急性髓系白血病(acute myeloid leukemia,AML)是一类起源于造血干细胞的恶性克隆性疾病;单克隆免疫球蛋白血症(monoclonal immunoglobulinemia,M蛋白血症)是由B淋巴细胞或浆细胞克隆性增生并分泌异常免疫球蛋白所引起的一组疾病.AML合并M蛋白血症鲜有报道,我们通过收集1例AML-M2b合并M蛋白血症患者的临床资料和文献复习,探讨该病的病因和治疗,进一步的提高认识.

Objective:To investigate the clinical characteristics of patients with myelodysplastic syndrome (MDS)-refractory neutropenia (RN),and summarize the experience of diagnosis and treatment of one case of MDS-RN.Methods:A retrospective analysis was performed on the clinical characteristics,treatment,morphologic and cytogenetic characteristics of the bone marrow cells of the patient diagnosed with MDS-RN.Results:The chief complaint of the 58-year-old male patient was fatigue.The counts of white blood cells were 2.9 x 109/L (neutrophils 35％,lymphocytes 56％ and monocytes 9％),the hemoglobin level was 136 g/L and the counts of platelets were 182 × 109/L.The bone marrow cytology revealed hyperplasia and myelodysplasia of both granulocytes and megakaryocytes,and the granulocyte dysplasia including pseudo-Pelger,hypo-/degranulation and hyposegmentation of neutrophils,which accounted for 24％ of the total granulocytes (57.5％).Micromegakaryocytes with mono-round-nuclear could also be observed,but without erythroid dysplasia.Chromosome analysis revealed the aberration of 46,xy,add (16) q24 [20].Conclusion:For patients with MDS-refractory cytopenia with unilineage dysplasia,it is suggested to undertake cytogenetic examination and next-generation sequencing technology to detect gene mutation,besides the traditional bone marrow cytology and biopsy,to ensure an accurate diagnosis and treatment.

Acute myeloid leukemia (AML) is a heterogeneous disease and shows different clinical features among different races, and great diversity in prognosis. In order to under the relationship between clinical characteristics and prognosis, 92 patients with t(8;21) AML in a single centre of China were retrospectively analyzed, and clinical characteristics such as peripheral blood, morphological type of blast cells, molecular detection and cytogenetics, optimal treatments, and outcomes are summarized. Among all 92 t(8;21) AML patients, 80 (87.0%) presented with AML-M2. Moreover, 80 (52.29%) cases of AML1/ETO-positive were identified among 153 patients diagnosed with AML-M2. AML1/ETO-positive patient groups are significantly younger than negative ones (34 vs 49 years old; P<0.001). The most common accompanying mutation of t(8;21) AML patients was stem cell factor receptor (c-KIT) mutation (47.22%). In addition to ectopic t(8;21), the most common chromosomal abnormality was sex chromosome deletions (38.18%). Patients with c-KIT mutation had lower haemoglobin (P=0.044) and complete remission (CR) rates (P=0.026) and shorter overall survival (OS) (P=0.004) than c-KIT-negative groups. Unexpectedly, the 3-year OS rate was not higher for patients treated with hematopoietic stem-cell transplantation (HSCT) than with only chemotherapy (53.83% vs 64.44%; P=0.740). AML1/ETO-positive patients showed a higher CR rate and longer OS than AML1/ETO-negative patients. However, neither difference was statistically significant (P=0.250 and P=0.675 respectively). These findings indicate that retrospective analysis of Chinese patients can provide critical information in the optimal treatment of t(8;21) AML patients with AML1/ETO. C-kit is a poor prognostic factor, and HSCT is not the best choice for t(8;21) AML patients.

Epstein-Barr virus (EBV) is a ubiquitous herpes virus whose infection is usually asymptomatic and persists for a lifetime. It often causes symptomatic diseases, such as infectious mononucleosis (IM), but rarely leads to EBV associated-NK-cell lymphoproliferative disorder (EBV-NK-LPD) in non-immunocompromised individuals. No studies have described EBV-NK-LPD treated with haploidentical hematopoietic stem cell transplantation (haplo-HSCT). The present study reports a case of a 29-year-old man with recurrent fever and cervical lymph node enlargement. The result of sorting lymphocytes with immunomagnetic beads was diagnosis of EBV-NK-LPD. To stop progression of this disease, haplo-HSCT was employed, with a good prognosis. The patient has survived for one year with no signs of recurrence.

