Clinical and Pathological Characteristics of Aggressive Natural Killer Cell Leukemia Patients / 中国实验血液学杂志

OBJECTIVE@#To investigate the clinicopathologic characteristics, diagnosis and therapy of aggressive natural killer cell leukemia (ANKL) patients.@*METHODS@#Clinical manifestations, cellular morphology, immunophenotypic analysis by flow cytometry (FCM), TCR gene rearrangement, pathology and Immunohistochemical analysis of bone marrow (BM) were combined to diagnose the six patients with ANKL.@*RESULTS@#The median age of the patients were 35.5 years old. All the patients with fever, cytopenia and liver dysfunction. Imageological examination presented hepatosplenomegaly (6/6), and PET/CT presented diffusely increased metabolism in liver, spleen and BM (3/3). BM cytologic examination presented increased hematophagocyte at the early stage and 1%-42% leukemic cell were detected in BM with the progression of diseases. FCM showed the leukemic cells were positive for CD2(6/6), CD56(5/6), CD16(2/6), CD94(3/6), CD38(3/6), cCD3(1/5), CD8(1/6), CD7(2/6), CD57(1/6) and negative for CD3, CD4, TdT, cMPO, TCR α/β, TCR γ/δ. The neoplastic cells were negative for TCR gene rearrangement. Five cases showed increased quantitation of whole blood Epstein-Barr virus (EBV) DNA.@*CONCLUSION@#ANKL is a highly aggressive disease. Prompt and repeating BM examination is important to patient with fever, cytopenia and liver dysfunction. The diagnosis of ANKL relies mainly on the integration of clinical, morphologic, immunophenotypic finding and EBV-DNA increasement.

Effect and Clinical Significance of Bortezomib and Thalidomide on the Memory T Cells Subsets and Regulatory T Cells in Peripheral Blood of Patients with Multiple Myeloma / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To investigate the effects of bortezomib(BTZ) and thalidomide(TM) on peripheral blood memory T-cells (T) and regulatory T cells(Tregs) in patients with multiple myeloma(MM).</p><p><b>METHODS</b>Eighty-six MM patients received 2 courses of chemotherapy were divided into effective (partial response at least) group (63 cases) and ineffective (no partial response) group (17 cases) according to therapeutic efficacy; these 80 patients were divided into BTZ group (38 cases) and TM group (42 cases) yet according to therapeutic regimens, 20 newly diagnosed MM patients were used as baseline group, 30 healthy volunteers were used as healthy control group. The T subsets and Treg in peripheral blood of each groups were detected by flow cytometry.</p><p><b>RESULTS</b>The CD4 central memory T cells (CD4 T) percentage of CD4 T, the CD18 T percentage of CD18T and ratio of CD8 T and CD8 effector memory T cells (T) (CD8 T/T) in baseline group were all significantly lower than those in healthy control group (P<0.05). After treatment with BTZ regimen or TM regimen, the CD8T percentage of CD8 T in effective group significantly increased to level of healthy control group (P<0.05); the Treg cell level in effective and in effective groups was not significantly different from that in baseline group(P>0.05), but the Treg percentage of CD4 cells ineffective group was significantly higher than that in baseline group and ineffective group (P<0.05). According to ROC curve, the critical value of CD8T/T for predicting chemotherapeutic response was 0.27 with sensitivity of 57.1% and specificity of 94.1%.</p><p><b>CONCLUSION</b>When MM patients are in an immuno-exhanstive status, the treatment with BTZ or TM both can reverse the immuno-inhibitory status of MM patients, moreover, does not affect the Treg cell count; the Treg percentage in BTZ and TM effective groups both are significantly higher than that in baseline group and ineffective group. The ratio of CD8T/T contributes to evaluating the chemotherapeutic efficacy.</p>

