Objective To evaluate the feasibility and safety of peripheral CD34~(+) cell mobilization in the patients with severe autoimmune disease.Methods The patients were mobilized by the regimen of cyclophosphamide + rhG-CSF.Results After mobilization,the MNC and CD34~(+) cells were gained by a median of 4.52(1.15～14.05)?10~(8)/kg and 5.18(1.30～26.35)?10~(6)/kg respectively.There were 19 case times among which the patients' ANC

POEMS syndrome is a rare clonal plasma cell disease characterized by polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. Significant advances have been made in the diagnosis and treatment of POEMS syndrome over the last decade. In this study, we reviewed the diagnostic criteria and characteristic features of POEMS syndrome. We also focused on the role of the characteristic features of POEMS syndrome in early diagnosis. Autologous peripheral blood stem cell transplantation has become the first-line treatment for younger patients with normal organ function because it has resulted in a high response rate and durable remission. Melphalan and dexamethasone is an effective and well-tolerated treatment for older patients and those with organ dysfunction. Patients with poor performance status or renal function can benefit from novel agents that can also improve transplantation eligibility.

Medical charts of tuberculotic patients with secondary autoimmune hemolytic anemia (AIHA) admitted to Peking Union Medical College Hospital (PUMCH)from 2006 to 2015 were retrospectively reviewed.Four cases with complete information of laboratory tests,imaging findings and bone marrow results were included.Therapy regimens and outcomes of survival were followed by phone call if needed.Ages at diagnosis of AIHA were 58-80 years old,hemoglobin levels were 31-73 g/L,Coomb tests were negative in all cascs.Thcrapy of cortical steroid did not work,while anti-TB therapy (including isoniazid,rifampicin,ethambutol) significantly improved hemoglobin to normal level in three cases during six to nine months.One case died within one week,for progressive TB to central neurological system.As one of rarc complications of TB,AIHA may be fatal.Early diagnosis and appropriately anti-TB therapy is helpful for good outcomes.

Objective To investigate the characteristics and related risk factors of patients with lymphoma complicated with tuberculosis (TB), the possible pathogenesis and effective therapy. Methods Twenty-one cases of lymphoma combined with TB were retrospectively analyzed by clinical manifestations,pathological features,diagnostic methods,treatments and prognosis.Results Six cases were diagnosed as TB after the diagnosis of lymphoma,5 cases of which were undergoing chemotherapy for lymphoma; 13 cases were diagnosed as lymphoma after TB,including 6 of 10 obsolete pulmonary TB were reactivated during undergoing chemotherapy. Two cases were diagnosed lymphoma and TB simultaneously. Six cases of 21 cases were Hodgkin lymphoma, whereas the other 15 cases were non-Hodgkin lymphoma. Conclusion TB and lymphoma may exist in the same patient in areas where TB is endemic; among this population,the proportion of Hodgkin lymphoma was higher than in the general population.Therefore,the possibility of TB reactivation when undergoing chemotherapy must be focused on.

Objective To establish a new method using Flaer for detecting abnormal clone in patients with paroxysmal nocturnal hemoglobinuria(PNH).Methods Peripheral WBC and bone marrow mononuclear cells from patients with PNH and normal controls were isolated and stained with flaer,CD55 PE,CD59 FITC and CD34 PE.Results PNH cells can easily be distinguished by flare.Compared with CD55,CD59,Flaer showed advantage in detecting minor clone of PNH,either in peripheral blood or in CD34+ bone marrow cells.Conclusion Flaer can be a new,simple and effective method to detect PNH clone,and especially when the PNH clone is small.

Objective To evaluate the efficacy and safety of prophylactic cefiazidime on early bacterial infection in APBSCT recipients during neutropenia.Methods APBSCT recipients were prospectively randomly assigned to intravenous ceftazidime treatment group and control group (no prophylaxis of antibiotics).The treatment started from the first day until resolution of neutropenia or the appearance of early bacterial infection.Results From March 2010 to January 2013,70 APBSCT recipients were enrolled in the study with 36 in treatment and 34 in control group.Overall,29 (41.4％) patients developed early bacterial infection,among which,9(25.0％) in the treatment group and 20(58.8％) in the control group (P =0.004).The median infection free survival (IFS) was not reached in the treatment group and was 8 days in the control group (P =0.005).Despite whether patients received single high dose melphalan or other conditioning regimes,the early bacterial infection rate was lower in the treatment group than in the control group,and the median IFS was longer in the treatment group than that in the control group.The mean courses of antibiotic administration were (8.08 ± 2.03) days and (3.68 ± 3.56) days respectively in the treatment and control groups (P ＜ 0.001).However,the duration of empirical carbapenems were (1.67 ±3.03) days and (3.68 ±3.56) days respectively (P =0.013).There was no significant difference of antibiotics cost per patient between the two groups.Four patients in the treatment group had a transient elevated serum creatinine.Overall,no infection related mortality was observed in either group.Conclusions Prophylaxis of intravenous ceftazidime for APBSCT recipients is effective in preventing early bacterial infection with an acceptable toxicity and cost profile.However,it doesn't have effect on infection related mortality.Therefore,our results do not support the use of antibiotic prophylaxis for patients undergoing APBSCT.

