Disseminated visceral Kaposi sarcoma (KS) following allogeneic haematopoietic stem cell transplantation (HSCT) is a rare but life-threatening posttransplant complication. A suitable management strategy for disseminated KS involvement in transplant patients is unclear. Here, we reported a patient who developed disseminated visceral KS following HSCT, which was the first detailed report documenting the relationship among KS development, delayed immune reconstitution, and HHV-8 DNA levels by metagenomic next-generation sequencing (mNGS). The HHV-8 viral load peaked at 2071 sequence reads with an absolute lymphocyte count of 0.17×109/L on day +242. On day +536, the HHV-8 viral load became undetectable, with an absolute lymphocyte count of 1.06×109/L and the KS disappearance. HHV-8 load in blood detected by mNGS may be used as an early prediction marker for KS, a guide for early withdrawal of immunosuppression, and a tool to monitor KS treatment response in the setting of HSCT, especially in patients with CMV-seropositive or graft failure postengraftment. Through whole-exome sequencing, we explored the molecular mechanism underlying the patient's longer latency of haematopoietic or immune reconstitution and recurrent infections. Germline mutations in the FANCI and RAD51 genes might impair the patient's DNA repair ability, leading to a degree of immunodeficiency and tumour susceptibility. We strongly recommended evaluating the clinical history of the donor and investigating whether there were possible germline mutations suspected for immunodeficiency or familial neoplasms. Disseminated visceral KS patients could likely benefit from chemotherapy, especially if the disease appears to be aggressive.

肺癌的早期诊断目前尚缺乏有效手段,特别是以肺外表现即副癌综合征和转移灶为首发症状的肺癌,易误诊、误治.以红细胞增高和血性胸水起病,确诊为肺癌继发性红细胞增多症的病例之前在国内外均未见报道.本文现报道1例以血性胸水起病的肺低分化腺癌继发红细胞增多症患者的疾病发展及转归,并复习相关文献.

目的:研究自身免疫性溶血性贫血(AIHA)患者的红细胞寿命,并与常见贫血患者的红细胞寿命比较.方法:回顾性分析AIHA、骨髓增生异常综合征(MDS)、重型再生障碍性贫血(SAA)和缺铁性贫血(IDA)4组共55例患者的临床资料,以同期12例健康志愿者为正常对照组.采用一氧化碳(CO)呼气法测定红细胞寿命,比较4种贫血患者和健康志愿者红细胞寿命并作分析.结果:AIHA患者红细胞寿命为(30.41±31.12)d,比MDS(53.44±32.61)d、SAA(54.53±22.56)d和IDA(58.75±31.29)d患者的红细胞寿命均缩短(P＜0.05),4组患者红细胞寿命均较12例健康志愿者红细胞寿命(118.16±25.88)d显著缩短(P＜0.01).结论:红细胞寿命缩短参与了AIHA、MDS、SAA和IDA患者贫血的发生,但在不同贫血疾病中所起作用程度不尽相同.

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.

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 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.

Microvesicles (MVs) are carriers of molecular and oncogenic signatures present in subsets of tumor cells and tumor-associated stroma, and a focus of cancer research. Although methods to detect MVs are mature, we were concerned that the buffer used could lead to false results when quantitating MVs by flow cytometry. In this work,we detected MVs by flow cytometry withthree different solutions: water, saline, and phosphate-buffered saline (PBS). The results demonstrated that PBS, when reacted with annexin V binding buffer, produced nano-sized vesicles even when there were no MVs in the sample. No similar events occurred in the saline and water groups (P < 0.01). Annexin V positive rate increased significantly when PBS was used as the buffer, compared to saline and water. These false negative results were also observed when we quantified some markers of MVs such as CD3 and CD19. A probable explanation for these findings is the production of insoluble Ca(H2PO4)2 or Ca3PO4 from calcium in the binding buffer and phosphate in PBS. Thus, considering the osmotic pressure of water, we suggest that saline is a more suitable buffer when counting MVs by flow cytometry.

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(-).

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.

Aggressive natural killer cell leukemia (ANKL) is a fatal hematological neoplasm characterized by a fulminating clinical course and extremely high mortality. Current diagnosis of this disease is not effective during the early stages and it is easily misdiagnosed as other NK cell disorders. We retrospectively analyzed the clinical characteristics and flow cytometric immunophenotype of 47 patients with ANKL. Patients with extranodal NK/T cell lymphoma, nasal type (ENKTL) and chronic lymphoproliferative disorder of NK cell (CLPD-NK), who were diagnosed during the same time period were used for comparisons. Abnormal NK cells in ANKL were found to have a distinctiveCD56bright/CD16dim immunophenotype and markedly increased Ki-67 expression, whereas CD57 negativity and reduced expression of killer immunoglobulin-like receptor (KIR), CD161, CD7, CD8 and perforin were exhibited compared with other NK cell proliferative disorders (p<0.05). The positive rates of flow cytometry detection (97.4%) was higher than those of cytomorphological (89.5%), immunohistochemical (90%), cytogenetic (56.5%) and F-18 fluorodeoxyglucose positron emission tomography/computer tomography (18-FDG-PET/CT) examinations (50%) (p<0.05). ANKL is a highly aggressive leukemia with high mortality. Flow cytometry detection is sensitive for the early and differential diagnosis of ANKL with high specificity.

