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

Class I Langerhans cell histiocytosis (LCH) is described in a two- and a half-year-old male. The initial expression of the disease was conspicuous by the presence of extensive pustular crusted rash and, later in its course, by purpura, diabetes insipidus, otitis media, enlargement of the liver and spleen, and infiltration into the lungs. The patient expired due to extensive hemorrhage over 24 hours.
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PMID:Langerhans cell histiocytosis: a case report and glimpses into its nomenclature. 760 83

Langerhans cell histiocytosis (LCH) is a class I histiocytosis characterized by the presence of the pathologic Langerhans cell, an unique histiocyte. In contrast to LCH, class II histiocytosis is characterized by the proliferation of mononuclear phagocytes other than Langerhans cells and includes sinus histiocytosis with massive lymphadenopathy, viral-associated hemophagocytic syndrome, and familial hemophagocytic lymphohistiocytosis. Until now, these two classes have been considered separate, if related, entities. We report a 10-month-old girl who presented with pyrexia, hepatosplenomegaly, an eczematous skin rash, anemia, thrombocytopenia, and a markedly elevated serum IgG and IgM antibody level to cytomegalovirus. Histologic proof of both hemophagocytosis in the liver and bone marrow and LCH in the skin was obtained at presentation. The clinical course and response to treatment over 6.5 years is recorded. Although the etiology of both class I and class II histiocytosis remains unknown, we speculate that the monocytic/macrophage disorder, as well as the LCH, were both triggered by virus or viral-related monokines secreted by activated macrophages.
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PMID:Simultaneous occurrence of viral-associated hemophagocytic syndrome and Langerhans cell histiocytosis: a case report. 762 81

Disseminated histiocytosis X with cutaneous and lymph node involvement was diagnosed in a 25-year-old women. The diagnosis was established on the basis of a positive cell-surface staining with OKT 6 and typical signs on electron microscopy. Both the specific skin rash and lymph node swelling completely disappeared during pregnancy, but recurred 2 weeks before delivery. Therapeutic trials with 0.25 mg ethinylestradiol/day and a later application of human chorionic gonadotropin up to 5000 IU i.m. twice weekly as well as prednisolone 25 mg three times per day were unsuccessful. A second pregnancy was not desired. Polychemotherapy with initial high-dose prednisolone plus vincristine and a consolidation therapy with 5-mercaptopurine 300 mg/day led to full recovery. The observation of transient remission of histiocytosis X during pregnancy suggests that at least some forms of this disease may have prevailing immunological features where an immunosuppressive effect of pregnancy could be beneficial.
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PMID:Spontaneous transient remission of disseminated histiocytosis X during pregnancy. 786 Jun 20

Diffuse histiocytosis X involving thyroid gland is rare. A 10-year-old boy with progressively enlarged neck mass, skin rash over scalp and face, splinter hemorrhage, otitis externa, episodes of pneumothorax and abnormal liver function was hospitalized. Thyroid scan showed a nonfunctioning mass in the right lobe of the thyroid and needle aspiration biopsy suspected histiocytosis X. Right thyroid lobectomy was performed and revealed histiocytosis X in light and electron microscopic examinations.
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PMID:Histiocytosis X involving thyroid gland: a case report. 798 41

A 20-month-old girl was diagnosed with Langerhans cell histiocytosis on the basis of a seborrheic skin rash, multiple punched out bony lesions, and skin biopsy findings. Combination therapy including alpha-interferon, vincristine, vindesine, cyclophosphamide, etoposide, cisplatin, betamethasone, THP-adriamycin, cytarabine and methotrexate was ineffective. Because cyclophosphamide enhanced lesion growth within the skull, we administered an intravenous infusion of interleukin-2 with remarkable efficacy. The reduction in lesion size with interleukin-2 treatment paralleled the increase in the percentage of CD16-positive natural killer cells in the peripheral blood.
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PMID:Interleukin-2 therapy of Langerhans cell histiocytosis. 856 40