Background: The increased survival of patients after a primary cancer diagnosis has led to an increasing number of patients with second primary malignancies (SPMs). Recent studies implied the risk of SPMs among specific survivor groups. However, little is known regarding the risks of developing SPMs across the spectrum of cancer survivors. The present study was undertaken to describe the incidence, common sites, and outcomes associated with SPMs among Chinese cancer survivors at a single center. Methods: This retrospective study evaluated patients who were >= 18 years old and diagnosed as having a malignancy during 2002-2016 at the Department of Hematology, Tongji Hospital, Tongji Medical College. Factors associated with subsequent SPMs were explored using bivariable and multivariable models. Results: The study identified 18,257 eligible patients diagnosed with hematological malignancies, including 67 patients (0.37%) who developed SPMs, which mainly consisted of leukemia. Survivors of lymphoma had the highest risk of developing SPMs. Hematological SPMs were more common among patients who received alkylating agents, topoisomerase 2 inhibitors, or allogeneic hematopoietic stem cell transplantation (HSCT) for the first primary malignancy. Non-hematological SPMs were more common among patients who received anti-metabolites, anti-tubulin agents, or radiotherapy for the first primary malignancy. The risks of developing SPMs did not exhibit any significant differences according to age, sex, and latency interval. Hematological malignancy was a non-significant risk factor for SPM development (P>0.05). Conclusions: Approximately 0.37% of our patients diagnosed with hematological malignancies had SPMs, mainly leukemia. Patients with lymphoma were most likely to develop SPMs. These findings may help identify patients who are at-risk of developing SPMs (e.g., based on the previous treatment and the first primary malignancy), which may help guide the treatment of cancer survivors.

Objective To investigate the distribution and antibiotic resistance of infection pathogens isolated from hematology ward.Methods Bacterial susceptibility test was carried out by K-B method.Data were analyzed by WHONET 5.4 software.Results A total of 667 pathogens were isolated and collected,including gram-negative bacilli (55.7％),gram-positive cocci (33.3％) and fungi (11.0％).The dominant source of pathogens was blood (40.9％),sputum (26.0％) and urine (14.0％).The top 3 pathogens were Escherichia coli (15.1％),Coagulase negative Staphylococcus (15.1％) and Klebsiella pneumoniae (10.1％).About 46.8 ％ of Klebsiella pneumoniae and 62.0％ of Escherichia coli produced extended spectrum [β-lactamases.The sensitive rates of Klebsiella pneumoniae and Escherichia coli to imipenem and meropenem were both more than 95％.The resistance rates of Pseudomonas aeruginosa to levofloxacin,imipenem,meropenem,Cefoperazone/sulbactam,piperacillin/tazobactam were lower than 20％.More than 85％ strains of Stenotrophymonas maltophilia were susceptible to levofloxacin,cotrimoxazole and minocycline.The prevalence of methicillinresis tant coagulase-inegative staphylococci and methicillin resistant staphylococcus aurreus were 34.6％ and 76.6％ in Coagulase negative staphylococcus and Staphylococcus aureus,and strain resistant to teicoplanin was isolated.The susceptibility of Enterococcus spp.to teicoplanin and vancomycin were 84.1％ and 70.3％,respectively.Gram-positive cocci resistant strains to teicoplanin and vancomycin were isolated.Conclusions Gram-negative bacilli keep the most prevalence pathogens in the hematology ward and high susceptibility to empirical antimicrobial agents.But the antibiotic resistance to glycopeptide antibiotics was getting worsen for the major gram-positive cocci.

