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

A case of therapy-related myelodysplasia followed by acute nonlymphocytic leukemia in a 5-year-old child successfully treated for diffuse Langerhans cell histiocytosis is described. A syndrome of severe cell-mediated immune deficiency and persistent Epstein-Barr virus (EBV) infection coincided with the evolution of myelodysplasia. Specific abnormalities of chromosomes number 7 and 3 were associated with the onset of myelodysplasia and acute nonlymphocytic leukemia and are believed to be linked to the patient's immune dysfunction and compromised ability to contain viral infection.
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PMID:Acquired immune deficiency, myelodysplasia, and acute nonlymphocytic leukemia associated with monosomy 7 and t(3;3) (q21;q26) in a child with Langerhans cell histiocytosis. 254 62

Langerhans cell histiocytosis (LCH) is an enigmatic histiocytic proliferative disorder of unknown etiology that affects children primarily. We have investigated the possibility that viruses are etiological or that they have a "triggering effect" in LCH. Sensitive in situ hybridization and polymerase chain reaction (PCR) techniques were used in 56 cases of LCH. We sought and failed to find evidence of genomes for adenovirus, cytomegalovirus, Epstein-Barr virus, herpes simplex virus, human herpesvirus type 6, human immunodeficiency virus, human T-cell leukemia virus types I and II, and parvovirus. Although some probes hybridized to tissues from several cases, PCR failed to confirm the presence of viral genome in any. We conclude that there is no evidence that these viruses are associated with LCH.
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PMID:Langerhans cell histiocytosis: lack of a viral etiology. 804 10

As a rare, sporadic disease Langerhans cell histiocytosis (LCH) presents a difficult problem in defining a likely etiology. Epidemiological data would not a priori lead one to choose a viral etiology. However, there are rare tumours which occur as sequelae of common infections from Epstein-Barr virus or human papilloma viruses. Likewise some viruses can cause cells to elaborate cytokines which could ultimately stimulate Langerhans cell growth. There is only a small amount of experimental data testing the hypothesis that viruses might be associated with LCH. The theoretical constructs surrounding this question and new data refuting the association are summarised.
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PMID:Viruses and Langerhans cell histiocytosis: is there a link? 807 3

We have examined the clinicopathological correlates of 74 patients with histologically confirmed Langerhans cell histiocytosis. Factors that influenced disease outcome included, three or more organ/systems being involved, a disease onset before the age of 2 years, the involvement of certain vital organs/systems such as liver/spleen, bone marrow and lungs, and male gender. The total number of involved organs/systems was the single most important determinant of disease outcome. Mortality rate in patients with three or more organs/systems involved, was 26%, as compared with 0% in the group with one or two organs/systems involved (chi 2 = 11.2, P = 0.008). There were no familial cases in our series, but we looked for a possible immunogenetic association by tissue typing 46 Caucasian sufferers and comparing the results with 117 controls. We used normal peripheral blood lymphocytes in 39 cases, Epstein-Barr virus-transformed lymphoblastoid cell lines in 12 cases, and both peripheral blood and Epstein-Barr virus-transformed lymphocytes in five cases. The HLA-B7 antigen was significantly increased Langerhans cell histiocytosis patients (19 of 46 = 41.3%) compared with 19 of 117 (16.2%) in the control group chi 2 = 11.2, relative risk = 3.6, P value after correction = 0.013). Attempt to stratify the disease into single-system or multisystem disease did not result in any significant association.
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PMID:Langerhans cell histiocytosis--clinicopathological reappraisal and human leucocyte antigen association. 877 56

The incidence, risk factors, and outcome of posttransplant lymphoproliferative disease (PTLD) were examined for 298 children undergoing liver transplantation. The overall incidence of PTLD was 8.4% (25 of 298). Intensity of immunosuppression was found to be a major risk factor for the development of PTLD. Cyclosporine and tacrolimus when used as primary immunosuppression were associated with the development of PTLD in 4.3% and 6.6% of cases (P=NS). OKT3 and tacrolimus, when used as rescue therapy for steroid-resistant rejection, were associated with a comparable increase in the risk of developing PTLD (10.9% and 11.1%, P=NS). Patients requiring both OKT3 and tacrolimus to treat refractory rejection were at significantly increased risk for PTLD (28.1% vs. 4.3% or 6.6%, P<0.0001). PTLD was more common in patients who received transplants for Langerhans cell histiocytosis relative to other indications for transplantation (66% vs. 8.4%, P=0.0005). The data also support an association between primary Epstein-Barr virus (EBV) infections following transplantation and the development of PTLD. While only three patients were EBV seropositive before transplantation (14%), 19 patients were EBV seropositive at the time of diagnosis of PTLD (90%), confirming a high incidence of primary EBV infections in patients with PTLD (21 patients had both pre- and posttransplant EBV serologies). In this series, PTLD was associated with a mortality rate of 60%, and 12 of the 15 patients who died had persistent tumor at the time of death. Five of the 13 patients rendered disease-free developed ductopenic rejection. Of the four with severe liver dysfunction, two have undergone successful retransplantation and are alive without evidence of PTLD. In conclusion, intense immunosuppression using OKT3 and tacrolimus as rescue agents was associated with a significant increase in the incidence of PTLD. Primary EBV infection after transplantation further accentuated this risk. Independent of these risk factors, patients with Langerhans cell histiocytosis were at significantly increased risk for PTLD. The identification of high-risk patients should allow the development of protocols to screen patients for primary EBV infections and early indications of PTLD, as well as the institution of preemptive antiviral and antitumor therapies.
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PMID:Posttransplant lymphoproliferative disease in pediatric liver transplantation. Interplay between primary Epstein-Barr virus infection and immunosuppression. 877 85

Langerhans cell histiocytosis (LCH) has been thought to be a disorder of immune regulation, and increasingly, evidence showing that the tissue damage in LCH involves lymphokines and pro-inflammatory cytokines is reported. We detected human cytomegalovirus (HCMV)-DNA in LCH cells in the foci of LCH lesions by immunohistochemistry, in situ hybridization and PCR. HCMV was detected in the nuclei and/or cytoplasm of LCH cells in 9 of 27 LCH cases by immunostaining. HCMV was probably an early antigen. In situ hybridization revealed signals for HCMV-DNA only in the nuclei of LCH cells in 10 of the 27 LCH cases. PCR analysis was performed in 20 of the LCH cases, and HCMV-DNA was detected in 7 of these. All 7 positive cases were also positive for HCMV by ISH and IHC. These findings suggested that early phase infection or reactivation of HCMV occurred in the LCH lesions. HCMV infection may be accompanied by impaired cytokine production. Our study also suggested a relationship between HCMV infection and expression of TNFalpha. In tissues affected by LCH, dermatopathic lymphadenopathy or malignant fibrous histiocytoma and in normal tissues no signals for Epstein-Barr virus-RNA were detected. These findings suggest that in some cases LCH is associated with HCMV infection.
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PMID:Human cytomegalovirus infection in foci of Langerhans cell histiocytosis. 1007 Dec 44

A role for viruses in the development or course of the main idiopathic chronic infiltrative lung disease has been suggested for a long time. Viruses that have usually been incriminated in cryptogenic fibrosing alveolitis are hepatitis C virus, whose role has not been accurately proven, and latent viruses including Epstein-Barr virus and adenovirus. These latent viruses might be re-activated in the lung of patients immunocompromised by treatments and might be accountable for disease progression. However, published studies have been very conflicting and the only clinical trial testing ribavirin has failed to demonstrate a beneficial effect. In sarcoidosis, the responsibility of human herpesvirus 6 and 8 and retroviruses has not been proven. Finally, data in the literature do not support a link between Langerhans cell histiocytosis and human herpesvirus 6 and 8. These viruses may act by several mechanisms: viral proteins may be antigens driving an appropriate immune response; they may also behave as transactivating factors to control the expression of various genes involved in immune response, cell growth or synthesis of matrix proteins.
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PMID:[Chronic infiltative lung disease and viruses]. 1146 86

Langerhans' cell histiocytosis (LCH) is a proliferative histiocytic disorder of unknown etiology. We previously reported that Epstein-Barr virus (EBV) infects and proliferates in macrophages, and investigated the possibility that EBV exhibits etiologic effects in LCH. To detect EBV expression, paraffin sections from 17 LCH cases were examined by mRNA in situ hybridization for EBV BamHIW, Epstein-Barr virus nuclear antigen-2 (EBNA2), and Epstein-Barr virus-encoded small nonpolyadenylated RNA (EBER1) sequences, and by indirect immunofluorescence staining for EBNA2, latent membrane protein 1 (LMP1), and BamHIZ-coding leftward-reading frame 1 (BZLF1). To detect EBV DNA, polymerase chain reaction (PCR)-Southern blotting was used. All cases showed positive hybridization signals by BamHIW mRNA in situ hybridization. Also, 13 and 14 cases showed positive signals for EBNA2 and EBER1 RNA in situ hybridization, respectively. Furthermore, almost all cases exhibited fluorescence after immunofluorescence staining with monoclonal anti-EBNA2 and anti-BZLF1 antibodies, and 15 cases were positive after treatment with monoclonal anti-LMP1 antibody. PCR-Southern blotting detected an amplified EBER1 sequence in all 9 cases examined. EBV expression was confirmed in LCH using in situ hybridization and immunofluorescence. Furthermore, EBV DNA was also detected by PCR-Southern blotting. These positive results of BZLF1 suggest that EBV replicates in LCH tissues.
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PMID:Expression of Epstein-Barr virus in Langerhans' cell histiocytosis. 1702 63

We report a case of Langerhans' cell histiocytosis (LCH) occurring after a living donor liver transplantation (LDLT) for fulminant hepatitis. A 9-month-old girl underwent an LDLT for fulminant hepatitis of an unknown cause. The histology of the native liver did not show any findings of LCH. On postoperative day 42, her Epstein-Barr virus (EBV)-DNA and cytomegalovirus antigenemia were both found to be positive. As a result, she was treated with antiviral agents and a reduction of the immunosuppression dosage. On postoperative day 98, acute rejection occurred, and she was treated with FK506, methylprednisolone, and finally, anti-CD3 murine monoclonal antibody was added. Subsequently, the EBV was re-activated. Thereafter, skin eruptions, swelling of the systemic lymph nodes, and pancytopenia appeared on postoperative day 127. LCH was diagnosed based on the typical histological findings as LCH, CD1a, and S-100-positive cells in her skin and a lymph nodes biopsy. She was treated by chemotherapy. The symptoms disappeared a few weeks after the start of the chemotherapy, and a clinical remission of LCH was obtained. We could not detect any evidence of EBV infection in the tumor cells. In spite of the fact that her LCH lesions thereafter remained in remission, she died of hepatic failure at 22 months after undergoing the liver transplantation. In conclusion, we discuss the factors influencing the occurrence of LCH in our patient after LDLT, while also evaluating the relationship between LCH and the immunosuppressive therapy administered to this patient.
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PMID:Langerhans' cell histiocytosis after living donor liver transplantation: report of a case. 1623 11

Epstein-Barr virus (EBV) is a herpesvirus for which latent infection in B lymphocytes occurs in most individuals by middle childhood. Clinically significant reactivation of this virus occurs in the context of suppressed cell-mediated immunity, occasionally developing into lymphoproliferative disease (EBV-LPD). EBV reactivation is rarely associated with intensive chemotherapy alone. Here we present the case of a 4-year-old female who developed EBV-LPD as a complication of prolonged immunosuppressive chemotherapy for her multiply-recurrent Langerhans cell histiocytosis (LCH).
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PMID:EBV+ lymphoproliferative disease following prolonged chemotherapy for refractory LCH. 1724 27


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