The purpose of this study was to assess the accuracy of the parameters used in conventional hemostatic testing and thromboelastography (TEG) in predicting bleeding risk in patients with hematologic diseases. Patients diagnosed with a hematologic disease were divided into bleeding (n = 125) and non-bleeding (n = 509) groups according to clinical signs and symptoms. Several parameters were measured in all patients via traditional hemostatic testing and TEG, including platelet counts (PLT) and maximum amplitude (MA). The sensitivity and specificity of each parameter for predicting bleeding risk were determined via receiver operating characteristic curves. PLT had a sensitivity of 81.1 % and a specificity of 74.4 %, and MA had a sensitivity of 74.7 % and a specificity of 72.0 %. Specificity was higher for both parameters together (77.6 %) than for either alone (P < 0.01). In a subgroup analysis of patients with PLT < 20 × 10(9)/L, sensitivity was higher for both parameters together (84.6 %) than for either alone (P = 0.003). Although all parameters evaluated predicted bleeding risk, PLT and MA were especially accurate. We suggest that the combination of PLT and MA better assesses bleeding risk than do other parameters and that the use of this metric may help to guide decisions regarding platelet transfusion in patients with thrombocytopenic hematologic diseases.

The objective of this study was to explore prognostic factors in lymphoma patients with bone marrow involvement (Ann Arbor stage IV). To that end, we analyzed a cohort study of 68 stage IV lymphoma patients. We found that the most predictive thresholds for lymphocyte rate, monocyte rate, and lymphocyte to monocyte ratio (LMR) were 30%, 13%, and 3, respectively. A lymphocyte rate <30%, a monocyte rate >13%, and the presence of B symptoms were associated with shorter OS. LMR >3, Eastern Oncology Cooperative Group performance status ≤1, indolent lymphoma, and B cell (as opposed to T and NK cell) lymphoma predicted longer OS. Our study showed that these basic, easily acquired data can predict the outcome and overall survival in lymphoma patients with bone marrow involvement. These prognostic markers should be taken into consideration when devising new prognostic scoring systems for lymphomas.

Chronic myeloid leukemia (CML) is most frequently observed in middle-aged individuals. In most patients, normal marrow cells are replaced by cells with an abnormal G-group chromosome, the Philadelphia (Ph) chromosome. The Ph chromosome that is characterized by the translocation (9;22) (q34;q11) is noted in 90-95% of patients diagnosed with CML. Studies have also shown that CML can be associated with various other cytogenetic abnormalities, with 5-10% of these cases showing complex translocation involving another chromosome in addition to the Ph chromosome. Here, we report the case of a Ph(+) CML patient with an inserted karyotype who presented clinically in the chronic phase but with atypical features. This case highlights the significance of cytogenetic abnormalities on the prognosis in CML.

目的:分析不同疾病所致继发性噬血细胞性淋巴组织细胞增生症(HLH)患者的临床特征,进一步加深对本病的认识.方法:回顾性分析65例继发性HLH患者的临床资料,将患者分为NK细胞淋巴瘤/白血病组、恶性肿瘤组、EB病毒相关组、风湿免疫相关组、重症感染组,比较5组患者的临床特点.结果:65例患者各种临床表现所占比例如下,100％发热,92.3％脾大,53.9％肝大,50.8％淋巴结肿大,30.8％存在水肿,20％出现皮疹.84.6％血细胞两系或三系减少,96.5％血清铁蛋白高,86.0％高甘油三酯血症,52.3％低纤维蛋白原血症,68.8％存在噬血现象,81.8％存在低或无NK细胞活性,93.9％肝酶异常,55.4％胆红素升高,96.9％低蛋白血症,72.3％低钠血症,96％高密度脂蛋白降低.5组患者在发热、脾大、淋巴结肿大、水肿、血细胞减少与否、血甘油三酯、铁蛋白水平、肝功能异常等方面无统计学差异(P＞0.05);而在肝大、皮疹、初诊时血常规、纤维蛋白原、噬血现象等方面有统计学差异(P＜0.05).结论:继发性HLH有共同临床表现,如发热、脾大、血细胞减少、肝酶异常、低蛋白血症、低钠血症等,但不同疾病所致继发性HLH有一定的特点,EB病毒相关HLH表现为发病年龄小、肝大、血细胞明显减少、CRP高、噬血现象少见,经积极治疗后预后较好.重症感染相关HLH常表现为初诊时血细胞减少不明显,甚至高于正常,且CRP明显升高.风湿免疫相关HLH常有皮疹、初诊时血细胞常不减少、无明显感染灶、抗核抗体阳性.故在临床工作中,应针对不同病因早期识别HLH,从而有效降低病死率.