Cost-Effectiveness Analysis of Different Chemotherapy Regimens in the Treatment of Patients with Multiple Myeloma / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To compare the pharmaco-economic effect of 3 chemotherapeutic regimens in the treatment of patients with multiple myeloma(MM).</p><p><b>METHODS</b>One hundred and thirty-eight newly diagnosed cases of MM in our hospital were analyzed retrospectively, and then MM patients were divided into group A, B and C group according to therapeutic regimen. Group A was treated with VCD therapeutic regimen (bortezomib + cyclophosphamide + dexamethasone, 63 cases), The patients in group B was treated with BiCTD therapeutic regimen (clarithromycin+cyclophosphamide+thalidomide+dexamethasone, 44 cases), The patients in group C was treated with CTD therapeutic regimen (cyclophosphamide+ thalidomide+dexamethasone, 33 cases). The clinical efficacy, adverse reaction, cost-effectiveness were observed and analysed after 4 courses of treatment among 3 groups.</p><p><b>RESULTS</b>The overall response rates of group A, B and C were 96.83%, 81.82% and 64.52% with statistical significant difference (P<0.01). The high efficiency response rates of 3 groups were 82.5%, 59.09%, 32.26% with very significant statistical difference (P<0.01). The infection rate of group A was statistically and significantly higher than other 2 groups (P=0.048), and the constipation rate in group A was statistically and significantly higer than that in group B and C (P<0.05). The cost-effectiveness ratios of 3 groups were 69567.44, 20765.12 and 21475.48, respectively. The incremental cost-effectiveness ratio of group A and B were 183933.21 and 22259.09, as compared with group C. The result was in accordance with sensitivity test.</p><p><b>CONCLUSION</b>Clinicial efficacy of group A is the best,but group B has advantages on cost-effectiveness ratio as compared with other groups, otherwise, group B has low incidence of adverse reaction. In the view of safety, therapeutic efficacy and pharmacoeconomics for treatment of patients with MM, the BiCTD regimen has been confirmed to be superior to the other 2 groups.</p>

Clinical study of neurotropin combined with methylcobalamin for the treatment of residual neurological symptoms after degenerative lumbar disease surgery / 复旦学报（医学版）

Objective To investigate the therapeutic effect of neurotropin (NTP) combined with methylcobalamin for the treatment of residual neurological symptoms after surgery for degenerative lumbar disease (DLD).Methods A total of 96 cases with residual neurological symptoms after surgery for DLD from Jan.,2012 to Jun.,2015 were enrolled in this study and divided into three groups,32 cases in each group.The patients in group A were treated with methylcobalamin,the patients in group B were treated with NTP,and the patients in group C were treated with NTP + methylcobalamin.All the patients were treated for 2 weeks.The visual analogue scale (VAS) and oswestry disability index (ODI) were observed and documented at the beginning of the treatment,14 days,3 months,6 months and 12 months after the treatment,respectively.Results The VAS and ODI scores at 14 days,3 months,6 months and 12 months after the treatment in each group decreased obviously comparing with the scores before the treatment (P＜0.05).At each time point the VAS and ODI scores in group C＜group B＜group A (P＜0.05).The excellent rate at each time point in group C＞group B＞group A (P ＜ 0.05).Conclusions NTP is effective in the treatment of residual neurological symptoms and its therapeutic effects is better than methylcobalamin.The combined application of NTP and methylcobalamin is superior to the single application of NTP or methylcobalamin for the treatment of residual neurological symptoms after surgery for degenerative lumbar diseases.

Clinical and Immunophenotypic Properties of Small Cell Variant of T-cell Prolymphocytic Leukemia / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinical, morphologic and immunophenotypic properties of the patients with small cell variant of T-cell prolymphocytic leukaemia(T-PLL).</p><p><b>METHODS</b>Peripheral blood and bone marrow cytomorphologic and immunophenotypic examination, and T-cell receptor(TCR) gene rearrangement detection were used to verify the diagnosis for 2 patients with lymphocytosis. Two patients were treated with combined chemotherapeutic protocol based on fludarabine.</p><p><b>RESULTS</b>At diagnosis of case 1, the main lymphocytes of peripheral blood smear were the small mature lymphocytes without nucleoli. The immunophenotype of the cells was CD3CD5CD7CD4CD8TCRα/β. The patient achieved complete remission after treatment with combined with CTX of fludarabine. The disease relapsed at 32 months after diagnosis. The abnormal lymphocytes were medium-sized ones with a visible nucleolus. Immunophenotyping showed that the leukemic cells were predominantly CD8 positive(CD3CD5CD7CD4CD8TCRα/β). Both the peripheral blood and bone marrow cells of case 2 were predominanthy the mature lymphocytes, and their immunophenotype was HLA-DRCD7CD5CD4CD3CD2CD56cCD3TCRα/β. The combined fludarabine therapy was ineffective.</p><p><b>CONCLUSION</b>Immunophenotypical switch from CD4CD8to CD4CD8may be associated with a poor response to chemotherapy. CD56 expression is an independent poor prognostic factor for primary refractory disease in T-PLL and may be considered for implementing risked-adapted therapeutic strategies.</p>

