Departamento de Hematologia, Laboratório de Citogenética, Hospital das Clinicas HCFMUSP, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, SP, BR Av. Dr. Eneas de Carvalho 255, Cerqueira César 01246-000, São Paulo, SP, Brazil; Hospital Israelita Albert Einstein. Av. Albert Einstein, 627/701 Morumbi 05652- 900 - São Paulo, SP, Brazil. Electronic address:
Published: April 2018
AI Article Synopsis
Article Abstract
Familial platelet disorder with propensity to acute myeloid leukemia (FPD/AML) associated with RUNX1 mutations is an autosomal dominant disorder included in the group of the myeloid neoplasms with germ line predisposition. We describe two brothers who were diagnosed with hematological malignancies (one with AML and the other with T-cell lymphoblastic lymphoma). There was a history of leukemia in the paternal family and two of their siblings presented with low platelet counts and no history of significant bleeding. A microdeletion encompassing exons 1-2 of RUNX1 (outside the cluster region of the Runt Homology domain and the transactivation domain) was detected in six family members using array-CGH and MLPA validation. A low platelet count was not present in all deletion carriers and, therefore, it should not be used as an indication for screening in suspected families and family members.
De Quervain's disease (DQVD) is the stenosing tenosynovitis of tendons in the first extensor compartment of the forearm. It is a common inflammatory condition that is often treated conservatively. While conservative therapy is an effective modality of treatment for a large number of patients suffering from DQVD, some patients do not improve with conservative measures.
Intraocular pressure (IOP) is regulated through the balance of production and drainage of aqueous humor. The main route of aqueous-humor outflow comprises the trabecular meshwork (TM) and Schlemm's canal (SC). We reported that IL-6 trans-signaling can inhibit TGF-β signaling in TM cells and may affect regulation of IOP.
Background: To explore the correlation between the levels of D-dimer (D-D), fibrinogen (FIB), fibrinogen degradation products (FDP) and platelets (PLT) in peripheral blood of patients with lower limb fractures and the formation of deep vein thrombosis in lower limbs, and to establish a new thrombosis prediction model for patients with lower limb fractures.
Methods: The patients were divided into DVT group and non DVT group according to whether there was deep vein thrombosis of the lower extremity. The differences in the levels of D-D, FIB, FDP and platelets between the two groups were analyzed and compared.
B-cell lymphoma extra large (BCL-X) is an important anti-apoptotic protein of BCL-2 family. It is frequently overexpressed in various hematologic and solid tumors, often positively correlated with chemotherapy resistance in tumors. However, the clinical development of the small molecule BCL-X inhibitor ABT-263 has been challenged on account of its on-target and dose-limiting toxicity.
The threats to chemical warfare-associated agents (CWA), including nitrogen mustard, are increasing, and no direct antidote is currently available to mitigate the deleterious cutaneous and systemic responses to prevent mortality. Though most of these agents act as alkylating agents, a significant knowledge gap exists in the molecular mechanisms of how these vesicants cause toxic effects. Studies, including ours, have shown that exposure to reactive oxygen species (ROS)-generating stimuli, including alkylating chemotherapeutic agents, and thermal burn injuries with ethanol produce the potent family of lipid mediators, Platelet-activating factor (PAF) agonists that induce local inflammation, and multi-system organ dysfunction (MOD).
