This report describes a case of adult temporal bone Langerhans cell histiocytosis（LCH） that presented as posterior canal dehiscence syndrome（PCDS）. The patient initially presented with vertigo, tinnitus, and hearing loss. Computed tomography（CT） revealed erosive changes in the mastoid and posterior semicircular canal. After the operation, the patient's hearing and vestibular symptoms disappeared and postoperative recovery was favorable. Adult LCH is relatively rare, and this case serves as an example to discuss the clinical features and treatment options of this disease, followed by a literature review.
Humans ; Histiocytosis, Langerhans-Cell/complications* ; Semicircular Canal Dehiscence/etiology* ; Temporal Bone/pathology*

Background: Pulmonary Langerhans cell histiocytosis (PLCH) is an interstitial primary pulmonary disease, characterized by Langerhans cell proliferation. It is easily misdiagnosed in children. This study aimed to characterize the clinical manifestations and features of PLCH by retrospective analysis. Methods: A retrospective analysis was performed in 117 PLCH patients out of 338 LCH patients who were admitted in our center from November 2006 to October 2013. Variables between two groups were compared by Mann-Whitney U-test and Chi-square test. Kaplan-Meier curves were constructed to compare the survival rates and Cox regression to evaluate the effect of risk factors. Results: The median age of PLCH group was significantly lower than that of non-PLCH group (18.63 months vs. 43.4 months, P < 0.001). All PLCH children had other organ involvement and only 11 cases (9.4%) had respiratory symptoms. The most common radiologic finding was cystic lesions (29 cases, 24.8%). Pulmonary function abnormalities were dominated by obstructive ventilatory dysfunction (63 cases, 82.9%). The 5-year overall survival (OS) of PLCH children was 93.6% ± 2.3% and the event-free survival (EFS) was 55.7% ± 5.2%. Among the 38 cases with progressed or relapsed disease, five cases (13.2%) were due to progression or recurrence of lung damage. The 5-year OS of PLCH children with "risk organ" involvement was significantly lower than those without "risk organ" involvement (86.0% ± 4.9% vs. 100%, χ = 8.793, P = 0.003). The difference of EFS between two groups was also significant (43.7% ± 7.7% vs. 66.3% ± 6.5%, χ = 5.399, P = 0.020). The "risk organ" involvement had a significant impact on survival (hazard ratio = 1.9, P = 0.039). Conclusions PLCH mainly occurs in young children, and only a small percentage of patients have respiratory symptoms. They generally have other organ involvement. Most of PLCH children have a good prognosis and most lung lesions could have improved or stabilized. Management of "risk organ" involvement is the key point to improving EFS.
Child ; Child, Preschool ; Female ; Histiocytosis, Langerhans-Cell ; complications ; diagnosis ; Humans ; Infant ; Langerhans Cells ; Lung ; physiopathology ; Lung Diseases ; etiology ; Male ; Retrospective Studies

OBJECTIVETo improve the recognition of the clinical presentation and radiologic manifestation of children with Langerhans cell histiocytosis (LCH) with pulmonary involvement.

METHODA retrospective analysis was conducted on children who presented with respiratory symptoms or abnormal lung radiologic findings, and finally diagnosed with LCH in Ward 2 of Divison of Respiratory Diseases, Beijing Children's Hospital during the last 4 years.

RESULTFourteen children (10 boys and 4 girls) were included in this study. Male to female ratio was 2.5: 1. The median age was 1.3 years. Pulmonary involvements were coexisted with other involved organs in all the patients, such as skin (10 cases, 71%), liver (8 cases, 57%), and bone involvement (7 cases, 50%). The most common symptoms were cough and fever (7 cases, 50%). Respiratory symptoms were nonspecific, and 3 children had no respiratory symptom but abnormal findings on lung high-resolution CT (HRCT). The most common HRCT finding was the coexistence of nodules and cysts (6 cases, 43%). Other findings include cysts only (5 cases, 36%), nodules only (1 case), and with neither nodule nor cyst (2 cases, 14%). Pneumothorax was found in 7% of children.

CONCLUSIONPulmonary involvement in children with LCH is easily misdiagnosed, and often coexisted with other involved tissues/organs such as skin and liver. Rash, which is easily missed in physical examination is very important for the diagnosis of LCH. The characteristic findings of lung HRCT (nodules and/or cysts) are helpful for diagnosis.

Child ; Cough ; etiology ; Cysts ; Diagnostic Errors ; Exanthema ; etiology ; Female ; Fever ; etiology ; Histiocytosis, Langerhans-Cell ; complications ; diagnostic imaging ; Humans ; Infant ; Liver ; Lung Diseases ; diagnostic imaging ; Male ; Retrospective Studies ; Skin ; Tomography, X-Ray Computed

OBJECTIVETo investigate the computed tomography (CT) manifestations of liver involvement in children with Langerhans cell histiocytosis (LCH).

