OBJECTIVETo evaluate the efficacy and safety of autologous peripheral blood stem cell transplantation (auto-PBSCT) after high dose melphalan in patients with POEMS syndrome.

METHODSNine patients including 6 males and 3 females received 10 auto-PBSCT after high dose melphalan in our hospital from June 2005 to October 2009. The median age at transplantation was 44 (39 - 48) years. The median time from onset of disease to transplantation was 12 (5 - 60) months. Peripheral stem cells were mobilized by G-CSF alone in one patient and 8 patients by G-CSF plus chemotherapy. Two patients were conditioned by melphalan 140 mg/m(2) and 7 by melphalan 200 mg/m(2). The median number of MNC was 3.75 (1.05 - 8.33) × 10(8)/kg, and that of CD34(+) cell was 5.37 (1.32 - 10.90) × 10(6)/kg.

RESULTOne patient received tandem auto-PBSCT and others received single one. Stem cell engrafted in all but 1 patient who died of severe infection and acute renal failure on day 9 after transplantation. Eight patients were evaluable for response. The median time to ANC ≥ 0.5 × 10(9)/L and platelet ≥ 20 × 10(9)/L was 10 (9 - 11) and 11.5 (9 - 14) days respectively. Two patient reached negative immunofixation electrophoresis (IFE) after stem cell mobilization and transplantation respectively, and the other 6 remained IFE postive after auto-PBSCT. Skin changes and edema of lower extremities were improved in 5 of 6 patients, lymphadenopathy relieved in 1 and papilledema improved in 2 of 3 patients. All but 1 patient achieved gradual neurologic improvement after transplantation.

CONCLUSIONPBSCT is an effective and safe therapy for POEMS syndrome patients with low treatment related mortality.

Granulocyte Colony-Stimulating Factor ; therapeutic use ; Hematopoietic Stem Cell Mobilization ; Humans ; Melphalan ; therapeutic use ; POEMS Syndrome ; Peripheral Blood Stem Cell Transplantation

Background: Acute aortic dissection is known as the most dangerous aortic disease, with management and prognosis determined as the disruption of the medial layer provoked by intramural bleeding. The objective of this study was to evaluate the safety and necessity of antiplatelet therapy on patients with Stanford Type B aortic dissection (TBAD) who underwent endovascular aortic repair (EVAR). Methods: The present study retrospectively analyzed 388 patients with TBAD who underwent EVAR and coronary angiography. The primary outcomes were hemorrhage, death, endoleak, recurrent dissection, myocardial infarction, and cerebral infarction in patients with and without aspirin antiplatelet therapy at 1 month and 12 months. Results: Of those 388 patients, 139 (35.8%) patients were treated with aspirin and 249 (64.2%) patients were not treated with aspirin. Patients in the aspirin group were elderly (57.0 ± 10.3 years vs. 52.5 ± 11.9 years, respectively, χ2 = 3.812, P < 0.001) and had more hypertension (92.1% vs. 83.9%, respectively, χ2 = 5.191, P = 0.023) and diabetes (7.2% vs. 2.8%, respectively, χ2 = 4.090, P = 0.043) than in the no?aspirin group. Twelve patients (aspirin group vs. no?aspirin group; 3.6% vs. 2.8%, respectively, χ2 = 0.184, P = 0.668) died at 1?month follow?up, while the number was 18 (4.6% vs. 5.0%, respectively, χ2 = 0.027, P = 0.870) at 12?month follow?up. Hemorrhage occurred in 1 patient (Bleeding Academic Research Consortium [BARC] Type 2) of the aspirin group, and 3 patients (1 BARC Type 2 and 2 BARC Type 5) in the no?aspirin group at 1?month follow?up ( χ2 = 0.005, P = 0.944). New hemorrhage occurred in five patients in the no?aspirin group at 12?month follow?up. Three patients in the aspirin group while five patients in the no?aspirin group had recurrent dissection for endoleak at 1?month follow?up (2.3% vs. 2.2%, respectively, χ2 = 0.074, P = 0.816). Four patients had new dissection in the no?aspirin group at 12?month follow?up (2.3% vs. 3.8%, respectively, χ2 = 0.194, P = 0.660). Each group had one patient with myocardial infarction at 1?month follow?up (0.8% vs. 0.4%, respectively, χ2 = 0.102, P = 0.749) and one more patient in the no?aspirin group at 12?month follow?up. No one had cerebral infarction in both groups during the 12?month follow?up. In the percutaneous coronary intervention (PCI) subgroup, 44 (31.7%) patients had taken dual?antiplatelet therapy (DAPT, aspirin + clopidogrel) and the other 95 (68.3%) patients had taken only aspirin. There was no significant difference in hemorrhage (0% vs. 1.1%, respectively,χ2 = 0.144, P = 0.704), death (4.8% vs. 4.5%, respectively, χ2 = 0.154, P = 0.695), myocardial infarction (2.4% vs. 0%, respectively,χ2 = 0.144, P = 0.704), endoleak, and recurrent dissection (0% vs. 3.4%, respectively, χ2 = 0.344, P = 0.558) between the two groups at 12?month follow?up. Conclusions: The present study indicated that long?term oral low?dose aspirin was safe for patients with both TBAD and coronary heart disease who underwent EVAR. For the patients who underwent both EVAR and PCI, DAPT also showed no increase in hemorrhage, endoleak, recurrent dissection, death, and myocardial infarction.