Acute Promyelocytic Leukemia Developed during Imatinib Therapy for Gastrointestinal Stromal Tumors / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathologic and molecular characteristics of acute promyelocytic leukemia(APL) developed during imatinib therapy for gastrointestinal stromal tumors(GIST).</p><p><b>METHODS</b>A 49-year-old woman was hospitalized for abdominal pain. The abdominal CT revealed a gastric mass. Laparoscopic resection of the tumor was performed. The histopathologic analysis showed poorly differentiated malignant cell infiltration with epithelioid features. Immunohistochemistry staining of these cells was positive for CD117 and CD34. GIST was confirmed and imatinib treatment was given.</p><p><b>RESULTS</b>After 1 year,the patient developed progressive pancytopenia. Bone marrow aspirate showed marked hyperplasia of bone marrow cells with 92.5% promyelocyte, consistent with APL. Cytogenetic analysis demonstrated t(15;17)(q22;q21) as the sole abnormality. PML/RARα fusion gene was positive and Kit mutation was negative. After combined treatment with ATRA, arsenic trioxide and idarubicin, patient achieved cytogenetic and molecular remission.</p><p><b>CONCLUSION</b>The metachronous coexistence of GIST with APL is uncommon. The potential nonrandom association and causal relationship between these malignancies remained to be investigated. Further studies would be necessary to clarify the relationship between imatinib and secondary malignancies in GIST patients.</p>

Clinicopathologic Characteristics and Outcome of Isolated Ovarian Relapse in Adolescent with Acute Lymphoblastic Leukemia / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathologic characteristics,diagnosis and treatment of isolated ovarian relapse of acute lymphoblastic leukemia(ALL).</p><p><b>METHODS</b>A 16-year-old girl presented with complaints of bone and joint pain. The peripheral blood and bone marrow(BM) smears showed 32% and 72% blasts, respectively, which were myeloperoxidase-negative. The blasts were positive for HLA-DR, TdT, CD10, CD19, CD22 and cCD79a and negative for CD34, CD5, CD7, CD13, CD33, CD56 and MPO detected by flow cytometry. BM cytogenetic analysis and fusion gene screening revealed t(1;19)(q23;p13) and E2A/PBX1. She was diagnosed as B-cell acute lymphoblastic leukemia (B-ALL) and was treated with CALGB8811 protocol. She presented lower abdominal pain with intermittent colick at 7 months after complete remission. The pelvic ultrasound showed a lobulated mixed echogenic mass in the right ovary, and an exploratory laparotomy was performed.</p><p><b>RESULTS</b>Pathologic examination and immunohistochemistry of resected ovarian tumor revealed extensive infiltration by lymphoblasts with positive for TdT, CD20, CD43 and CD79a. Further investigations failed to reveal any other extramedullary involvement. Hemogram, peripheral blood and bone marrow smear examination were unremarkable at the same time. The isolated extramedullary ovarian relapse of ALL was confirmed. Simultaneous, the detection of minimal residual disease by multiparametric flow cytometry showed positive with 5.0×10. The reinduction chemotherapy including a high-dose methotrexate and cytarabine was given to the patients. She experienced the second ovarian relapse after 1 year and refused further treatment.</p><p><b>CONCLUSION</b>Although uncommon, ovarian recurrence after chemotherapy for ALL should be considered in the patients with suggestive symptoms. Screening by pelvic ultrasonography may be valuble for early detection of pelvic disease in ALL.</p>

Clinico-pathologic Characteristics of Adult Patients with Atypical Infectious Mononucleosis / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathologic characteristics of adult patients with atypical infectious mononucleosis(IM).</p><p><b>METHODS</b>From January 2003 to December 2013, a total of 5 cases of atypical IM misdiagnosed as lymphoma were selected, and the clinico-pathological characteristics and efficacy of treatment were analyzed. Biopsy of lymph node or tonsil was performed to evaluate the possibility of lymphoma. Peripheral blood EBV antibody and EBV-DNA were examined by ELISA and real-time fluorescence quantitative PCR, respectively.</p><p><b>RESULTS</b>All the cases were considered as lymphoma on the basis of morphological features in initial evaluation before relapse. These features included a florid immunoblastic proliferation, distortion of the underlying nodal or tonsillar architecture and the presence of necrosis. The immunophenotypic features, EBV encoded RNA (EBER) in situ hybridization and the gene rearrangement of immunoglobulin or T cell receptor may be helpful for the distinction of atypical IM from lymphoma.</p><p><b>CONCLUSION</b>IM as EBV-related lymphoproliferative process shows marked clinical and histological diversity. Atypical case of IM may mimic many different type of lymphoma in clinical and pathologic features, and the misdiagnosis should be avoided by using molecular and pathological examination.</p>

