b/ps in etoposide-related APL.
 ...
PMID:Etoposide-related acute promyelocytic leukemia. 969 69

Langerhans cell histiocytosis is an interesting disorder with a variety of presentations and variable outcomes. This study evaluates response to treatment, recurrence, and survival in disseminated Langerhans cell histiocytosis treated at Regional Cancer Centre, Trivandrum, India from 1983 through 1994. Thirty-five patients with disseminated Langerhans cell histiocytosis were seen. Six had group A disease, 21 had group B disease, and eight had group C disease. In group A, five of six patients are disease free at a median follow-up of 48 months. Two had recurrence after initial treatment, which was salvaged. In group B, 13 of 15 patients had complete response after chemotherapy, nine of whom experienced recurrence later. Three of five patients who received irradiation alone experienced recurrence. One died of progressive disease. Two patients were lost to follow-up. Seventeen of 20 are alive with median follow-up of 67 months. In group C, one of eight patients are alive after multiple recurrences. Of the surviving patients, 29% had significant sequelae. In summary, group A and B patients do well with treatment, and most of the recurrences can be salvaged. A significant proportion of patients have sequelae. Newer aggressive protocols must be developed for treating group C patients. Measures to prevent sequelae must also be developed.
 ...
PMID:Disseminated Langerhans cell histocytosis in children: treatment outcome. 1019 56

The spectrum of histopathologic changes in four cases of chronic recurrent multifocal osteomyelitis encountered in our orthopedic outpatient clinic in the past 3 years was studied in conjunction with clinical and radiologic findings. All presented with pain with or without swelling in the affected region. Radiographically, the appearance of the lesions varied from a mixed picture of bone lysis and sclerosis with expansion to sclerosis alone to bone collapse. Bone scintigraphy demonstrated asymptomatic and separate foci of activity in all cases. Prior to biopsy, the clinical and radiologic differential diagnoses included Ewing's sarcoma, metastatic neuroblastoma, hematolymphoid malignancy, Langerhans cell histiocytosis and chronic infection, notably tuberculosis. The spectrum of histopathologic changes ranged from acute (acute inflammatory infiltration, active bone resorption and necrosis, reactive bone formation) to subacute (predominantly lymphocytic and plasma cell infiltration) to chronic inflammation (fibroblastic organization and bony sclerosis). Histologic changes correlated poorly with clinical features, but relatively well with radiologic findings. Lesional excision was performed in one case, cortical saucerization in another, while the final two cases received supportive treatment. All remained well 18-21 months post-therapy. Chronic recurrent multifocal osteomyelitis is a great clinical and radiologic mimic, which merits recognition by the pathologist. Awareness of the spectrum of histologic features encountered enables a correct diagnosis to be made in the appropriate clinical setting. The patient can thus be reassured of a favorable prognosis.
 ...
PMID:Chronic recurrent multifocal osteomyelitis: a great clinical and radiologic mimic in need of recognition by the pathologist. 1023 Jun 89

We report a 74-year-old man who presented with a rash on the trunk showing clinical and histological features of non-Langerhans cell histiocytosis. Two years after presentation he developed weight loss, lymphadenopathy and hepatosplenomegaly; a diagnosis of lymphocyte-predominant Hodgkin's disease was made on lymph node biopsy. The cutaneous signs and lymphoma responded to chemotherapy. Taken in conjunction with previously published reports of associations between cutaneous histiocytoses and haematological malignancies, we recommend close observation of patients in whom a diagnosis of non-Langerhans cell histiocytosis is made.
 ...
PMID:Non-Langerhans cell histiocytosis associated with lymphocyte-predominant Hodgkin's disease. 1056 22


<< Previous 1 2 3 4 5 6 7 8 9 10