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)

We have prospectively evaluated the feasibility and results of the biotin-avidin immunoadsorption method (Ceprate SC system) for a phase I/II study of T-cell depletion of granulocyte colony-stimulating factor (G-CSF) mobilized peripheral blood progenitor cells (PBPC) for allogeneic transplantation. Twenty consecutive patients, median age, 40 years (21 to 54) and diagnoses of chronic myeloid leukemia in chronic phase (n = 5), acute myeloblastic leukemia (n = 7), acute lymphoblastic leukemia (n = 2), chronic myelomonocytic leukemia (n = 1), refractory anemia with excess of blasts in transformation (n = 3), histiocytosis X (n = 1), and chronic lymphocytic leukemia (n = 1), were conditioned with cyclophosphamide (120 mg/kg) and total body irradiation (13 Gy; 4 fractions). HLA identical sibling donors received G-CSF at 10 microg/kg/d subcutaneously (SC); on days 5 and 6 (19 cases) and days 5 to 8 (1 case) donors underwent 10 L leukapheresis. PBPC were purified by positive selection of CD34+ cells using immunoadsorption biotin-avidin method (Ceprate SC) and were infused in the patients as the sole source of progenitor cells. No growth factors were administered posttransplant. The median recovery of CD34+ cells after the procedure was of 65%. The median number of CD34+ cells infused in the patients was 2.9 (range, 1.5 to 8.6) x 10(6)/kg. The median number of CD3+ cells administered was 0.42 x 10(6)/kg (range, 0.1 to 2). All patients engrafted. Neutrophil counts >500 and >1,000/microL were achieved at a median of 14 days (range, 10 to 18) and 15 days (range, 11 to 27), respectively. Likewise, platelet counts >20,000 and >50,000/microL were observed at a median of 10 days (range, 6 to 23) and 17 days (range, 12 to 130), respectively. Graft-versus-host disease (GVHD) prophylaxis consisted of cyclosporine plus methylprednisolone. No patient developed either grade II to IV acute or extensive chronic GVHD. After a median follow-up of 7.5 months (range, 2 to 22) three patients have relapsed, and one of them is again in hematologic and cytogenetic remission after infusion of the donor lymphocytes. Two patients died in remission: one on day +109 of pulmonary aspergillosis and the other on day +251 of metastasic relapse of a previous breast cancer. Sixteen of the 20 patients are alive in remission after a median follow-up of 7.5 months (range, 2 to 22). In conclusion, despite the small number of patients and limited follow-up, it appears that this method allows a high CD34+ cell recovery from G-CSF mobilized PBPC and is associated with rapid engraftment without significant GVHD, and with low transplant related mortality.
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PMID:Rapid engraftment without significant graft-versus-host disease after allogeneic transplantation of CD34+ selected cells from peripheral blood. 916 34