METHODSRetrospective analysis was performed on 9 LCH children with liver involvement confirmed by clinical, laboratory and pathological examinations to investigate the CT manifestations of this condition. These children, including 6 males and 3 females, had undergone both plain CT scan and dual-phase (the arterial and portal venous phases) contrast-enhanced CT scan.

RESULTSThe main CT manifestations included hepatomegaly (8 cases); periportal dendritic hypodense lesions or "periportal halo sign" (7 cases) which were mildly or moderately enhanced in the arterial phase; intrahepatic bile duct dilatation (5 cases); lymphadenopathy in the hepatic hilar or retroperitoneal region (4 cases); and diffuse small hypodense nodules (3 cases), which showed annular enhancement on the contrast-enhanced CT scan.

CONCLUSIONSCT findings may be helpful in the early diagnosis and treatment of LCH in children.

Child ; Child, Preschool ; Female ; Hepatomegaly ; diagnostic imaging ; Histiocytosis, Langerhans-Cell ; complications ; diagnostic imaging ; Humans ; Infant ; Liver ; diagnostic imaging ; pathology ; Male ; Retrospective Studies ; Tomography, X-Ray Computed ; methods

Carcinoma ; complications ; Carcinoma, Papillary ; Female ; Histiocytosis, Langerhans-Cell ; complications ; Humans ; Middle Aged ; Thyroid Neoplasms ; complications

Administration, Oral ; Adolescent ; Female ; Histiocytosis, Langerhans-Cell ; complications ; drug therapy ; pathology ; Humans ; Lymph Nodes ; pathology ; Prednisone ; administration & dosage ; therapeutic use ; Retrospective Studies ; Skin Ulcer ; drug therapy ; etiology ; pathology ; Thalidomide ; administration & dosage ; therapeutic use ; Treatment Outcome

A 22-year-old man was referred to our institution due to lower back pain and was diagnosed with Langerhans cell histiocytosis of the thoracic and lumbar spine. The patient achieved complete remission with radiotherapy and chemotherapy. One year later, right cervical lymphadenopathy was observed and Hodgkin's lymphoma was confirmed on biopsy. The patient was treated with chemotherapy and autologous stem cell transplantation, and experienced no further symptoms. Further, no evidence of recurrence was observed on follow-up imaging. This report discusses the association between Langerhans cell histiocytosis and Hodgkin's lymphoma.
Antineoplastic Combined Chemotherapy Protocols ; Combined Modality Therapy ; Histiocytosis, Langerhans-Cell/*complications/diagnosis/therapy ; Hodgkin Disease/*complications/diagnosis/therapy ; Humans ; Male ; Stem Cell Transplantation ; Young Adult

Adult ; Cervical Vertebrae ; injuries ; Female ; Fractures, Spontaneous ; etiology ; Histiocytosis, Langerhans-Cell ; complications ; Humans ; Paralysis ; etiology ; Spinal Fractures ; etiology

The aim of this study was to analyze characteristics of chronic active Epstein-Barr virus (CAEBV) infection associated hematological disorders in children. Clinical characteristics were summarized; the morphology of hematopoietic cells in bone marrow was observed by microscopy; the lymphocyte subpopulations were analyzed by flow cytometry; the immunophenotype of liver biopsies was assayed by immunohistochemistry; EBV-related antibodies were measured by ELISA; serum EBV-DNA loads were detected by real-time quantitative PCR; EBV-encoded small RNA 1-positive cells in peripheral blood mononuclear cells were identified by in situ hybridization. The results indicated that the clinical manifestations in patients included persistent or recurrent fever, hepatosplenomegaly, liver dysfunction, anemia, thrombocytopenia, systemic inflammatory reaction. Bone marrow presented as hypocellularity, dysmaturation, myelodysplasia and hemophagocytosis. CD8(+) cell high counts were demonstrated in all 4 patients, one of them developed into a T cell lymphoma. Serum EBV-DNA load was 3.26 x 10(3) copies/ml in one patient, EBER1(+) cells were detected at a frequency of 1.7% in PBMNCs from another patient; the titers of IgG to EBV-VCA were >or= 1:5120 in the rest 2 patients. All 4 patients described above were diagnosed as CAEBV infection. In conclusion, the immune-related cytopenia, macrophage activation syndrome and lymphoproliferative disorders are characteristics of CAEBV infection associated hematological disorders in these 4 children patients.
Child ; Child, Preschool ; Chronic Disease ; Epstein-Barr Virus Infections ; complications ; immunology ; virology ; Female ; Hematologic Diseases ; immunology ; virology ; Histiocytosis, Non-Langerhans-Cell ; immunology ; virology ; Humans ; Lymphoproliferative Disorders ; immunology ; virology ; Male

Child, Preschool ; Diagnosis, Differential ; Histiocytosis, Langerhans-Cell ; complications ; diagnosis ; physiopathology ; Humans ; Male ; Thyroid Gland ; pathology ; Thyroid Nodule ; diagnosis ; etiology ; pathology