Test of Serum Free Light Chain and Its Clinical Significance in Light Chain Multiple Myeloma / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinical significance of serum free light chain (sFLC) detection in light chain multiple myeloma (LCMM).</p><p><b>METHODS</b>A total of 37 newly diagnosed LCMM patients were enrolled in this study, including 17 patients with k light chain type and 20 patients with λ light chain type, the sFLC and 24 hours urine light chain (ULC) were measured before and after chemotherapy. The correlation of sFLC level with ULC and renal impairment was analyzed.</p><p><b>RESULTS</b>All the patients displayed an abnormally increased level of sFLC at diagnosis wtih median value of 105.44 mg/L and 146.39 mg/L for k and λ light chain types, respectively. The sFLC did not correlate with ULC before and after chemotherapy. Among the 12 patients with very good partial remission and normal ULC level, the sFLC still was abnormally increased in 8 patients. Renal impairment was associated with the urine λ-type light chain, and the area under the ROC curve of urine λ light chain at diagnosis is 0.792 (P = 0.031).</p><p><b>CONCLUSION</b>All patients with LCMM show an abnormally increased level of sFLC at diagnosis. sFLC can be used to monitor the response to chemotherapy because it is more sensitive for analysis of therapeutic effect than urine λ light chain.</p>

Long-term follow-up for 39 newly diagnosed diffused large B-cell lymphoma patients treated by (R)-EPOCH / 中国实验血液学杂志

The purpose of this study was to evaluate the efficacy and safety of (R)-EPOCH protocol on patients with diffuse large B-cell lymphoma(DLBCL). From February 2004 to April 2009, a total of 39 patients who suffered from DLBCL and received (R)-EPOCH protocol were enrolled in the study. The median age of patients was 52 years old. 24 patients were on stage I/II, and 15 cases were on stage III/IV. Patients with stage I/II were administered with 4-6 cycles of (R)-EPOCH, while other patients with stage III/IV received 6-8 cycles of (R)-EPOCH. DLBCL patients with bulky disease received radiotherapy after completion of chemotherapy. 39 patients received a total of 209 cycles of chemotherapy and the median chemotherapy cycles was 6 (range, 2-8 cycles). The results showed that the overall response rate of 39 assessable patients was 87.2%, including 28 patients (71.8%) in complete remission (CR) and 6 patients (15.4%) in partial remission(PR). With a median follow-up of 57.7 months, the 1-year overall survival rate was 81.8%, while 70.9% for 3-year and 58.8% for 5-year. The major toxicity of (R)-EPOCH protocol was hematologic toxicity and the incidence of grade III-IV neutropenia and anemia were 29.2% and 14.4%, respectively. Other toxicities were mild, no treatment-related deaths occurred. At the end of follow-up,no secondary tumors occurred. It is concluded that (R)-EPOCH protocol is a effective and safe protocol for the patients with DLBCL.

Significance of low molecular weight urinary protein for assessment of early renal damage in patients with multiple myeloma / 中国实验血液学杂志

This study was purposed to evaluate the clinical significance of low molecular weight urinary proteins for diagnosis of early renal damage in patients with multiple myeloma (MM). Medical records of 278 patients with MM in Nanjing School of Clinical Medicine from January 2004 to May 2012 were analyzed retrospectively. These patients were divided into 3 groups: glomerular damage group (n = 143), tubular damage group (n = 114) and normal group (n = 21). The clinical and laboratorial data were compared among them. The correlations of urinary retinol-binding protein (RBP) or urinary N-acetyl-β-D-amino-glucosaminidase (NAG) with blood urea nitrogen (BUN), Scr, blood cystatin-C (Cys-C), clearance of creatinine (Ccr), 24 h protein uria and 24 h urine light chains were further analyzed, and the correlation of renal tubulointerstitial lesion scores with low molecular weight urinary proteins in 61 patients were also analyzed. The area under curve (ROC curve) was used to evaluate and compare the discrimination of urinary RBP and urinary NAG. The results showed that glomerular damage group had higher urinary RBP than tubular damage group. However, glomerular damage group had lower urinary NAG than tubular damage group. The two groups had higher urinary RBP and urinary NAG than that in normal group. Urinary RBP related positively to the level of Scr, BUN, Cys-C, 24 h proteinurias and related negatively to the level of Ccr. Urinary NAG related positively to the level of 24 h proteinurias, Ccr and related negatively to the level of Cys-C. Renal tubulointerstitial lesions were significantly correlated with urinary RBP, but weakly correlated with urinary NAG. It is concluded that urinary RBP significantly correlates with renal tubular damage. Compared with urinary NAG, urinary RBP can better assess the extent of renal damage, and has higher specificity.

