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)

In 3 patients with histiocytosis X the following complications are described: 1. A 16 year old boy suffered from generalized histiocytosis in infancy. He died after acute intracranial hypertension caused by a basilar impression. 2. Recurrent spontaneous pneumothoraces was the first symptom of an initial exclusively pulmonary histiocytosis X. 3. Exophytically growing soft tissue tumors in the late disease state of a progressive histiocytosis X.
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PMID:[Unusual complications in the course of histiocytosis X (author's transl)]. 30 67

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

Optimal treatment for Langerhans Cell Histiocytosis (LCH) has not yet been established. Preliminary reports suggest some effect of cyclosporine (CSA), both alone or in combination with steroids and/or vinblastine, in untreated cases. Twelve children (6 females and 6 males, age at diagnosis 3 months to 4 years) with biopsy proven, systemic LCH received oral CSA (12 mg/kg/day in two divided doses given daily) as a second-line therapy following chemotherapy including vinblastine and/or etoposide (10 cases) or steroid alone (one case); one child was not pretreated. A total of 16 CSA courses were administered to the 12 patients: 8 were completed, 4 were interrupted as unsuccessful, and 4 are still ongoing. CSA related toxicity consisted of hypertrichosis and transient hypertension and was never limiting. Treatment was associated with a clinical response in 8/12 patients: 3 had a complete response and are off therapy, and 5 had a partial response; disease reactivation following first favorable response required additional CSA courses in 3 patients. Four patients failed to respond to CSA: two died of progressive disease, while two had a favorable response to CSA + VP16. Favorable response to CSA was not related to CSA trough and peak levels and was usually observed during the first 2 weeks of CSA therapy. CSA is effective for treatment of LCH also in pretreated children with progressive disease including life-threatening organ dysfunction. Long-lasting complete remission may be achieved after 6 to 12 months of CSA therapy. When disease reactivation occurs after treatment withdrawal, a second course may be followed by favorable response. As minimal or no adverse effects are observed during or after prolonged CSA therapy, this may also be safely used in young patients.
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PMID:Cyclosporine therapy for refractory Langerhans cell histiocytosis. 775 96

We report the case of a man with biopsy-proved pulmonary Langerhans' cell granulomatosis (histiocytosis X) and pulmonary precapillary hypertension. Pulmonary vascular resistances and mean pulmonary artery pressure decreased with corticosteroid therapy and dyspnea improved.
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PMID:Steroid-responsive pulmonary hypertension in a patient with Langerhans' cell granulomatosis (histiocytosis X). 868 45

Benign intracranial hypertension is known to be associated with obesity, endocrine abnormalities, various medications, and cerebral venous sinus thrombosis. We report a patient presenting with headaches and vomiting attributed to benign intracranial hypertension. The diagnostic work-up revealed Langerhans' cell histiocytosis of the occipital bone. There was no evidence for cerebral vein thrombosis by cranial computed tomography scan, Doppler ultrasonography, planar and single photon emission computed tomography technetium 99m-labelled red blood cell scintigraphy, and magnetic resonance angiography. Excision of the occipital bone lesion and a short course of acetazolamide and prednisone were curative. We hypothesize that cytokines secreted by the tumor were responsible for the development of intracranial hypertension.
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PMID:Langerhans' cell histiocytosis presenting as intracranial hypertension. 1141 6

Despite the introduction of new drugs that have changed the course of pulmonary arterial hypertension (PAH), some patients are still refractory to treatment and deteriorate rapidly. Long-acting phosphodiesterase-5 inhibitors are a new class of drugs that are effective in PAH. This prospective study assessed the potential of combination therapy with prostacyclin and tadalafil for treatment of severe PAH. We report four cases of severe PAH that deteriorated despite prostacyclin therapy. Two patients had Eisenmenger syndrome, one had pulmonary hypertension associated with scleroderma and one had histiocytosis X. All were treated with tadalafil, 10-20 mg once daily, in addition to prostacyclin. After 3 months of treatment, all patients improved clinically, with an increase in mean 6MWD from 214 to 272 m. In three patients, the New York Heart Association functional class decreased from IV to III. Echocardiograms showed no significant changes in pulmonary arterial pressure. Although this study was limited by the small sample size, it suggests that tadalafil in combination with prostacyclin is an effective treatment for severe PAH. Tadalafil may be beneficial for the treatment of patients with advanced disease.
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PMID:Combination therapy with prostacyclin and tadalafil for severe pulmonary arterial hypertension: a pilot study. 1881 91