Plasmablastic myeloma is a rare pathological classification of multiple myeloma. This condition must be differentially diag-nosed because of lack of a distinctive phenotype. Involvement of the central nervous system is a rare complication of multiple myelo-ma. The choice of treatment is important for plasmablastic myeloma. Thus, this article presents a rare case of plasmablastic myeloma with multiple cranial nerve involvement. We clarify the diagnosis through the multidisciplinary team and select the optimal therapy for the patient.

Objective To explore the diagnostic value of morphology in patients with blastic plasmacytoid dendritic cell leukemiafrom bone marrow cell.Methods Clinical data of 5 patients with BPDCN leukemia in Peking Union Medical College Hospital from 2011 to 2016were collected.The morphological characteristics of the cell size,nuclei,chromatin and cytoplasm of the BPDCNtumor cells in the bone marrow smears were observed under the microscope.Results The clinical manifestation of the 5 cases involved skin lesions (5/5,100％),lymphadenopathy (4/5,80％),hepatomegaly (3/5,60％),splenomegaly (4/5,80％).Immunophenotype showed all cases were positive for CD4,CD56 and CD123.The morphologiesof BPDCN cell characterized by heterogenous clls,were frequent,including,frequent mediumsized cells with a round or irregular nucleus,lacy chromatin,basophilic cytoplasm with lack of granules,marked large pseudopodia and vacuolation which may arranged as pearl necklace along the cytoplasmic outline.They might mimicking pseudomonoblast,Pseudolymphoblast or Pseudolymphoma cell.Conclusions Plasmacytoid dendritic cells had some peculiar morphological features,the patients suffered from the clinical manifestation of skin lesion may highly suggested the diagnosis of BPDCN,Flow cytometry and pathological biopsy are necessary for the final diagnosis of BPDCN.

Objective To improve the anderstanding of clinical profile of primary plasma cell leukemia.Methods Case report and literature review.Results A rare case of nervous system relapse in primary plasma cell leukemia was reported.Six patients were identified from the literature.The type of immunoglobulin included IgG(3 patients),IgD(2 patients).Clinical manifestations of nervous system were variable.The average interval from initial diagnosis to the development of nervous system relapse was 16.5 months.Plasma cells were found in cerebrospinal fluid in 4 patients.The mean surviaval time was 6.7 months after nervous system relapse.Conlusion Nervous system relapse in primary plasma cell leukemia is rare with poor prognosis.

Objective Myelodysplastic spndrome (MDS) patients' chromosome aberrations were detected by CC and FISH method respectively,to establish standard FISH platform of diagnosis of MDS,to compare the differences of FISH and CC in the detection of MDS chromosome aberrations.Methods Chinese MDS patients fulfilled with the Vienna minimum diagnostic criteria were tested by CC and FISH method,and the patients' clinical data was collected at the same time.Results The standard FISH platform of diagnosis of MDS was established successfully,including probe combination of-5/5q-,+8,-7/7q-,20q-,-Y,the success rate was 100 ％.In 83 cases of MDS,chromosome aberrations rate detected by two methods was 42.2 ％,30.1％ of CC method and 33.7 ％ of FISH method,the accordant diagnostic rate was 76.1％.FISH had higher positive rate than CC method (29.6 ％ vs 18.6 ％,P =0.049) in the IPSS intermedian-risk 1 group,but lower positive rate in IPSS intermedian-risk 2 group (62.5 ％ vs 81.2 ％,P =0.036).There were 11 (19.0 ％) cases of clonal chromosome aberrations detected by FISH method but not detected by CC method,in which 10 (90.9 ％) cases were intermedian-risk 1 group (IPSS grouping).Evaluate IPSS by FISH and CC respectively,the accordant rate was 86.7 ％.Evaluate IPSS by CC alone will result in 3 patients (3.6 ％)decline in risk stratification,and evaluate IPSS by FISH alone will result in 8 patients (9.6 ％) decline in risk stratification.There were 35 (42.2 ％) cases of chromosome aberrations detected by the two methods,including 13 (15.7 ％) cases of complex karyotype and 22 (26.5 ％) cases of single abnormal karyotype.The most common chromosomal aberration was +8,a total of 9 (10.8 ％) cases,followed by abnormalities of chromosome 7,a total of 7 (8.4 ％) cases.Conclusion The study has established standard FISH platform of diagnosis of MDS,the method is stable,sensitive and rapid.FISH technology joint CC technology can improve the patients with MDS chromosome aberration detection rate.Compared to CC,FISH technique can improve the detection rate of the intermedian-risk 1 IPSS grouping MDS patients.Because of limited probe,FISH technique can not entirely replace the role of CC method in MDS,but can complement each other.