Relationship between the catalysis of Bence Jones protein and renal impairment in patients with multiple myeloma / 中国实验血液学杂志

This study was purposed to investigate the relationship between the catalysis of Bence Jones protein (BJP) in urine of patients with multiple myeloma(MM) and toxicity on the renal proximal tubular cells in vitro, and to explore the potential mechanism for the toxicity of BJP to renal impairment in patients with MM. The Michaelis-Menten constant (K(m)) and catalytic constant (k(cat)) of the amidase activity of BJP was calculated by Hanes equation. The LLC-PK1 cells were cultured with different concentration of BJP for 24 h, then proliferation of the cells were determined by MTT method and apoptosis were determined by flow cytometry. The results showed that the BJP from the MM patients with renal impairment significantly inhibited cell proliferation, as compared with that from MM patients without renal impairment. The BJP with higher k(cat) had higher toxicity to LLC-PK1 cells. BJP could induce apoptosis and necrosis of LLC-PK1 cells when reached a certain concentration and this effect enhanced with increase of BJP concentration. It is concluded that the catalysis of BJP and its toxicity to renal tubular epithelial cells has a positive correlation, and toxic effect of BJP on renal tubular epithelial cells results from inhibiting proliferation and inducing apoptosis and necrosis of the cells, which may be one of renal impairment mechanisms in MM patients.

The treatment of monosegmental fixation and short-segment fixation on thoracolumbar burst fracture a retrospective controlled study / 中华外科杂志

<p><b>OBJECTIVE</b>To investigate the safety and therapeutic effects of monosegment pedicle instrumentation in treating incomplete thoracolumbar burst fracture.</p><p><b>METHODS</b>A retrospective analysis was conducted on 56 inpatients with incomplete thoracolumbar burst fracture (AO classification: A3.1 and A3.2) from April 2005 to January 2010. There were 28 cases were fixed with monosegment pedicle instrumentation (MSPI), 28 cases were fixed with short segment pedicle instrumentation (SSPI). The operative time, blood loss, visual analogue scale (VAS) and vertebral kyphotic angle (VK) before and after surgery were evaluated.</p><p><b>RESULTS</b>In the group of MSPI, the mean operative time was (93 ± 20) min; the intraoperative blood loss was (184 ± 64) ml; the VK angle was 17° ± 10° before operation, 7° ± 7° at one week after operation, and 10° ± 7° at latest follow-up; VAS score was 7.6 ± 1.5 before operation, 2.4 ± 0.8 at one week after operation, and 1.5 ± 0.9 at latest follow-up; no adjacent segment degeneration was found. In the group of SSPI, the operative time was (102 ± 30) min; the intraoperative blood loss was (203 ± 88) ml; the VK angle was 17° ± 9° before operation, 7° ± 7° at one week after operation, and 8° ± 5° at latest follow-up; VAS score was 6.8 ± 1.3 before operation, 3.1 ± 0.5 at one week after operation, and 1.2 ± 0.7 at latest follow-up. One case of adjacent segment degeneration was found in 36 months after operation. There were no significantly statistical differences between two groups in operative time, blood loss, VAS score and VK angle before and after surgery (P > 0.05). The VAS score and VK angle at one week after surgery and latest follow-up all decreased obviously than preoperative ones in both groups (P < 0.05).</p><p><b>CONCLUSIONS</b>MSPI for incomplete thoracolumbar burst fracture is effective and safe. The operative blood loss, the mean operative time, the improvement of VAS score and the VK angle in group MSPI are equal to those in group SSPI.</p>

