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)

Eighty-nine cases variously diagnosed as histiocytosis X, eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease, or malignant histiocytosis from one institution were reviewed. Follow-up data were obtained for all patients. On the basis of clinicopathologic correlation, the following distinct groups were identified. 1. Disseminated histiocytosis (12 patients) with involvement of more than two organ systems at the time of recognition of the disease, affecting children less than 3 years of age (the patient's general condition is poor and hepatosplenomegaly is common. The patients do poorly, and all the patients in this group of the present study died. 2. Histiocytosis confined to bone, whether monostotic (36 patients) or polyostotic (eight patients). This type is self-healing, does not disseminate, and does not require intensive therapy. Clinically the patients are older and in good general condition during the course of the disease. Histologically histiocytes in these two groups are cytologically benign, and the whole process is inflammatory rather than neoplastic. Presence of giant cells, eosinophils, and necrosis are usually associated with better prognosis. Many neoplastic diseases and infectious processes manifest as histiocytosis. More than a third of the cases of this study diagnosed as histiocytosis proved to be other processes. This confusion contributes to differences of various reports and difficulty of interpretation of findings.
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PMID:Idiopathic differentiated histiocytosis. 30 79

Histiocytosis X describes a disease characterized by histiocytic infiltration of the reticuloendothelial system, skin, bones, and pituitary gland. The disseminated form frequently occurs in infants and children. Chemotherapy has significantly improved the prognosis in this disorder. Sixty-three per cent of survivors, however, have some residual disability related to fibrosis of tissues previously infiltrated by histiocytes. In instances of liver involvement, healing by fibrosis may result in cirrhosis with portal hypertension and bleeding esophageal varices. Clinical findings include hepatosplenomegaly, jaundice, ascites, hypoalbuminemia, prolonged prothrombin time, and Bromsulphalein retention. Histologic examination of the liver shows a characteristic dense "macronodular" periportal cirrhotic pattern. Three children with portal hypertension and bleeding varices due to healed histiocytosis X were sucessfully managed by portosystemic shunt procedures. Portacaval, mesocaval, and central splenorenal shunts were equally effective in relieving poral hypertension. These children had neither recurrence of bleeding nor evidence of encephalopathy. Two children remain well whereas in one patient a primary hepatoma developed fourteen years posthung and he died of pulmonary metastases. Portosystemic shunt procedures effectively relieve the threat of potentially fatal variceal hemorrhage and improve the opportunity for long-term survival in children with cirrhosis and portal hypertension due to healed histiocytosis X.
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PMID:Portal hypertension in infants and children with histiocytosis X. 108 50

The case of a two-year-old girl with generalized histiocytosis, probably induced by phenobarbital, is reported. Symptoms, including intermittent fever, systemic lymphadenopathy, maculopapular skin eruption and hepatosplenomegaly, suggested Langerhans cell histiocytosis. Laboratory examinations revealed leukocytosis with lymphocytosis and eosinophilia and a high LDH serum level, while GOT and GPT were within normal ranges. Cytological studies of lymph node and pleural effusion specimens revealed proliferation and infiltration of Langerhans cell histiocytes with eosinophilia. No histiocyte proliferation was observed in the bone marrow or skin. The clinical manifestations shown by the patient were, however, transient, and improved spontaneously after the discontinuation of phenobarbital. The case was considered to be one of phenobarbital hypersensitivity syndrome based on clinical course and laboratory findings. The mechanism and differential diagnosis of the syndrome are discussed.
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PMID:A case of hypersensitivity syndrome resembling Langerhans cell histiocytosis during phenobarbital prophylaxis for convulsion. 129 59

Monozygotic twin boys presented at 1 year of age with seborrheic skin rash, otorrhea, and hepatosplenomegaly. Skin biopsy confirmed Langerhans cell histiocytosis. Treatment with conventional antineoplastic drugs and with calf thymus extract was ineffective. The disease remained refractory to recombinant human alpha-interferon and to low-dose total body irradiation, and the children died between 3 and 3 1/2 years of age.
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PMID:Disseminated Langerhans cell histiocytosis in identical twins unresponsive to recombinant human alpha-interferon and total body irradiation. 151 Jan 99