Pulmonary veno-occlusive disease (PVOD) is currently classified as a subgroup of pulmonary arterial hypertension (PAH) and accounts for 5-10% of cases initially considered to be idiopathic PAH. PVOD has been described as idiopathic or complicating other conditions, including connective tissue diseases, HIV infection, bone marrow transplantation, sarcoidosis and pulmonary Langerhans cell granulomatosis. PVOD shares broadly similar clinical presentation, genetic background and haemodynamic characteristics with PAH. Compared to PAH, PVOD is characterised by a higher male/female ratio, higher tobacco exposure, lower arterial oxygen tension at rest, lower diffusing capacity of the lung for carbon monoxide, and lower oxygen saturation nadir during the 6-min walk test. High-resolution computed tomography (HRCT) of the chest can be suggestive of PVOD in the presence of centrilobular ground-glass opacities, septal lines and lymph node enlargement. Similarly, occult alveolar haemorrhage is associated with PVOD. A noninvasive diagnostic approach using HRCT of the chest, arterial blood gases, pulmonary function tests and bronchoalveolar lavage could be helpful for the detection of PVOD patients and in avoiding high-risk surgical lung biopsy for histological confirmation. PVOD is characterised by a poor prognosis and the possibility of developing severe pulmonary oedema with specific PAH therapy. Lung transplantation is the treatment of choice. Cautious use of specific PAH therapy can, however, be helpful in some patients.
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PMID:Pulmonary veno-occlusive disease. 1911 30

Pulmonary hypertension is a pathophysiologic condition characterized by the increase of mean pulmonary arterial pressure > or =25 mmHg. A concomitant increase of pulmonary wedge pressure >15 mmHg may be present (post-capillary pulmonary hypertension) or not (precapillary pulmonary hypertension). The increase of pulmonary arterial pressure and of pulmonary vascular resistance and consequent elevation of the right ventricular afterload lead to right ventricular failure after variable periods of time. Pulmonary hypertension is present in multiple clinical conditions which have been classified in five groups. Pulmonary arterial hypertension (group 1) includes the familial and the idiopathic form and the forms associated with anorexigen drug use, connective tissue diseases, congenital heart diseases, HIV infection and portal hypertension. Group 2 includes all left heart diseases characterized by the increase of left atrial pressure and pulmonary wedge pressure (post-capillary pulmonary hypertension). Group 3 includes parenchymal lung diseases (chronic obstructive lung disease, lung fibrosis, ecc). Chronic thromboembolic pulmonary hypertension (group 4) is characterized by the obstruction of elastic pulmonary arteries at different levels by organized thromboembolism. Group 5 includes heterogeneous conditions such as sarcoidosis and histiocytosis X. These clinical groups are characterized by different pathobiologic and pathophysiologic mechanisms and therapeutic strategies. The exact pathobiologic mechanisms leading to pulmonary arterial hypertension (group 1) are unknown. Genetic factors (inheritable forms), predisposing factors (female gender) and exogenous factors (drugs, antibodies, viruses, congenital heart disease, etc). Endothelial dysfunction of lung microcirculation is invariably present and is characterized by the reduction of vasodilator and antiproliferative substances (prostacyclin, nitric oxide) and by the increase of vasoconstrictor and mitogenic factors (endothelin, thromboxane A2). Current approved therapies are targeted to the correction of this imbalance, which leads to the progressive increase of pulmonary vascular resistance. Different therapeutic strategies that are effective in diverse groups require an appropriate diagnostic algorithm in order to identify the precise group and specific conditions within the group. Evaluation of vasoreactivity and assessment of the severity of functional and hemodynamic changes are also required in pulmonary arterial hypertension for an appropriate therapeutic decision-making and estimate of results.
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PMID:[Pulmonary arterial hypertension. Part I: pathobiologic, pathophysiologic, clinical and diagnostic aspects]. 1953 40

Langerhans' cell histiocytosis is a disease usually found in children and characterized by idiopathic proliferation of histiocytes in the reticuloendothelial system. Intracranial Langerhans' cell histiocytosis presenting as multifocal intraparenchymal lesions is very rare. In this article, the authors report on a 4-year-old boy diagnosed with multifocal intraparenchymal Langerhans' cell histiocytosis concomitant with an arachnoid cyst. After a series of laboratory examinations, the right frontal mass was surgically excised. Histological examinations confirmed the diagnosis of intracranial Langerhans' cell histiocytosis. The patient's intracranial hypertension symptoms were alleviated, and the remaining foci were treated by Langerhans' cell histiocytosis-directed standard chemotherapy. At the 8-month follow-up visit, no recurrence of the excised lesion was found, and no change in the size of other lesions was seen. Supratentorial intracerebral lesions with mass effect and enhancement have rarely been described; in this report, the histological features of and therapeutic options for such a case are discussed.
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PMID:Multifocal intraparenchymal Langerhans' cell histiocytosis concomitant with an arachnoid cyst in a child: case report and review of the literature. 2209 14

To report a rare case of nonatherosclerotic renal artery disease (NARAD) in a patient with Erdheim-Chester disease (ECD), a non-Langerhans cell histiocytosis. The patient is a 75-year-old male who presented with 2 years of hypertension secondary to right renal artery occlusion incidentally found on cardiac catheterization. Open bilateral renal revascularization was performed because of improved long-term graft patency demonstrated in other forms of NARAD. Nine months postoperatively, the patient's hypertension was significantly improved and surveillance duplex demonstrated patent bilateral renal artery bypasses. Revascularization in patients with NARAD, such as ECD, should be managed in an open approach because of the superior long-term graft patency and blood pressure control in a disease where progression to vessel fibrosis and atherosclerosis could interfere with endovascular stent patency.
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PMID:Bilateral renal artery involvement of erdheim-chester disease. 2470 54


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