Multiple myeloma (MM) is a malignant hematological tumor characterized by the proliferation of monoclonal plasma cells, often accompanied by systemic complications such as bone destruction, renal dysfunction, and hypercoagulability. Plasma D-dimer, as a fibrinolytic marker, may be associated with disease activity. However, there are few reports on the application of plasma D-dimer in evaluating treatment efficacy in MM. This multicenter retrospective study aimed to evaluate the utility of plasma D-dimer levels in predicting treatment response in patients with MM. 160 patients with newly diagnosed MM were enrolled. Blood samples were collected from these patients during their first hospital visit and again after eight cycles of chemotherapy to measure D-dimer levels. This study explores the relationship between plasma D-dimer levels before and after treatment and patient clinical characteristics. Patients showed no statistically different plasma D-dimer levels at initial consultation across treatment-response groups: CR group (0.96 µg/mL [0.67–1.9]), PR group (0.88 µg/mL [0.56–1.39]), SD group (0.68 µg/mL [0.56–0.89]), and PD group (0.91 µg/mL [0.52–1.55]). After chemotherapy, both the absolute change (Dd) and percentage change (Ddp) in D-dimer levels showed significant differences among groups (p < 0.05). The CR and PR groups exhibited significantly greater reductions in D-dimer (Dd: -0.42 and − 0.40 µg/mL; Ddp: -45.2% and − 39.1%) compared to the PD group (Dd: 0.16 µg/mL; Ddp: 18.7%). Patients with overall response (OR) (CR and PR groups) had significantly greater decreases in both Dd and Ddp compared to no-response patients (PD and SD groups) (p < 0.001). Our results suggested that changes in plasma D-dimer levels before and after treatment may provide valuable insights for assessing chemotherapy efficacy in MM.

Hemophagocytic lymphohistiocytosis (HLH) is a rare and fatal disease with a low survival rate. It is important to identify the patients at risk of poor prognosis among HLH patients. In this multicenter and retrospective study, we reviewed 90 newly diagnosed HLH patients treated at the Second Affiliated Hospital of Nanjing Medical University and the First Affiliated Hospital of Anhui Medical University from April 2014 to February 2025. Four pre-treatment clinical characteristics of HLH patients were confirmed to be the independent risk factors: age (hazard ratio (HR) 1.041, 95% confidence interval (CI)1.023-1.059, p < 0.001); splenomegaly (HR 2.112, 95% CI 1.178-3.787, p = 0.012); platelet (PLT) count (HR 0.992, 95% CI 0.984-0.999, p = 0.035); aspartate aminotransferase (AST) (HR 1.002, 95% CI 1.001-1.003, p < 0.001). Subsequently, the patients' prognostic risk scores were calculated based on these four risk factors to stratify patients to high-risk and low-risk groups. Besides, these four factors were included in the final clinical prediction model. Receiver operating characteristic (ROC) curves revealed that this model had a good discrimination with optimism-corrected area under the curve (AUC) values of 0.78 (95% CI: 0.68-0.88) for 30-day mortality and 0.82 (95% CI: 0.74-0.92) for 90-day mortality. The calibration curves aligned with the model predictions and actual observations. The decision curve analysis also confirmed our model's robust predictive power. These results point out that age, splenomegaly, PLT, and AST levels are independent indicators of overall survival in patients with HLH and that the proposed model has a good prognostic value.

OBJECTIVE:To study the role of cytokines and lymphocyte subsets in the diagnosis, prognosis and efficacy evaluation of DLBCL patients, and the effects of Tislelizumab on immune function and cytokines in DLBCL patients.METHODS:Twenty-three patients with newly diagnosed DLBCL were selected as DLBCL group and 34 patients with megaloblastic anemia as the control group. The levels of peripheral blood cytokines IL-2, IL-4, IL-6, IL-10, TNF- α and IFN-γ by ELISA method. The levels of peripheral blood CD3+, CD4+, CD8+ T lymphocytes, B lymphocytes and NK cells， the ratio of CD4+/CD8+ were detected by flow cytometry. The levels of cytokins and lymphocyto subsets in DLBCL patients with different clinical data and different therapeutic effects were compared.RESULTS:The levels of cytokines IL-2, IL-6 and IL-10 in DLBCL group were significantly higher than those in control group, but there was no significant correlation between cytokine levels and age and gender. The higher IPI score, higher Ann Arbor stage, B symptoms, higher β 2-MG, LDH and CRP levels, IL-6 and IL-10 levels were significantly higher, and IL-4 was also significantly higher in patients with high LDH levels. Compared with the ineffective group, the levels of IL-6 and IL-10 were significantly lower and the level of CD4+ T cells and the ratio of CD4+/CD8+ was significantly higher in the effective group before therapy. The levels of IL-6, IL-10 and B lymphocytes in the effective group decreased significantly after therapy compared to those before therapy. After 4 cycles of therapy, the level of IL-2 and the ratio of CD4+/CD8+ in the Tislelizumab group were significantly higher than those in the non-Tislelizumab group, and the level of CD8+ T cells was significantly lower than that in the non-Tislelizumab group(P<0.05). The level of B lymphocytes in both the Tislelizumab group and the non-Tislelizumab group after therapy was significantly lower than that before therapy.CONCLUSION:The expression of cytokines and lymphocyte subsets in peripheral blood of patients with DLBCL is abnormal, which is related to the severity, prognosis and therapeutic effect of the disease. Tislelizumab can improve the immune function of patients with DLBCL by affecting cytokines and lymphocyte subsets and strengthen anti-tumor immunity.