OBJECTIVETo analyze efficacy of radiotherapy for adult patients with Langerhans cell histiocytosis (LCH).

METHODSClinical features and efficacy of radiotherapy for biopsy-proven adult patient with LCH from January 2000 to October 2012 in our hospital were retrospectively analyzed.

RESULTSSeventeen (11 male and 6 female) adult LCH patients with a mean age of 31 (18-56) years old were treated by irradiation, all patients presented as single-system disease. The mean duration from diagnosis to irradiation was 8.3 (0-108) months. Although 12 of 17 patients (70.6%) had short-term response to radiotherapy, all patients but one (94.1%) progressed during long-term follow-up, the mean progression-free survival (PFS) was 14 (0-131) months. Of the progressed patients, one relapsed in situ, the remaining 15 patients progressed outside the irradiated region. Thirteen patients (76.5%) eventually progressed to multisystem disease.

CONCLUSIONThough radiotherapy for LCH in adults produced a high short-term response up to 70.6%, most of patients eventually progressed in situ or outside the irradiation region during long-term follow-up.

Adolescent ; Adult ; Disease Progression ; Disease-Free Survival ; Female ; Histiocytosis, Langerhans-Cell ; radiotherapy ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Treatment Outcome ; Young Adult

OBJECTIVETo compare the efficacy and adverse effects between arsenic trioxide (ATO) and all-trans retinoic acid (ATRA) in patients with acute promyelocytic leukemia (APL).

METHODSThe clinical data of 71 patients with newly diagnosed APL were retrospectively analyzed. Two groups were classified according to the induction regimens, namely ATO group (n = 41) and ATRA group (n = 30). The complete remission (CR) rate and the time to CR were compared between these two groups.

RESULTSThe CR rate was 97.5% in ATO group and 93.3% in ATRA group (P > 0.05). The median time to CR was 29 days (21-45 days) in ATO group, which was significantly shorter than 38.5 days (24-63 days) in ATRA group (P < 0.001). Retinoic acid syndrome occurred in 52.9% of patients treated with ATRA, which affected the further use of ATRA.

CONCLUSIONSBoth ATO and ATRA have high response rates for newly diagnosed patients with APL. Compared with ATRA, ATO induction therapy has shorter time to achieve CR and less adverse effects, and therefore may be the first-line therapy for APL.

Adolescent ; Adult ; Aged ; Arsenicals ; adverse effects ; therapeutic use ; Female ; Humans ; Leukemia, Promyelocytic, Acute ; drug therapy ; Male ; Middle Aged ; Oxides ; adverse effects ; therapeutic use ; Remission Induction ; Retrospective Studies ; Treatment Outcome ; Tretinoin ; adverse effects ; therapeutic use ; Young Adult

OBJECTIVETo explore the changes of CD(14)(+) monocyte human leucocyte antigen DR (HLA-DR) and their relationship with multiple organ dysfunction syndrome (MODS) in severe sepsis.

METHODSNinety-one patients with a definite diagnosis of severe sepsis in the intensive care unit (ICU) were included. CD(14)(+) monocyte HLA-DR levels were detected by flow cytometry on the first, 4th and 7th days of the study, and Marshall scores and prognosis on day 28 were evaluated.

RESULTSThirty-four patients died within 28 days following the onset with a mortality rate of 37.4%. Persistently lowered levels of HLA-DR were detected and significantly increased Marshall scores were found in the fatal cases at all the time points (P<0.001). In the surviving patients, the levels of HLA-DR were significantly increased (P<0.01) and Marshall scores were gradually decreased (P<0.001). During the observation period, the levels of HLA-DR decreased significantly as the number of dysfunctional organs and Marshall scores increased (P<0.001). The levels of HLA-DR were significantly increased in severe sepsis patients with 2-4 dysfunctional organs and Marshall score of 5-12 (P<0.05 or P<0.001). No changes in HLA-DR levels in severe sepsis patients with 5-6 dysfunctional organs and Marshall scores of 13-22. The levels of HLA-DR showed a significant inverse correlation to Marshall scores (r=-0.368, P<0.001).

CONCLUSIONIn patients with severe sepsis, persistent low CD(14)(+) monocyte HLA-DR levels predicts high mortality. The levels of HLA-DR are significantly correlated to the severity of organ dysfunction.

Adult ; Aged ; Aged, 80 and over ; Female ; HLA-DR Antigens ; metabolism ; Humans ; Lipopolysaccharide Receptors ; Male ; Middle Aged ; Monocytes ; immunology ; metabolism ; Multiple Organ Failure ; pathology ; Sepsis ; immunology ; metabolism

The paper introduces the general thinking,methods and basic principles for study on the operating procedure of informatization work in Traditional Chinese Medicine (TCM) hospitals,and sets up the content frame including hardware system,software system,information security,relevant operation sheets,etc.,so as to supplement the.standards for informatization work in TCM hospitals at the operating level and provide support for secure,reliable and sustainable operation of information systems in TCM hospitals.

