A rare case of POEMS(polyneuropathy,organomegaly,endocrinopathy,M-band,skin changes) syndrome associated with multicentre Castleman's disease and thrombocytosis is reported in a 48-year-old man.Three years prior to the presentation,he developed a series of symptoms without obvious triggers,including persistent myalgia and myasthenia of extremities,abnormal skin sensation (burning),gradually increased roughness and pigmentation of skin,thickening and mild induration of lower-extremity skin giving an ichthyosiform appearance.Two years prior to the presentation,electromyography indicated peripheral neuropathy,and computed tomography(CT) revealed hepatomegaly,splenomegaly,lymphadenectasis,ascites,hydrotborax and hydropericardium.Multiple biopsies of lymph nodes indicated an obvious vascular proliferation between lymphoid follicles with the involvement of lymphoid follicles,which,together with the histochemical findings and special staining results,was suggestive of hyaline-vascular type Castleman's disease.Three months prior to the presentation,painful erythema emerged and gradually spread in both thighs,with a progressive deepening in the colour of central lesions,and flushing of periphery lesions.Two months prior to the presentation,a soybean-sized nodule arised in the erythema on the right inner thigh,which was then ulcerated with a little pus left and a shallow ulcer formed.Physical examination revealed a mild atrophy of lower-extremity muscles,with muscle strength graded as 5 for bilateral upper extremities and as 4 for bilateral lower extremities.The skin was rough,diffusely pigmented and thickened,giving an ichthyosiform appearance,which was more apparent in the extremities than in the trunk.An atropurpureus patch sized 5 cm × 2 cm was seen respectively in bilateral inner thighs; the surface of the central lesions was coarse and covered with dry crusts and scales,giving a rough paperlike appearance,and the peripheral lesions were surrounded by a faint prunosus halo,which faded on pressure.Laboratory examination found hypothyroidation,reduced insulin secretion,abnormal calcium and phosphorus metabolism,and progressive thrombocytosis.Immunofixation electrophoresis showed that the serum was weakly positive for IgG type M protein.This patient was diagnosed with POEMS syndrome associated with multicentre Castleman's disease and thrombocytosis.After treatment with methylprednisolone and thalidomide,the main symptoms of POEMS syndrome were improved,whereas thrombocytosis progressively aggravated.

OBJECTIVETo explore the epidemiological characters of severe acute respiratory syndrome (SARS) in Sichuan province in order to provide evidence for prevention and control.

METHODSTo generate data on SARS in Sichuan province through descriptive and analytical studies on time, geographic distribution, population, source of infection, the way of case finding, symptom, diagnosis and treatment of the cases.

RESULTSThe peak of the epidemic last from April 16 to May 7. The number of cases in Luzhou and Guangyuan cities took up 60% of the total. Mobile population occupied 68% of the cases. Most of the patients were above the age of 20 with a sex ratio of 1.5:1 (m/f). 80% of the cases had a history of working in Guangdong province and recently returned to their hometowns. The main symptoms and signs of the SARS patients would include fever, cough and chest X-ray changes.

CONCLUSIONAll cases were imported. Fluctuation of the epidemics was mainly affected by the mobility of working population who recently returned to their hometowns. Measures concerning the prevention and control of the epidemics would mainly target on the isolation of confirmed and suspected patients who might serve as the sources of infection through setting up quarantine station, assigned hospitals and special 'fever-clinics'.

Adult ; Aged ; China ; epidemiology ; Female ; Humans ; Male ; Middle Aged ; Severe Acute Respiratory Syndrome ; diagnosis ; epidemiology ; prevention & control

Abstract　　Chimetic antigen receptor T cells (CAR-T) are a kind of novel killer cells derived from autogenous or allogeneic T cells targeting the tumor specific antigens by gene engineering transpormation. The CAR-T cells possess the advantages of high specificity, high efficiency and non-MHC restriction, thus achieving good therapeutic effects in relapsed/refractory hematological tumors and some solid tumors. The process of CAR-T cell preparation includes cell isolation and purification, activation and differentiation, gene modification, in vitro amplification, phenotypic quality control, preservation and reinfusion. In recent years, with the rapid development of flow cytometry, this technology has been widely used in all aspects of CAR-T cell therapy, including pre-treatment screening, in vitro preparation and post-transfusion monitoring, which plays an important role in its innovative optimization.

Craniopharyngioma ; Humans ; Parapharyngeal Space ; Pharyngeal Neoplasms ; Pituitary Neoplasms

OBJECTIVETo summarize the clinical characteristics of peripheral blood, immune phenotypes, fusion genes and cytogenetics of patients with t(8;21) acute myeloid leukemia(AML) through the retrospective analysis of 586 patients with t(8;21) AML from 15 blood disease research centers in Northern area of China.

METHODSThe factors affecting prognosis of patients with t(8;21) AML were investigated by using univariate and multivariate COX regression.

RESULTSThe immune type of t(8;21) AML patients was mainly with HLA-DR, CD117, CD34, MPO, CD38, CD13and CD33(>95%), part of them with CD19and CD56; the most common accompanied mutation of t(8;21) AML patients was C-KIT mutation (37.8%); in addition to t(8;21) ectopic, the most common chromosomal abnormality was sex chromosome deletions (38.9%). The univariate analysis revealed a significant survival superiority of OS and PFS in t(8;21) AML patients of WBC≤3.5×10/L without C-KIT mutation, the newly diagnosed ones achieved HSCT(P<0.05), only survival superiority on OS in t(8;21) AML patients with extramedullary infiltration and CD19 positive; the results of multivariate analysis showed a significant survival superiority on OS and PFS in t(8;21) AML patients with WBC≤3.5×10/L(P<0.05).

CONCLUSIONThe clinical features of t(8;21) AML patients in China are similar to those in other countries, WBC≤3.5×10/L is a good prognostic factor while the C-KIT mutation is a poor one in t(8;21) AML patients.

We wished to establish an expert consensus on late stage of critical care (CC) management. The panel comprised 13 experts in CC medicine. Each statement was assessed based on the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) principle. Then, the Delphi method was adopted by 17 experts to reassess the following 28 statements. (1) ESCAPE has evolved from a strategy of delirium management to a strategy of late stage of CC management. (2) The new version of ESCAPE is a strategy for optimizing treatment and comprehensive care of critically ill patients (CIPs) after the rescue period, including early mobilization, early rehabilitation, nutritional support, sleep management, mental assessment, cognitive-function training, emotional support, and optimizing sedation and analgesia. (3) Disease assessment to determine the starting point of early mobilization, early rehabilitation, and early enteral nutrition. (4) Early mobilization has synergistic effects upon the recovery of organ function. (5) Early functional exercise and rehabilitation are important means to promote CIP recovery, and gives them a sense of future prospects. (6) Timely start of enteral nutrition is conducive to early mobilization and early rehabilitation. (7) The spontaneous breathing test should be started as soon as possible, and a weaning plan should be selected step-by-step. (8) The waking process of CIPs should be realized in a planned and purposeful way. (9) Establishment of a sleep-wake rhythm is the key to sleep management in post-CC management. (10) The spontaneous awakening trial, spontaneous breathing trial, and sleep management should be carried out together. (11) The depth of sedation should be adjusted dynamically in the late stage of CC period. (12) Standardized sedation assessment is the premise of rational sedation. (13) Appropriate sedative drugs should be selected according to the objectives of sedation and drug characteristics. (14) A goal-directed minimization strategy for sedation should be implemented. (15) The principle of analgesia must be mastered first. (16) Subjective assessment is preferred for analgesia assessment. (17) Opioid-based analgesic strategies should be selected step-by-step according to the characteristics of different drugs. (18) There must be rational use of non-opioid analgesics and non-drug-based analgesic measures. (19) Pay attention to evaluation of the psychological status of CIPs. (20) Cognitive function in CIPs cannot be ignored. (21) Delirium management should be based on non-drug-based measures and rational use of drugs. (22) Reset treatment can be considered for severe delirium. (23) Psychological assessment should be conducted as early as possible to screen-out high-risk groups with post-traumatic stress disorder. (24) Emotional support, flexible visiting, and environment management are important components of humanistic management in the intensive care unit (ICU). (25) Emotional support from medical teams and families should be promoted through"ICU diaries"and other forms. (26) Environmental management should be carried out by enriching environmental content, limiting environmental interference, and optimizing the environmental atmosphere. (27) Reasonable promotion of flexible visitation should be done on the basis of prevention of nosocomial infection. (28) ESCAPE is an excellent project for late stage of CC management.
Humans ; Consensus ; Critical Care/methods* ; Intensive Care Units ; Pain/drug therapy* ; Analgesics/therapeutic use* ; Delirium/therapy* ; Critical Illness