MCL has a poor long term response to
MCL has a poor long-term response to current treatment strategies, for the most commonly used R-CHOP chemotherapy (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone), the remission rate is good (75–96%) but brief in duration. Despite the study of several other chemotherapy regimens, the median overall survival of MCL is only two to five years.
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Introduction Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome which is characterized by excessive macrophage function. The diagnosis of HLH in adults is challenging because initial signs and symptoms of HLH seem like common infections or fever of unknown origin. Infections (41.1%), malignancies (28.8%), autoimmune disorders (6.8%), primary immunodeficiency (1.4%), or post solid organ transplantation (2.7%) have been linked to this syndrome.
Discussion In HLH, macrophages become activated and secrete excessive amounts of cytokines, ultimately causing severe tissue damage that can lead to organ failure. Natural killer (NK) cells and/or cytotoxic T lymphocytes fail to eliminate activated macrophages. This lack of normal feedback regulation results in excessive macrophage activity and highly elevated levels of interferon gamma plus other cytokines. Immune activation is typically initiated by an infection. The most common infectious trigger for HLH is viral infection, particularly with Epstein-Barr virus (EBV). Patients with HLH should receive appropriate transfusions, and prevention and treatment of bleeding and opportunistic infections. Etoposide-containing immunochemotherapy, including HLH-94 protocol or HLH-2004 protocol, has become the recommended treatment for HLH. However, corticosteroids, IVIG, or a combination of these agents continues to be widely used. Hot et al. used only immunoglobulin, with a very successful response in cytomegalovirus associated HLH. Perhaps virus-associated HLH has a particularly favorable result with immunoglobulin as evidenced in Epstein–Barr associated HLH seen in children. Intravenous immune globulin (IVIG) contains the polyvalent IgG buy glibenclamide extracted from a pool of at least 1000 individual donors. It is believed to have multiple mechanisms of action, but the precise mechanism has not been definitively established. It is believed to involve suppression of inflammatory and immune-mediated processes. The donor antibody may interact with Fc receptors on phagocytic cells, reducing immune activity. Alternatively, the massive quantity of antibody may accelerate the fractional rate of catabolism of IgG. IVIG also promotes solubilization and clearance of immune complex deposits. IVIG can also alter the number of T cells and T cell subsets. In 2014, Ezhilarasi Subbiah from India reported that IVIG and HLH-2004 protocol may be equally effective in the management of HLH in children. IVIG may be a preferable initial regimen, to avoid the adverse events of etoposide such as myelosuppression, mucositis, secondary leukemia, and myelodysplasic syndromes. Despite the fact that data regarding IVIG in herpes simplex virus-associated hemophagocytic syndrome was limited, few cases of virus-associated hemophagocytic syndrome have been described in the literature. In previous reports, some HLH patients were diagnosed with ARDS during their clinical course, as in our case. Nahum et al. reported that respiratory failure including ARDS was noted in 7 of 11 HLH children, with multiple organ failure after a diagnosis of HLH having been made in the pediatric population. The incidence of hemophagocytic syndrome coexisting with ARDS in adult population was unknown. The treatment of underlying causes of ARDS might be of import because no drug has proven beneficial in the prevention or management of ARDS.
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Introduction Pleuropulmonary Blastoma (PPB) is an exceedingly rare intrathoracic malignancy that typically arises in childhood. Specifically, PPB accounts for less than 1% of all primary lung tumors of children less than 6 years of age. The PPB tumor is a dysembryonic neoplasm of thoraco-pulmonary mesenchyme, which stems from the lung, the pleural surface or both locations. PPB may also arise from pre-existing cysticpulmonary lesions. Typically, PPB lesions are classified based on Dehner\'s histological classification. Specifically, type I PPB comprises absolute cystic lesions (i.e., no solid component), type II PPB lesions contain mixed cystic and solid components, while type III PPB consists of solid tumors without epithelial-lined cysts. The details of diagnostic features of PPB, such as pneumothorax symptoms, bilateral or multi-segment involvement, the presence of a complex cyst, particularly for type I PPB, germline and mutation in the DICER1 gene, have been recently reported.