BACKGROUND The Coexistence of myeloid and lymphoid malignancies is rare. Myeloid leukemia occurs more frequently as a secondary event in patients receiving chemotherapy agents for lymphoid malignancies. Synchronous diagnoses of diffuse large B-cell lymphoma (DLBCL), acute myeloid leukemia (AML), and untreated lymphoplasmacytic lymphoma/Waldenström macroglobulinemia (LPL/WM) in the same patient have not been reported. Here we report one such case. CASE SUMMARY An 89-year-old man had a chest wall mass histopathologically diagnosed as DLBCL. The bone marrow and peripheral blood contained two groups of cells. One group of cells fulfilled the criteria of AML, and the other revealed the features of small B lymphocytic proliferative disorder, which we considered LPL/WM. Multiple chromosomal or genetic changes were detected in bone marrow mononuclear cells, including ATM deletion, CCND1 amplification, mutations of MYD88 (L265P) and TP53, WT1 overexpression, and fusion gene of BIRC2-ARAP1, as well as complex chromosomal abnormalities. The patient refused chemotherapy because of old age and died of pneumonia 1 mo after the final diagnosis. CONCLUSION The coexistence of DLBCL, AML, and untreated LPL/WM in the same patient is extremely rare, which probably results from multiple steps of genetic abnormalities. Asymptomatic LPL/WM might have occurred first, then myelodysplastic syndrome-related AML developed, and finally aggressive DLBCL arose. Therefore, medical staff should pay attention to this rare phenomenon to avoid misdiagnoses.

目的 采用Meta分析考察西达本胺联合标准化疗治疗外周T细胞淋巴瘤的临床疗效.方法 搜索了中国知网、万方、维普、Web of Science、PubMed、Cochrane Library、Embase等数据库,检索文献发表时间为从数据库建库到 2023年 6月的西达本胺联合标准化疗治疗外周T细胞淋巴瘤的试验,应用Cochrane中心提供的Revman 5.4 软件对数据进行统计学处理分析.结果 筛选符合标准的文献为 10 篇,共 494 例患者.西达本胺在外周T细胞淋巴瘤治疗中可显著改善完全缓解率、客观缓解率、1 年生存率、1 年无进展生存率,与标准化疗间的差异具有统计学意义.结论 对于外周T细胞淋巴瘤患者,西达本胺联合化疗在完全缓解率、客观缓解率、1 年生存率、1 年无进展生存率均优于单纯化疗.

Objective:To improve the understanding of indolent mantle cell lymphoma (MCL).Methods:The data of a patient with indolent leukemic MCL in the Second Affiliated Hospital of Nanjing Medical University in May 2013 were collected. The cell morphology was analyzed by using cell smear, the flow cytometry was used to make immunophenotype analysis, the karyotype analysis was performed by usig cytogenetic technique, and polymerase chain reaction (PCR) was used to make the immunoglobulin gene analysis. At the same time, lymph node pathology and immunohistochemistry were also analyzed. The related articles published were reviewed to sum up the characteristics and the treatment of indolent MCL.Results:The male patient aged 60 years was obviously asymptomatic accompanied with slow disease progression, leukemic manifestation and without lymphadenopathy. He received pathological biopsy because of located lymphadenopathy in 2008. Small cell morphology, Kappa light chain immunophenotype, t(11;14) translocation showed after the cytogenetic examination, clonal immune globulin gene rearrangement and low Ki-67 positive index were identified. In situ MCL was diagnosed by retrospective pathology.Conclusions:Indolent MCL is extremely rare. It is typically asymptomatic with none or minimal nodal involvement, indolent disease course, leukemic phase with mild lymphocytosis, Kappa light chain expression, simple karyotype, classical or small cell morphology of tumor cells and the positive index of Ki-67 <10%. In situ MCL can be seen in pathology examination. IgVH gene mutation positive and SOX11 negative expression are notable in indolent MCL. International prognostic index of MCL is probably not appropriate in the prognostic analysis of leukemic indolent MCL. It is emphasized that initial observation and having therapies only after the disease progression can be suited for indolent MCL.

Rationale: POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome is a rare and complicated disease related to multiple organs and systems. Here, we report a case of systemic mastocytosis (SM) that was misdiagnosed as a POEMS syndrome. Patient concerns: A 42-year-old man presented with skin changes, diarrhea, and limb numbness. Diagnoses: Positron emission tomography/computed tomography revealed extravascular volume overload, organomegaly, lymphadenopathy, and bone lesions with mixed lesions of osteosclerosis and osteolysis. Therefore, POEMS syndrome was suspected. Further histopathological and immunohistochemical examination of the bone marrow, lymph nodes, and gastric mucosa suggested a diagnosis of mastocytosis. The c-Kit D816V mutation confirmed the diagnosis of SM. