2015年 · 第54卷第02期

At 3: 52 a.m. on December 11th, 2014, Professor Wang Haiyan, a famous medical scientist, medical educator and academic figure of nephrology in China, and the First Hospital of Peking University, died suddenly. As her student and colleague for many years, I feel saddened and hard to believe the cruel fact that this must be faced.

The bad news raided, and the medical community was shocked. Professor Wang Haiyan, a member of the Communist Party of China, an internationally renowned nephrologist, former vice president of Chinese Medical Association, former chairman of Internal Medicine Branch and Nephrology Branch of Chinese Medical Association, the eighth and ninth editors in chief and the tenth honorary editor in chief of Chinese Journal of Internal Medicine, former vice president of Peking University First Hospital, director of Peking University Institute of Nephrology, my respected teacher and friend of mine, died in Beijing at 3: 52 a.m. on December 11th, 2014 at the age of 77 due to ineffective treatment of sudden illness. We were surprised to learn that Professor Wang drove to the west in the year of happy birthday, and we have been immersed in grief. When the remains bid farewell to witness the familiar face again, the past lingers. In the 30 years of working in the society, the author has spent more than 20 years with Professor Wang, especially in the 12 years since she became the editor-in-chief of Chinese Journal of Internal Medicine. Through frequent daily contact, she not only was influenced by her rigorous study style, but also admired her upright and maverick character. Looking back at my own growth experience, I am full of the reward and support of this strict teacher and helpful friend. In order to remember Professor Wang's great contribution to the society and the magazine, I will now record the snow and mud claws related to her in my memory here to express my endless sorrow.

Hematological malignant tumors such as leukemia and lymphoma are major diseases that seriously endanger people's life and health. In recent years, hematological diseases have made great progress and even major breakthroughs in pathogenesis, molecular markers, targeted drugs, etc. The 5-year survival time of patients with hematological malignant tumors has increased to 60% ~90%, gradually changing from "incurable" to "curable" diseases. On the one hand, the development of hematological malignancies in China has benefited from the progress of global medicine; on the other hand, the original achievements of Chinese scholars in the fields of acute promyelocytic leukemia (APL), t (8; 21) acute myeloid leukemia (AML), chronic myeloid leukemia (CML), and hematopoietic stem cell transplantation (HSCT) have become the "motive force" to promote the global hematological malignancies. Actively drawing on the world's academic frontier and learning its research methods, forming an original and standardized system with Chinese characteristics, and expanding international influence through cooperation are the core of development in the field of hematological malignancies in China at present.

The Philadelphia chromosome (Ph) is a common genetic abnormality in acute lymphoblastic leukemia (ALL) called Ph^＋ALL, whose incidence was significantly associated with age, chemotherapy-alone Ph^＋The 3-year overall survival (OS) rate of ALL patients is less than 20%. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) can increase the 3-year OS rate to 36% ~44%^[1,2,3]。 Tyrosine kinase inhibitors (TKIs) are targeted therapeutic agents for patients with positive BCR-ABL fusion genes, and the first-generation TKIs imatinib combined with chemotherapy induce the treatment of Ph^＋The complete response (CR) rate of ALL is as high as more than 90%, and some even reach 100%. The second-generation TKIs dasatinib is effective against multiple mutations that are resistant to imatinib, and can inhibit SRC family kinases, platelet-derived growth factor receptor (PDGFR), c-Kit and EPH receptor A2. In this study, the Ph patients treated with TKIs in our hospital from January 2008 to September 2013^＋ALL patients were retrospectively studied to observe their clinical characteristics, induced remission and long-term survival, and the factors affecting OS and disease-free survival (DFS) were analyzed, so as to provide evidence for clinical treatment.

hemophagocytic lymphohistocytosis (HLH), also known as hemophagocytic syndromes (HPS), is a syndrome of excessive inflammatory response caused by hereditary or acquired immune abnormalities. The clinical symptoms and signs were mainly fever, hepatosplenomegaly and pancytopenia. The disease has the characteristics of rapid progression and high mortality, so more and more people have paid attention to it. Its etiology can be divided into primary and secondary (acquired), the former is mainly related to gene defects; Secondary HLH is related to various potential diseases. Although its mechanism is still unclear at present, its trigger spectrum has been continuously updated in recent years. It is a reactive disease caused by infection, tumor, autoimmune diseases, metabolic diseases and other causes to initiate the activation mechanism of the immune system. It can be seen in all ages. Infection and rheumatic diseases are more common in children. Malignant tumors, especially lymphoma, are the main triggers of HLH in adults, and most of lymphoma-related hemophagocytic syndrome (LAHS) are T-cell lymphomas. In this study, the clinical data of 71 patients with HLH who were pathologically diagnosed with T-cell lymphoma were retrospectively analyzed.

Eosinophilia syndrome (HES) is a group of diseases in which eosinophils persistently proliferate to a high degree and infiltrate various tissues and organs throughout the body, showing complicated clinical features, which is easy to cause misdiagnosis and mistreatment. If the exact cause cannot be found in clinic, it is called idiopathic eosinophilia syndrome (IHES). This disease is rare in clinic. Most of the reported cases in the past are exclusion diagnoses. With the development of genetic testing technology, this disease has a new understanding. Two cases of clinical misdiagnosis of IHES were reported, and the final bone marrow test of FIP1L1-PDGFR α (F/P) fusion gene was positive and excluded. Combined with the literature, the diagnostic process and treatment of HES were summarized, and the understanding of IHES was improved.

The patient was a 76-year-old male. He was admitted to hospital because of "repeated chest tightness and chest pain for more than 10 years, and recurrent aggravation for 4 days". The patient began to have episodic chest tightness and chest pain 10 years ago. The pain was located in the precordial area, showing a swelling-like discomfort, without acid reflux, belching, nausea, vomiting, and without radiating pain in the shoulder and back. Each attack lasted about 10 min. Chest tightness and chest pain have no obvious correlation with eating, posture and activity. The symptoms can be relieved after taking "Suxiao Jiuxin Pill". They have been treated in local hospitals many times and are considered as "coronary heart disease and angina pectoris", but they have not been treated regularly, and the above symptoms have recurred. Four days ago, the above symptoms recurred, and the pain was worse than before. He was hospitalized in a local hospital. CT examination of coronary artery (coronary artery) showed coronary heart disease and three-vessel disease, so he was transferred to the Department of Cardiology of our hospital for hospitalization. Coronary angiography after admission to the hospital showed diffuse plaque formation in the right coronary artery (RCA), with stenosis of about 50% at the most stenotic point; Left main trunk (LM) was substantially normal; Subtotal occlusion of the anterior descending artery (LAD) in the proximal and middle segment; Circumflex artery (LCX): 95% mesiomedial stenosis. One 2.5 mm ×25 mm and one 3.0 mm ×18 mm stent were implanted in LAD and LCX respectively (the release pressure was 16 standard atmospheres, 1 standard atmosphere =101.325 kPa), seeFigure 1。 The patient had three times of in-stent thrombosis within the 9th, 10th and 19th days after operation, respectively. For details, seeTable 1，Figure 2，Figure 3，Figure 4。 Despite active management, the patient still repeatedly experienced in-stent thrombosis. Considering that the patient may have "clopidogrel resistance", the polymorphism of CYP2C19 gene related to clopidogrel metabolism was tested, which indicated that its genotype was CYP2C19*2/*2. According to the patient's genotype, the new antiplatelet drug ticagrelor 90 mg was immediately switched to, and the antiplatelet treatment was performed twice/d. The patient recovered well, and no in-stent thrombosis occurred again. There was no chest tightness or chest pain after 1 year follow-up.

In recent years, with the rapid development of China's economy and industrialization, the change of lifestyle and the acceleration of aging process, the prevalence of chronic diseases such as diabetes, cardiovascular diseases and tumors in China's adult population is rising year by year. The demand of Chinese residents for medical and health services has increased significantly, which has become an important public health problem. Community health service is an important content of urban medical and health system reform in China, and strengthening the level of community medical and health service is an effective means to solve this public health problem. However, in the current development of health undertakings, there are still problems such as high concentration of high-quality resources in large hospitals, shortage of community health service resources and weak service capacity, which bring great challenges to community chronic disease management.

Pulmonary cryptococcosis is a subacute or chronic pulmonary mycosis caused by inhalation of cryptococcal spores, with Cryptococcus neoformans and Cryptococcus gettus being the main pathogenic species. In recent years, the incidence of cryptococcosis has been increasing year by year, and it has become the third major class of pulmonary mycosis after Candida albicans and Aspergillus, so it has attracted increasing clinical attention^[1]。 Especially in recent years, the reports of pulmonary cryptococcosis in patients with normal immune function are increasing, and its clinical manifestations have certain characteristics, which deserve the attention of clinicians.

Minimal residual disease (MRD) refers to the state in which a small number of leukemic cells remain in the body after leukemia is completely remitted by induction chemotherapy or after bone marrow transplantation. At present, MRD monitoring methods mainly include morphology, cytogenetics, molecular biology and other methods. Among them, fluorescence quantitative PCR (RQ-PCR) and flow cytometry have been developed in recent years with good sensitivity and specificity. The panleukemia gene, WT1 gene (Wilms' tumor gene 1), plays an important role in the monitoring of MRD in acute leukemia, especially acute myeloid leukemia (AML). This review aims to share the research results that have made great contributions to the above aspects in recent years.

Inflammatory myopathies are a group of autoimmune diseases with muscle involvement as the main manifestation. Clinically, polymyositis (PM) and dermatomyositis (DM) are the most common. Various serum muscle enzymes, such as phosphocreatine kinase (CK), aldolase (AST), ALT and lactate dehydrogenase (LDH), are often elevated during the active phase of the disease. Among them, CK is the most commonly used in clinic. It is a sensitive and specific index to reflect muscle injury, and it is correlated with the severity of PM/DM and treatment response. However, the study found that some patients with PM/DM had normal serum CK during the active phase of disease^[1]The normal cause and its clinical significance are still inconclusive. Whether the detection of other muscle enzymes with slightly less specificity, such as aldolase, in this subclass of patients has clinical value is worth exploring.

With the transformation of biomedical model to bio-psychological-social medical model^[1]The role of social psychological factors in the occurrence and development of diseases has gradually been paid attention to. The concept of "stress" was first proposed by Hans Selye. It is defined as the sum of the non-specific responses of the body to various external or internal stimuli^[2]Then psychologists extended the definition of psychological stress, that is, the psychological response produced by various stimuli, and its processes include stressors, stress processes and psychological outcomes triggered by stress^[3]。 It was found that psychological stress is closely related to disease outcome, and psychological stress is not only related to the increased risk of cardiovascular disease^[4], also associated with the onset of type 2 diabetes^[5]。 In the digestive system, the association of psychological stress with upper gastrointestinal ulcer has been confirmed^[6]It is also closely related to functional bowel disease^[7]And its correlation with inflammatory bowel disease (IBD), an organic bowel disease, has also attracted much attention recently.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that causes multiple organ and system damage^[1]The main characteristics are polyclonal activation and proliferation of B lymphocytes, production of a large number of autoantibodies, increase of circulating immune complexes, decrease of complement and pathological inflammation of multiple organs. However, the pathogenesis of SLE has not been fully elucidated at present. In recent years, more and more studies have shown that microRNAs play an important role in the occurrence and development of SLE. The role of microRNAs in the development of SLE is summarized as follows.