Langerhans cell histiocytosis is an infrequent and enigmatic proliferative disorder that commonly presents in the head and neck region. This is an analysis of 77 patients with Langerhans cell histiocytosis treated at Children's Hospital and Dana Farber Cancer Institute from 1974 through 1993. The study focused on clinical findings, anatomic location and extent of disease, therapy, and outcome. The patients were, on average, under 5 years of age at initial presentation. Over 62 percent of the patients had signs and symptoms referred to the craniofacial skeleton. Osteolytic lesions of the cranium were the most common, followed, in frequency, by scalp rash, osteolytic mandibular tumor(s), enlarged nodes, and gingival swelling or ulceration. Single bony lesions usually were treated with curettage or radiotherapy. Chemotherapy was used commonly for advanced disease with multifocal or disseminated presentation. Initial therapy included moderate doses of single agents; other agents were added if no response was achieved. The natural history of Langerhans cell histiocytosis varied from an acute fulminant course, a waxing and waning chronic disease, to spontaneous regression. Young age at presentation and organ dysfunction predicted a poor prognosis. Statistical analysis showed that there was no significant relationship between outcome and extent of skeletal involvement when controlling for age or organ dysfunction.
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PMID:Langerhans cell histiocytosis: an uncommon disease commonly manifesting in the craniofacial skeleton. 876 8

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.
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PMID:Non-Langerhans cell histiocytosis associated with lymphocyte-predominant Hodgkin's disease. 1056 22

Langerhans' cell histiocytosis is a disease of the dendritic histiocytes with a wide variety of clinical manifestations. This report describes a boy with Langerhans' cell histiocytosis who presented with primarily neurologic and endocrinologic findings, without pain. The diagnosis of Langerhans' cell histiocytosis was not made until 10 years after symptom onset. The pathology database at Mayo Clinic was searched for cases of Langerhans' cell histiocytosis between 1985 and 1999 under 19 years of age (65 children), and information regarding clinical presentation was abstracted. Database review found a range of 1 day to 156 weeks (mean 13.8 weeks) from symptom onset to diagnosis. No other patients with primarily neurologic symptoms were found. The diagnosis of Langerhans' cell histiocytosis was made significantly sooner after onset if pain was present (chi-square = 19.1, P < .001, two-tailed, phi coefficient 0.54). Our findings indicate that neurologic manifestations of Langerhans' cell histiocytosis are rare, and the combination of diabetes insipidus, ataxia, skin rash, or osseous pain should alert the clinician to the possibility of Langerhans' cell histiocytosis and avoid delayed diagnosis.
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PMID:Delayed diagnosis of pediatric Langerhans' cell histiocytosis: case report and retrospective review of pediatric cases seen at Mayo Clinic. 1145 55

Langerhans' cell histiocytosis (LCH) is a rare paediatric disease of unknown etiology affecting 1 to 5 children per 1 million each year. It is characterized by the idiopathic proliferation of Langerhans' cells. The clinical spectrum of disease is quite varied, ranging from a solitary eosinophilic granuloma to diffuse multisystem involvement. The head and neck is the most common site of involvement, occurring in approximately 60% of LCH patients. Head and neck manifestations are diverse and include skull and temporal bone lesions, cervical lymphadenopathy, and skin rash. Diagnosis can be difficult as these lesions mimic other common conditions seen by the otolaryngologist, including otitis externa, acute mastoiditis, and gingivitis. A retrospective study was carried out to study our centre's experience with LCH over the last 10 years. Twenty-one patients were diagnosed between January 1990 and December 1999. Patient's age at time of diagnosis ranged from 6 days to 14 years. Fifty-seven percent of patients had localized bony lesions; the remaining 43% had diffuse multisystem disease. The head and neck was also the most commonly involved site in our study, affecting 67% of our patients. Presentation and diagnosis of these lesions are discussed in detail. Treatment, complications, and patient outcomes will also be discussed.
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PMID:Langerhans' cell histiocytosis in the paediatric population: presentation and treatment of head and neck manifestations. 1277 59

We present a very rare congenital immunologic disease, severe combined immunodeficiency syndrome (SCID) in 6-months-old-boy with prolonged mucocutaneous candidiasis, severe anaemia, skin rash similar to the infiltrative eczema of Langerhans cell histiocytosis (LCH) and subcutaneous nodules with histiocytic infiltration. Laboratory findings show profound absence of humoral and cell-mediated immunity. Pathology specimens analysis of subcutaneous nodule revealed numerous S-100 protein and Cd1a negative histiocytes, occupied by BCG intracellular growth. Histopathology and immunohistochemistry confirmed the diagnosis of BCG dissemination. BCG vaccination in infants with SCID can lead to life threatening dissemination, resembling to the infiltrative eczema of LCH and may mislead the clinician.
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PMID:Disseminated BCG infection resembling langerhans cell histiocytosis in an infant with severe combined immunodeficiency: a case report. 1555 21


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