Objective To investigate the distribution and antibiotic resistance of the pathogens isolated from blood of the inpatients in hematology ward.Methods Antimicrobial susceptibility test was carried out using Kirby-Bauer method.The data were analyzed by WHONET 5.6 software.Results Of the 521 microbial isolates collected,gram-negative bacilli accounted for 47.2％,grampositive cocci 45.7％ and fungi (7.1％).The most frequently isolated microorganisms were coagulase negative Staphylococcus (154),E.coli (88),K.pneumoniae (51),P.aeruginosa (39) and Enterococcus spp (34).ESBLs were produced in about 40.4％ of the K.pneumoniae isolates and 63.4％ of the E.coli isolates.At least 90％ of the E.coli isolates were susceptible to imipenem and meropenem,and at least 70％ susceptible to piperacillin-tazobactam.At least 85％ of the K.pneumoniae strains were susceptible to imipenem and meropenem,and at least 70％ susceptible to levofloxacin,piperacillin-tazobactam and cefoperazone-sulbactam.The percentage of the P.aeruginosa susceptible to ciprofloxacin and tobramycin was at least 90％,and higher than 70％ to levofloxacin,meropenem,imipenem,piperacillin-tazobactam,cefepime,and cefoperazone-sulbactam.More than 90％ strains of the coagulase negative Staphylococcus and Enterococcus were susceptible to linezolid and teicoplanin.Overall,82.5％ of the coagulase negative Staphylococcus isolates were resistant to methicillin.Three E.coli isolates and 4 K.pneumoniae isolates were found resistant to carbapenems,and 14 Enterococcus isolates were resistant to vancomycin.Conclusions Gram-negative bacilli are the major pathogens from blood samples in hematology ward,which show high susceptibility to piperacillin-tazobactam and cefoperazone-sulbactam,imipenem and meropenem.The grampositive cocci show high susceptibility to linezolid and teicoplanin.These data are helpful for empirical antimicrobial therapy.

病历资料患者女,60岁.因"乏力4月余"于2015年9月7日入院.2015年5月患者感冒后出现乏力、睡眠欠佳,后乏力逐渐加重,不伴发热、咳嗽、腹痛、关节痛等不适.2015年7月患者日常活动后出现心慌、气短,行血常规检查:WBC4.17×109/L,Hb78g/L,PLT352×109/L,Coombs试验阴性,血清铁蛋白527.1μg/L, 血清铁48.91μmol/L,血清叶酸及维生素B12水平正常,未接受任何治疗.8月26日检查血涂片:红细胞轻度大小不等,部分体积偏大,血小板散在,小丛易见.骨髓细胞学示:取材欠佳,制片、染色良好,骨髓有核细胞增生减低,粒系占65％,未见幼粒细胞,分叶核细胞比值偏高,幼红细胞未见,未见巨核细胞,未见血液寄生虫及其他.诊断意见:幼粒、幼红及巨核细胞均未见,请结合临床诊断.骨髓活检:取材、制片、染色良好,造血组织增生极度低下,造血细胞占小梁间隙5％,粒红系细胞罕见,巨核细胞未见,骨小梁正常.免疫组化:CD34(-) CD117(-) CD61(-) TDT(-) MPO(-) CD42b(-).

>骨髓增生异常综合征（MDS）的发病机制包括基因、表观遗传学、凋亡、分化和细胞因子环境的异常等。造血干细胞（HSC）与骨髓基质细胞、微环境分泌的细胞因子或化学因子之间的相互作用是维持正常造血所必须的 [1] 。证据显示MDS的无效造血可能由于骨髓微环境异常,包括造血-基质相互作用的改变、生长因子和造血调节因子的异常产生 [2] 。

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematological malignancy that is derived from the precursors of plasmacytoid dendritic cells. Although BPDCN typically manifests in the skin, it can also evolve into a leukemic form or be complicated by acute myeloid leukemia, and some cases involve preceding myelodysplastic syndrome. We report the case of a 73-year-old Chinese man who presented with 2-year-old erythematous papules on his prothorax and back. Skin biopsy and immunohistochemistry revealed that the cells exhibited an immature blastic appearance (positive for CD4, CD123, and CD56, and negative for CD2, CD3, and CD20), which indicated a diagnosis of BPDCN. Due to his age, the patient was treated using methylprednisolone (20 mg/m2/day on days 1-7), which was followed by a mini-CHOP regimen (cyclophosphamide, doxorubicin, vincristine, and prednisone). We observed a significant treatment effect, and report this case to describe and discuss our experience with this successful treatment.