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 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.

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.

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 analyze the clinical characteristics of AIDS-related non-Hodgkin lymphoma(ARL)and review relative literature for the diagnosis and treatment of ARL.Method The clinical data of ARL patients admitted to Peking Union Medical College Hospital from April 2009 to April 2011 were retrospectively analyzed.Results Five male ARL patients aged 32 to 65 years old were included in this retrospective study.Among them,two patients were found to be HIV-positive for the first time,three were on regular highly active anti-retroviral therapy(HAART)for 7-8 months before the emergence of lymphoma-related symptoms.CD4+ T cell count was(69-232)× 106/L at presentation.Two patients firstly presented with sore throat and throat ulcer,one with cervical nodules,one with pelvic mass,one with fever and edema in right thigh.Through pathological analysis,four patients had B cell-originated lymphoma,with one Burkitt lymphoma and three diffuse large B cell lymphomas; one patient had T-cell lymphoma.Four patients were treated with chemotherapy,with one complete remission,one relapse,one non-response,and one death.One patient had radiotherapy only and had progressed disease.Bone marrow suppression and gastrointestinal disturbance were the main adverse effects of chemotherapy.Conclusions Lymphoma should be considered in any HIV-infected patients presented with unexplainable adenopathy,recurrent sore throat or throat ulcer,or fever of unknown origin.Biopsy should be rigorously carried out.Appropriate chemotherapy,together with HAART,may improve the prognosis greatly.

Objective To explore the diagnostic value of oligoclonal band (OB) detected by isoelectric focusing (IEF) with immunoblotting in inflammatory demyelinating diseases (IDD) in nervous system.Methods Serum and cerebrospinal fluid (CSF) OB was detected by IEF with immunoblotting in 112 patients with IDD ( multiple sclerosis ( MS):n = 48;neuromyelitis optica ( NMO):n = 21:acute disseminated encephalomyelitis ( ADEM):n = 4;secondary IDD from systemic autoimmune diseases:n = 19;peripheral nervous system IDD:n =20) and 24 patients with non-inflammatory neurological disease (NIND).Results CSF-restricted OB was detected in 91.7% (44/48) of MS patients,23.8% (5/21) of NMO patients(x2nmO vs MS= 32.679),1/4 of ADEM patients (Fisher' s excact test),15.8% (3/19) of secondary IDD patients (x2secondary IDD vs MS = 37.425 ),0 of peripheral nervous system IDD patients (x2peripheral nervous system IDD vs MS =37.425) and 0 of NIND patients (x2NIND vs MS =37.425).MS patients had significantly higher percentage of patients with CSF-restricted OB ( all P ＜0.01),compared with NIND and other IDD patients.The sensitivity and specificity of OB detected by IEF with immunoblotting for MS were 91.7% and 89.8%,which were higher than that of OB detected by other methods.Identical serum and CSF OB ( mirror pattern ) was detected in 2 of 4 ADEM patients and 1 of 48 MS patients.Conclusion IEF with immunoblotting to detect OB is a reliable method of diagnosis for MS.

Objective To investigate the characteristics of subcutaneous panniculitis-like T cell lymphoma ( SPTCL) in order to facilitate prompt identification and proper treatment of this rare and heterogeneous disease entity. Methods For 8 patients who had been misdiagnosed as rheumatic diseases but eventually confirmed as SPTCL though pathology and immunohistochemistry, a retrospective chart review was made with regard to their clinical symptoms, laboratory test results, pathological features, responses to therapy as well as outcomes. Results These 8 patients with a male to female ratio of 1: 1 were initially misdiagnosed as a variety of rheumatic diseases such as erythema nodosa, nodular panniculitis, systemic vasculitis,etc. The period from the onset of symptoms to the confirmation of diagnosis as SPTCL was 28. 6 months on average (range, 4-84 months). All the cases presented with multiple subcutaneous nodules, plaques or tumors which involved various anatomic sites including the head and neck, the trunk, and the extremities. Fever was the most frequently accompanying symptom (7/8) , followed by lymphadenopathy (4/8) , hepatomegaly (3/8), splenomegaly (3/8). Hemophagocytic phenomenon was seen in 3 cases.A total of 22 times of biopsy involving multiple anatomic sites were performed on these 8 cases with 2. 75 times on average (range, 1-5 times). All cases demonstrated a dense lymphoid infiltrate with significant cellular heteromorphism located in the subcutaneous tissue. CD_3 was positive in the majority of the cases.Immunostaining for γδTCR was positive in one case. The anti-rheumatic therapy including steroids and immunosuppressants administered before the identification of SPTCL attained minimal therapeutic effect. In contrast, 6 cases gained partial response after chemotherapy except that the other 2 cases died of fatal pulmonary infiltration and subsequent infection. Conclusions SPTCL is a rare and heterogeneous entity which is unseldomly misdiagnosed as rheumatic disease. The anti-rheumatic therapy including steroids and immunosuppressants can attain minimal therapeutic effect. Early identification by means of histology and immunohistochemistry as well as immunostaining for PCR is critical for proper treatment.