2015年 · 第95卷第18期

polycythemia vera (PV) is a clonal and chronic myeloproliferative tumor mainly caused by abnormal proliferation of red blood cells leading to increased red blood cell volume. Determining that JAK2 and MPL are the initiating mutant genes that cause PV will be negative throughout the Philadelphia chromosome (Ph^–) The study of myeloproliferative tumors has brought into the era of molecular targeting.

As a new type of vascular diseases in the world, venous vascular malformations have become the most frequent and common lesions in congenital vascular malformations. In the past, it was often called cavernous hemangioma. Its pathological characteristics and clinical manifestations are essentially different from hemangioma. Its lifelong progressive development is the main source of health hazard of this disease. Severe venous vascular malformation syndrome is a large area severe venous vascular malformation combined with multiple organ involvement and accompanied by significant coagulation dysfunction syndrome, which is relatively rare in the world. At present, in view of the lack of understanding of severe venous vascular malformation syndrome in the world and the lack of uniform name, the author temporarily uses this name to explain and discuss the rare cases of systemic multiple severe venous malformation syndrome in clinic.

BCR/ABL fusion gene-negative myeloproliferative neoplasm (MPN) is a group of malignant myeloproliferative diseases originating from pluripotent hematopoietic stem cells, including polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). 90% of PV, half of ET and PMF patients have mutations in JAK2 V617F gene^[1]In 2008, the WHO regarded JAK2 V617F mutation as one of the main diagnostic criteria for PV, ET and PMF. 3% ~5% of patients had MPL gene mutations. Recently, it was found that 30% ~50% of ET and PMF without JAK2 and MPL gene mutations had CALR gene mutations^[2,3,4]The diagnostic criteria have not been included at present. The clinical manifestation of MPN is a chronic process, and the main risks are thrombosis and hemorrhage, and the potential for transformation into acute leukemia. Recent studies have shown that bone marrow angiogenesis plays an important role in MPN including ET, PV, and PMF^[5,6]Bone marrow microvessel density (MVD) is associated with vascular embolism, an important clinical manifestation of PV and ET^[7], associated with poor prognosis of MPN^[8]。

Sepsis refers to a systemic inflammatory response syndrome caused by infection, with clinically confirmed presence of bacteria or highly suspected foci of infection; Severe sepsis refers to sepsis accompanied by organ dysfunction and/or signs of hypoperfusion and hypotension; Septic shock refers to severe sepsis that is still accompanied by uncorrectable persistent hypotension after sufficient fluid resuscitation, and even may be accompanied by multiple organ (more than one) dysfunction. The incidence and mortality of severe sepsis and septic shock remain high. About 1.5 million patients in North America and Europe each year, with mortality rates ranging from 35% to 50%^[1]。 The pathophysiological changes of these patients are large, which causes the pharmacokinetics (PK) of antimicrobial drugs to change accordingly. In addition, the infected pathogenic bacteria are often multi-drug resistant bacteria, so conventional antimicrobial treatment is easy to lead to treatment failure.

Allergic asthma is an allergic disease with abnormal immune function, and the incidence rate is 5% ~16%^[1]。 The number of patients with allergic asthma in China is also increasing year by year, especially the incidence of childhood asthma is on the rise. From 1994 to 2009, the incidence of childhood asthma doubled. Allergy to house dust mite allergens is a major risk factor for rising asthma incidence^[2]Therefore, allergic asthma treatment for house dust mites is one of the main research directions.