The patient was a 45-year-old male. He came to the emergency department of our hospital because of "repeated chest tightness, chest pain combined with systemic rash for 6 days". The patient had fever once without obvious trigger 2 weeks ago, and went to the local clinic to measure his body temperature to "41℃". After being treated with bacteria, his body temperature dropped to normal. However, the next day, he developed a rash, mainly located on the trunk and limbs, without blisters, pain and itching. At that time, he went home after being treated with drug allergy in the local hospital (the specific medication is unknown). After returning home, he felt his body temperature was high, but he did not go to the hospital to measure it, and he was not given further diagnosis and treatment. On the 3rd day, the patient repeatedly experienced chest tightness after activity, accompanied by chest pain in severe cases, which can be relieved after rest, but the chest tightness persisted, and the symptoms were mild at first, without drug treatment. On the 10th day, the chest tightness worsened and the area of trunk rash increased, so he came to the emergency department of our hospital. Emergency physical examination: temperature 38℃, pulse 105 beats/min, breathing 18 beats/min, blood pressure 85/65 mmHg (1 mmHg =0.133 kPa). Conscious, lethargic, multiple rashes at the proximal end of the limbs, the rashes are isolated, slightly higher than the skin surface, and normal skin can be seen between the rashes (Figure 1)。 The heart rate was 105 beats/min, the rhythm was uniform, the heart sounds were normal, and no murmur was heard; The breathing sounds of both lungs are clear, and a small amount of wet rales can be smelled in both lower lungs; Abdominal examination showed no abnormalities; No edema was observed in both lower limbs. Electrocardiogram showed sinus tachycardia, right bundle branch block with T wave changes (Figure 2)。 Blood routine: white blood cells 10.49×10⁹/L, neutral 0.702, mononuclear 0.043, lymphatic 0.246, hemoglobin 155 g/L, creatine kinase (CK): 1 297 U/L, creatine kinase isoenzyme (CK-MB): 45 U/L, myoglobin 350.5 μ g/L, B-type natriuretic peptide (BNP) 9 033 ng/L, troponin T (TnT): 1.8 μ g/L. Chest CT revealed pneumonia of both lower lungs. The patient was in good health in the past, denied a history of hypertension and diabetes, did not smoke, denied a history of similar diseases in the family, denied a history of infectious diseases; No history of travel and contact with suspected epidemic areas. The emergency department was admitted to the hospital with "chest tightness to be examined, acute coronary syndrome (ACS)? Viral myocarditis?".

September 2014BloodA clinical trial led by Professor Huang He of the Bone Marrow Transplantation Center of the First Affiliated Hospital of Zhejiang University School of Medicine was published-"Comparison of the efficacy of low-dose anti-T lymphocyte immunoglobulin-based and non-in vitro T-cell-depleted HLA haploconsistent hematopoietic stem cell transplantation with all-consistent sibling donor transplantation and non-congenital donor transplantation" [Luo Y, Xiao H, Lai X, et al. T-cell-replete haploidentical HSCT with low-dose anti-T-lymphocyte globulin compared with matched sibling HSCT and unrelated HSCT. Blood, 2014, 124 (17): 2735-2743.]. The center created an optimized new protocol for the treatment of HLA haploconsistent hematopoietic stem cell transplantation (HSCT) based on low-dose anti-T lymphocyte immunoglobulin (ATG-F), simple peripheral blood hematopoietic stem cell transfusion, and non-in vitro T cell removal, and designed a prospective controlled study for the first time to compare the efficacy with HLA totally consistent sibling donor transplantation and non-blood donor transplantation at the same time.

Top international academic journals in the field of hematology, August 2014Blood(Impact factor 9.775) published a study "Optimal selection of donors for haploid hematopoietic stem cell transplantation" completed by Professor Huang Xiaojun's team at the Institute of Hematology of Peking University over more than ten years [Wang Y, Chang YJ, Xu LP, et al. Who is the best donor for a related HLA haplotype-mismatched transplant?. Blood, 2014, 124 (6): 843-850.]. The paper serves asBloodFocus article on the cover, specially recommended by Editor-in-Chief Professor Bob Löwenberg at the front of the volume.

Analysts point out that the UK could drive patient-centred healthcare by assessing "how well people can manage their health".

Studies have shown that a lack of salt in the diet increases the risk of major cardiovascular events and death in patients as much as consuming too much salt.

Progressive weight loss is widely believed to be better for long-term weight control than rapid weight loss in the short term, but a new study challenges this idea. The study showed that weight gain was not related to the rate of weight loss.

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A follow-up study of patients with chronic lymphocytic leukemia (CLL) who were refractory or relapsed after receiving first-line FCR (fludarabine + cyclophosphamide + rituximab) examined the long-term efficacy of FCR. The data show that disease deterioration and the first remission period after the first rescue (REM1) are a key determinant of survival, and patients with longer REM1 are suitable for FCR retreatment (Blood, 2014, 124:3059-3064).