Clinical categories of Langerhans cell histiocytosis (LCH) include single and multi-system disease. Pulmonary LCH is rare, which is an unusual interstitial lung disease with the characteristics of monoclonal proliferation and infiltration of Langerhans' cells to organs. We report our experience of a rare LCH case of multiple organs such as pulmonary and liver as the main clinical manifestation. The patient was treated with chemotherapy which included prednisone, vinblastine, methotrexate and 6-mercaptopurine for 52 weeks and follow up all along. The patient has a favorable clinical outcome.
Histiocytosis, Langerhans-Cell ; diagnosis ; drug therapy ; Humans ; Infant ; Liver ; pathology ; Lung ; pathology ; Male ; Mercaptopurine ; therapeutic use ; Methotrexate ; therapeutic use ; Prednisone ; therapeutic use ; Vinblastine ; therapeutic use

Purpose of this study was to analyse the characteristics of clinical, iconographical, pathological and treatment methods of Langerhans cell histiocytosis (LCH), so as to improve the diagnosis and treatment level of this disease. The clinical data of 35 LCH patients were studied retrospectively. These patients were divided into 2 groups according to age <14 years old and ≥ 14 years old. The clinical symptoms were analysed and the signs, imageology and pathology manifestation and treatment results were evaluated. The results showed that LCH clinical manifestations were diverse and complex. Surgical treatment for patients with single system involvement of LCH was better than that of multi-system involvement of LCH (MS-LCH). For the latter, combined chemotherapy effects was better. After 3-year follow-up, 1-year OS was 94% ± 4%, 2-years OS was 91% ± 5%, 3-year OS was 86% ± 7%. 3 years OS of group <14 years old and ≥ 14 years old was 94% ± 6% and 81% ± 10% respectively. The OS of former was better than that of the later, but because a small number of cases, this difference was not statistically significant. It is concluded that LCH is easy to be misdiagnosed, the pathological biopsy is the gold standard of LCH diagnosis. The PET-CT can be of great help in identifying stages and finding lesion areas of the disease. Pulmonary Langerhans cell histiocytosis (PLCH) is more common in adult. Combined chemotherapy can improve the prognosis of the patients. The treatment methods should be choosed according to the stage and classification of disease.
Adolescent ; Adult ; Child ; Child, Preschool ; Female ; Histiocytosis, Langerhans-Cell ; diagnosis ; drug therapy ; pathology ; Humans ; Infant ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Young Adult

We present three cases of concurrent Langerhans cell histiocytosis (LCH) and B-lineage lymphoid cell infiltrations and/or nodules in the bone marrow. The first patient was a 25-month-old boy who presented with LCH on the right shoulder and multiple osteolytic lesions. Bone marrow biopsy showed the presence of LCH and two large lymphoid nodules of B-lineage, which were located in the paratrabecular region. Both LCH and the lymphoid nodules resolved after treatment with prednisone, vinblastine, methotrexate, and cyclophosphamide. The second patient was a 7-month-old girl who presented with LCH in the scalp and bone marrow. In spite of the treatment, a follow-up bone marrow analysis performed after 16 months showed LCH and increased B-lineage lymphoid cells in the interstitial area. The third patient was a 26-month-old girl, and imaging studies revealed reddish skin lesions and multiple osteolytic lesions. Skin biopsy and bone marrow biopsy did not show the presence of LCH; however, we initiated the treatment on the basis of the results of imaging studies. The follow-up study after 6 months showed the presence of LCH and large, patchy infiltration of B-lymphoid cells. We report three rare cases of concurrent bone marrow involvement of LCH and B-lineage lymphoid proliferation, which strongly suggest lymphoid malignancy. Further, clonal changes should be studied to elucidate the common pathogenic mechanism between the two diseases.
Antineoplastic Agents/therapeutic use ; B-Lymphocytes/immunology/*pathology ; Bone Marrow/immunology/*pathology ; Cell Proliferation ; Child ; Child, Preschool ; Cyclophosphamide/therapeutic use ; Drug Therapy, Combination ; Female ; Histiocytosis, Langerhans-Cell/*diagnosis/drug therapy/pathology ; Humans ; Male ; Methotrexate/therapeutic use ; Prednisone/therapeutic use ; Vinblastine/therapeutic use

Antineoplastic Agents ; therapeutic use ; Diagnosis, Differential ; Female ; Histiocytosis, Langerhans-Cell ; metabolism ; pathology ; Humans ; Interferons ; therapeutic use ; Leukemia, Mast-Cell ; metabolism ; pathology ; Mastocytosis, Cutaneous ; metabolism ; pathology ; Mastocytosis, Systemic ; diagnostic imaging ; drug therapy ; metabolism ; pathology ; Middle Aged ; Proto-Oncogene Proteins c-kit ; metabolism ; Radiography ; Radionuclide Imaging ; Spleen ; pathology ; surgery ; Splenectomy ; Tryptases ; metabolism