Objective: To investigate clinic-pathological characteristics, diagnosis, treatment and prognosis of intravascular large B cell lymphoma (IVLBCL) in China. Methods: Clinical and pathological records were analyzed from 12 IVLBCL patients diagnosed between Jan 2010 to Jun 2016. Kaplan-Meier method was used to estimate overall survival (OS), and univariate analysis was performed to identify prognostic factors. Results: A series of 12 patients with IVLBCL (median age, 53.8 years; range, 32-76 years; 6 males and 6 females) was reviewed. Fever was the most common symptom (10/12), respiratory symptoms (cough, pleural effusion, dyspnea, 50%) and hemophagocytic lymphohistiocytosis (50%) were frequently observed, and only 12 patients had neurological symptom. All patients had elevated lactic dehydrogenase and serum ferritin. International Prognostic Index score was high in 75% of total patients. All patients had extra-nodal involved, pulmonary (6/12) and bone marrow (4/12) were frequently involved. Large lymphoid cells within vessel lumina or sinuses were observed in all patients. These cells were large, with scant cytoplasm, vesicular nuclei, and one or more nucleoli, and the structures of vessels and sinus were reserved. CD20 and CD79a were positive in all cases. 11patients received rituximab combined CHOP regimen chemotherapies, overall response rate (ORR) was 90.1%, and complete response rate was 66.7%. Median survival time and median progression time were not reached after a median follow-up of 20 months. Univariate analysis revealed that no clinical characters were associated with OS. Conclusion: As a rare variant of DLBCL, IVLBCL presented with pulmonary involved frequently, and trans-bronchial lung biopsy had good positive rates. Rituximab contained chemotherapy was the backbone for IVLBCL.
Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; China ; Female ; Humans ; Lymphoma, Large B-Cell, Diffuse ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Rituximab

Objective: To report the first case of lymphomatoid gastropathy in China, and to demonstrate the clinical characteristics, diagnostic approach, treatment and prognosis in this kind of patients. Methods: One patient was diagnosed as lymphomatoid gastropathy at Peking Union Medical College Hospital, and her clinical characteristics, lab data, treatment and follow-up outcomes were reviewed. Results: A case of a 51-year-old female was presented, who underwent esophagogastroduodenoscopy (EGD) due to slight epigastric discomfort. EGD revealed multiple ulcers and erosions. Biopsies showed atypical lymphocytes infiltration with CD3(+), CD56(+), CD20(-), CD8(-), TIA(+), Granzyme B(-) and Ki-67 (75%). Epstein-Barr virus-encoded RNA in situ hybridization was negative. Four months later, repeated EGD examination showed regression of the lesions without specific treatment. Conclusion: Lymphomatoid gastropathy was a unique disease entity mimicking NK/T-cell lymphomas in pathology, with the quite different profile of treatment and prognosis. It's important to consider this issue during the differential diagnosis to avoid any excessive treatment.
Biopsy ; China ; Female ; Humans ; Immunophenotyping ; In Situ Hybridization ; Lymphatic Diseases ; Lymphoma, T-Cell, Peripheral ; Middle Aged ; Stomach Diseases

OBJECTIVETo evaluate the efficacy of porcine anti-human lymphocyte globulin (P-ALG) plus cyclosporine A (CsA) therapy for severe aplastic anemia (SAA).

METHODSForty-eight SAA patients (31 males, 17 females) including 17 very severe aplastic anemias (vSAA) were treated with ALG plus CsA between 1999 to 2009 in our hospital and the outcomes were analyzed retrospectively for early mortality, response rate and quality, survival rate, toxicity and complications.

RESULTSThe median age was 28 (13 - 64) years. The interval from diagnosis to treatment was 45 days. The median neutrophil count at diagnosis was 0.178 × 10(9)/L. Overall response was 83.3% (54.2% complete, 29.2% partial) with a median time of 90 (23 - 380) days. 10.4% died of infection within 30 days mainly of fungi infection. Only 1 patient relapsed 2 years after treatment. No clonal disease was found. The 1.5-year survival rate was 87.5%. vSAAs had less response, higher early mortality and less survival (64.7%, 29.4% and 51.8%, respectively) compared to that of SAA (93.5%, 0, 100%, respectively, P < 0.05). Grouped patients with different age, gender, intervals between diagnosis and treatment and pre-existing infections had similar response. The main side effects were fever and skin rash (52.1%), serum sickness (16.7%), impaired liver function (60.4%) and hemorrhage (2.1%). No treatment-related mortality was found.

CONCLUSIONP-ALG plus CsA is an ideal and well tolerated treatment for SAA but not for vSAA.

Adolescent ; Adult ; Anemia, Aplastic ; drug therapy ; Animals ; Antilymphocyte Serum ; therapeutic use ; Cyclosporine ; therapeutic use ; Female ; Humans ; Immunosuppressive Agents ; therapeutic use ; Lymphocytes ; immunology ; Male ; Middle Aged ; Retrospective Studies ; Swine ; Treatment Outcome ; Young Adult