ABSTRACT
Background: Langerhans cell histiocytosis (LCH) is a rare clonal malignancy of the monocyte-macrophage system. Patients with lesions in 搑isk organs� have significantly higher risk of mortality than patients with lesions limited to 搉on-risk� sites. The influence of early response to therapy on long-term survival in this heterogeneous multi-system disease was analyzed. Methods: During a 7-year period, we retrospectively analyzed the findings in 24 consecutive patients who required systemic chemotherapy for LCH [single system with multifocal bone involvement and multisystem involvement with or without risk organ (RO) involvement]. All patients were started on vinblastine and prednisolone. Progressive disease was treated with salvage protocols or targeted therapy. Positron emission tomography-computed tomography (PET-CT)/conventional CT based response assessment was performed at week 6 of chemotherapy, and if needed after week 12 of chemotherapy. Results: MFO bone, MS ROneg, and MS ROpos LCH was observed in 3, 4, and 17 patients, respectively. Age range of patients varied from 1 month�years (median = 18 months). The EFS and OS were 100% and 100% for MFO bone, 50% and 100%, respectively, for MS ROneg and 35% and 52%, respectively, for MS ROpos. OS was 93% and 100% for CR attained at 6 and 12 weeks respectively regardless of the risk status (P < 0.01). Conclusion: Rapid early response, that is, complete remission at 6 and 12 weeks was associated with significantly improved overall survival. In slow responders, early salvage with alternative regimens or targeted therapy may result in better outcomes
ABSTRACT
Background: Tissue diagnosis of Childhood interstitial lung diseases is of paramountimportance to outline management. Case characteristics: A 10-year-old boy withprolonged cough, and computed tomography of thorax with features suggestive ofprimary Langerhans’s cell histiocytosis. Intervention: Transbronchial cryobiopsy of lungusing flexible cryoprobe, revealed a final diagnosis of Surfactant protein C/ABCA3deficiency. Message: Transbronchial cryobiopsy of the lung can provide adequate lungtissue for a categorical diagnosis of interstitial lung diseases in children.
ABSTRACT
Langerhan cell histiocytosis, formerly known as histiocytosis X, traditionally denotes a group of diseases that stem from proliferative reticuloendothelial disturbances.The etiology and pathogenesis of the disease remain debatable. In this paper we report a case of Langerhan cell histiocytosis in a 3 1 / 2 -year-old child who reported to the department of oral pathology with gingival enlargements. The radiological features and histopathological features are also discussed.
ABSTRACT
Histiocytosis Syndromes of Childhood (HSC) are a group of rare and diverse disorders characterized by aggressive proliferation or accumulation of cells of monocyte - macrophage system of bone marrow. The clinical spectrum of this syndrome is distinctly varied. The exact pathophysiology of HSC is yet to be determined; however, evidence suggests that one of the subtypes, Hemophagocytic Lymphohistiocytosis, is due to decreased Natural Killer cell activity, resulting in increased activation of other T cell subtypes and production of cytokines. We present four cases of HSC managed at our center between October 2008 & February 2010.
ABSTRACT
Objective To investigate the relationship between the density of langerhan cell(Lc) and Tcell (Tc) and the histological classification, biological behavior and prognosis of esophageal carcinoma.Methods The immunohistochemical (ABC) method with S-100 and CD3 staining was used in samples ofprimary esophageal cancer in 249 cases for quantitative analysis. All cases were followed up for more than7 years. We observed the morphology,number and distribution of langerhan cell (Lc) and T cell (Tc) indifferent portionof the tumor mass. Results Lc positive expressed in the nucleus and cytoplasma. To positiveexpressed in cytomembrane. The number of Lc in cancer nest had positive correlation with malignantdegree of tumor, infiltration depth and metastasis of lymph node, and had negative correlation with thesurvival period. On the contrary, the number of To and Lc in peritumor interstitial tissue had negativecorrelation with the survival period. Conclusion The distribution density of Lc and Tc in cancer nest andperitumor interstitial tissue might be used as an immunological parameter for assessment of the prognosis.