Granulocyte-macrophage colony-stimulating factor and interleukin 4 induce the malignant transformation of the bone marrow-derived human adult mesenchymal stem cells / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>The purpose of the study was to examine the effects of granulocyte-macrophage colony-stimulating factor (GM-CSF) and interleukin 4 (IL-4) on the bone-marrow-derived human adult mesenchymal stem cells (hMSCs).</p><p><b>METHODS</b>The hMSCs were isolated and cultured with GM-CSF and IL-4 for a period of one month. A single colony of transformed cells was then isolated and their phenotype was characterized by morphology, surface marker expression, and in vivo tumorigenesis.</p><p><b>RESULTS</b>After one month culture, the transformed mesenchymal cells exhibited the morphology and phenotype similar to those of tumor cells, and also caused multiple fast growing lung deposits when it was injected into immunodeficient mice.</p><p><b>CONCLUSION</b>Cytokines-driven malignant transformation of hMSCs may be a useful model for studying signaling pathways initiating malignant transformation of hMSC.</p>

Clinic-pathologic characteristics of autoimmune diseases combined with non-Hodgkin's lymphoma / 中国实验血液学杂志

This study was aimed to investigate the clinical characteristics and treatment of patients with autoimmune disease combined with non-Hodgkin lymphoma (NHL). The clinical characteristics and pathologic patterns of 6 patients with NHL who concurrently suffered from autoimmune diseases were analysed retrospectively from aspects of clinical course, pathologic features, and therapy. Treatment outcomes for autoimmune diseases and NHL were observed. The results showed that 6 patients included 4 females and 2 males, range in age from 28 to 65 years with a median age of 56 years. The autoimmune diseases are Sjogren's syndrome (SS, 2 cases), rheumatoid arthritis (RA, 2 cases), ulcerative colitis (UC, 1 case) and Crohn's disease (CD, 1 case). The NHL diseases located not only in the lymph node (n = 3) but also in extranodal sites (n = 3). Histologically, 3 cases were diffuse large B cell lymphoma (DLBCL), 2 cases were extranodal nasal NK/T lymphoma (ENKL) and 1 case was peripheral T cell lymphoma, not otherwise specified. Based on CD10, Bcl-6 and MUM1 expression patterns, all 3 DLBCL were classified as non-GC subtype. EBER positive tumor cells were detected in 2 case of ENKL. 5 patients achieved a complete remission (83%) and 1 patient was primary drug-resistant after CHOP chemotherapy or involved radiotherapy. Median survival from the time of lymphoma diagnosis was 3 years. 1 patient showed clinical improvement of the SS symptoms, 2 patients (CD and UC) showed stable state of disease and 2 patients with RA and 1 patient with SS needed continuing treatment for their autoimmune diseases after chemotherapy for NHL. It is concluded that the development of NHL is one of the most serious complications in patients with autoimmune diseases. There is an increased frequency of non-GC subtype DLBCL. CHOP combined with or without radiotherapy proves to be effective for autoimmune disease patients with aggressive NHL but ineffective for concurrent autoimmune diseases.

Treatment of primary systemic amyloidosis with the combination of bortezomib and dexamethasone / 中华血液学杂志

<p><b>OBJECTIVE</b>To evaluate the efficacy and feasibility of bortezomib plus dexamethasone (BD) in patients with primary systemic (AL) amyloidosis.</p><p><b>METHODS</b>Eleven AL amyloidosis patients, including four relapsed or progressed after previous therapies and 7 newly diagnosed were treated with BD. Ten patients had two or more organs involved. Precursor protein analysis showed that 1 was κ light chain, 9 λ light chain; 5 patients with positive immunofixation including 1 IgG κ, 3 IgG λ and 1 IgA λ. BD was administered according to standard two-week schedule.</p><p><b>RESULTS</b>Eight patients were evaluable, the median number of treatment cycles was 3 (range 1 - 6). Median follow-up duration was 6 months. At least one affected organ response was observed in six patients and median time to organ response was 2 months. Three patients progressed and two of them died. Toxicities were mainly diarrhea, thrombocytopenia, peripheral neuropathy, fatigue and herpes zoster, and 7 evaluable patients who had toxicities were adjusted dosage and 2 of them interrupted therapy. Epilepsia, paralytic ileus, acute cardiac dysfunction, and postural hypotention were occurred in 3 inevaluble patients.</p><p><b>CONCLUSION</b>Bortezomib plus dexamethasone is effective in AL amyloidosis. Adverse events are common, and in some patients are severe.</p>