Familial hemophagocytic lymphohistiocytosis (FHL) is probably a genetically transmitted disease affecting infants and very young children. Cardinal symptoms are fever, hepatosplenomegaly, and pancytopenia. Frequently meningeal involvement is seen, manifested by neurologic symptoms and a lymphohistiocytic pleocytosis with increased protein levels in the cerebrospinal fluid. Characteristic laboratory findings in FHL are hypertriglyceridemia and hypofibrinogenemia, which are reversible with treatment. The disease has been rapidly fatal in most patients, but recently longterm remissions have been achieved with cytotoxic agents. Pathohistologic examination shows a widespread infiltrate of lymphocytes and mature macrophages with prominent hemophagocytosis affecting especially liver, spleen, lymph nodes and the central nervous system. Atrophy of the lymphatic tissue is a common finding. From the histologic picture FHL has to be grouped among the histiocytoses of reactive origin since the cells involved show no signs of malignancy. The etiology and pathogenesis of FHL are not known at present. Immunologic studies present evidence for a disturbed function of T lymphocytes, but a secondary immune defect seems to be more likely than primary immune deficiency. Among the broad clinical spectrum of histiocytic disorders especially histiocytic reactions due to infection, histiocytosis X and malignant histiocytosis have to be considered in the differential diagnosis of FHL.
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PMID:Familial hemophagocytic lymphohistiocytosis. 635 20

The case of an 81-year-old woman suffering for about 1 year from a typical form of Letterer-Siwe disease (LSD) is reported. The only important systemic involvement was hepatosplenomegaly. The patient was treated with steroids and vinblastine and then with thymopoietin pentapeptide. This last drug proved useful for decreasing the dose of steroid and thus for checking the appearance of new skin lesions. The ultrastructural investigations, carried out before and after vinblastine, showed the presence in some histiocytosis X cells (HXC) of coated Birbeck granules (BG). Immunoelectron microscopy showed about 90% of HXC to be OKT6+ and the OKT6 labeling of these cells to be similar to that of the Langerhans cells of normal skin. Five percent of OKT6+ cells apparently contained no Langerhans granules.
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PMID:Letterer-Siwe disease in an octogenarian. 654 37

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

Hospital records of seventeen patients (11 males & 6 females) with Langerhans cell histiocytosis, confirmed by electron microscopic demonstration of Birbeck granules, were studied retrospectively from October 1982 to October 1992 at Taichung Veterans General Hospital. The ages at presentation ranged from 5 months to 17 years (a median of 6 years). The clinical features were protean and included fever, pain, bony lesions, lung lesions, abnormal dentition, diabetes insipidus, oral ulcer, otorrhea, dermatitis, anemia, thrombocytopenia, lymphadenopathy, and hepatosplenomegaly. Skull and femur were the most common sites of bony lesions. The main therapeutic modalities were excision, radiotherapy, and chemotherapy with vincristine and prednisolone. The young age at presentation, several involved organ systems, presence of organ dysfunction, and clinical diagnosis with Letterer-Siwe disease were poor prognostic factors. Although Langerhans cell histiocytosis is not a rare disease, the cause is still unknown. It needs further research to disclose the mystery.
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PMID:Langerhans cell histiocytosis: a 10-year review. 794 24

We present a case of a patient with common variable immune deficiency presenting as the Letterer-Siwe syndrome (disseminated Langerhans cell histiocytosis). To our knowledge, this is the only known patient with this association. The clinical presentation was chronic diarrhea, weight loss, recurrent infections, hepatosplenomegaly, and interstitial pneumonitis. Laboratory evaluation revealed evidence of immunodeficiency, with agammaglobulinemia and diminished number and function of T cells. The diagnosis of Langerhans cell histiocytosis was confirmed by electron microscopic examination of the lung biopsy specimen demonstrating Birbeck granules in the cells of the infiltrate. It is known that patients with the combined immunodeficiency syndrome may present as disseminated Langerhans cell histiocytosis, and the case presented demonstrates that patients with common variable immune deficiency may similarly present. It is advisable that patients newly diagnosed with Langerhans cell histiocytosis be evaluated to screen for immunodeficiency. Conversely, patients presenting with combined immunodeficiency or common variable immune deficiency may display features of disseminated Langerhans cell histiocytosis. These associations must be considered in newly diagnosed immunodeficient patients.
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PMID:A 13-month-old child with chronic diarrhea, weight loss, and tachypnea. 821 98

A 28-month-old boy had firm, red nodules for 4 months that were mostly localized to the face. The eruption was preceded by conjunctivitis, eyelid edema, and swelling of the root of the nose. He also had dyspnea, anisocoria with areflexia, swelling of the parotid glands, and hepatosplenomegaly. A bone marrow aspirate showed 25% eosinophils. A skin biopsy specimen revealed a lymphohistiocytic infiltrate that involved the entire dermis. Most of the cells expressed antimacrophage markers; S-100 was negative. Electron microscopy showed poorly differentiated histiocytes without any specific marker. Steroid therapy induced complete clearing. Two years later, after several cutaneous recurrences, the patient is free of disease. We believe that this case represents a nodular, benign non-Langerhans cell histiocytosis with cutaneous lesions that differ from previously described histiocytopathies.
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PMID:An unusual case of non-Langerhans cell histiocytosis. 829


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