The ground-glass pattern is a common but nonspecific finding on CT. In certain clinical circumstances, it can suggest a specific diagnosis, indicate a potentially treatable disease, and guide a clinician to an appropriate area for biopsy. A pattern of centrilobular ground-glass nodules is fairly specific for the diagnosis of hypersensitivity pneumonitis with the appropriate clinical history. The tree-in-bud pattern indicates disease affecting the small airways. The differential diagnosis is lengthy; however, the most common process leading to this CT appearance is infection. Although commonly associated with M. tuberculosis, many infectious organisms can produce this pattern. When honeycombing is seen on HRCT, a confident diagnosis of lung fibrosis can be made. The most common causes of interlobular septal thickening on HRCT are pulmonary edema, pulmonary hemorrhage, and lymphangitic spread of cancer, and smooth thickening is characteristic of all three. Diffuse lung cysts in patients who are not immunocompromised generally signify Langerhans' cell histiocytosis, lymphangioleiomyomatosis, or centrilobular emphysema. Centrilobular emphysema can be diagnosed when the centrilobular artery is seen as a small nodular opacity in the center of the cyst. Langerhans' cell histiocytosis is often associated with parenchymal nodules, helping to distinguish it from lymphangioleiomyomatosis. When a nodular pattern is seen on HRCT, the differential diagnosis is very long, but can be narrowed by noting whether the nodules are random, centrilobular, or perilymphatic in distribution. A mosaic pattern of lung attenuation can represent an infiltrative, small airway, or vascular process. The distinction can often be made by noting the size of the pulmonary vessels in the abnormal areas of lung, and whether air trapping is present on expiratory scanning. Computed tomographic signs can be useful indicators of a specific disease process. For instance, the air bronchogram sign indicates that an opacity is intrapulmonary in location, and signals the possibility of two types of neoplasm: lymphoma and bronchioloalveolar cell carcinoma. An air crescent sign indicates recovery of the immune system in an immunocompromised patient with invasive pulmonary aspergillosis. The fallen lung sign is diagnostic of a bronchial transection in the correct clinical setting. The gloved finger sign is very suggestive of allergic bronchopulmonary aspergillosis. The halo sign is highly suggestive of early angioinvasive pulmonary aspergillosis in patients with acute leukemia. When a split pleura sign is seen, the diagnosis is often empyema, although other causes of pleuritis can lead to a similar CT appearance.
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PMID:CT signs and patterns of lung disease. 1169 64

The prognosis of multisystem LCH in children with risk organ involvement is extremely poor when they fail to respond to conventional chemotherapy. In such patients, allogeneic SCT may produce complete and sustained remission; however, high-dose myeloablative regimens are frequently associated with treatment-related morbidity and mortality. More recently, allogeneic SCT following an RIC regimen has been performed as an alternative salvage approach. We describe a nine-month-old boy with refractory multisystem LCH with pulmonary aspergillosis who was successfully treated with reduced-intensity cord blood transplantation.
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PMID:Successful treatment of refractory Langerhans cell histiocytosis with pulmonary aspergillosis by reduced-intensity conditioning cord blood transplantation. 1917 14

A 40-year-old man was diagnosed with Langerhans cell histiocytosis (LCH) in October 2010. His LCH was refractory to conventional chemotherapy, and thus worsened to Langerhans cell sarcoma (LCS) in May 2011. Although we repeated combination chemotherapies, new infiltration of the liver and bone marrow, as well as primary lesions of the bone, lymph nodes, and skin, appeared. These intensive chemotherapies caused candida liver abscesses, invasive aspergillosis, disseminated varicella zoster virus infection and bacterial sepsis. We administered bendamustine for chemotherapy, which resulted in a partial response (PR) with no severe adverse events. Because of pancytopenia caused by secondary myelodysplastic syndrome, we stopped the bendamustine chemotherapy after two courses. PR was maintained for 4 months. We plan to perform allogeneic hematopoietic stem cell transplantation from a sibling donor after a conditioning regimen. Optimal therapy for adult LCH, which is a rare and treatment-resistant disease, has yet to be established. Bendamustine is a potential chemotherapeutic agent for standard treatment of LCS.
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PMID:Clinical experience of bendamustine for adult Langerhans cell sarcoma. 2488 23

Treatment-related myelodysplastic syndrome (t-MDS)/acute myeloid leukemia (AML) is a devastating early or late complication of treatment for childhood cancer related with a significant morbidity and mortality. We retrospectively studied survivors of childhood cancer. Overall, 287 patients were recorded in the databases and we identified three (1.04%) with t-MDS. The primary cancer diagnoses were Langerhans cell histiocytosis (one patient) and acute lymphoblastic leukemia (ALL; two patients). The mean age of patients was 12.1 years. All patients had received systemic antifungal treatment for invasive pulmonary aspergillosis successfully treated with voriconazole and liposomal amphotericin B before diagnosis of t-MDS. Two patients (66%) remain alive after a median follow-up period of 3.5 years.
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PMID:Treatment-related myelodysplastic syndrome (t-MDS)/acute myeloid leukemia (AML) in children with cancer: A single-center experience. 2764 64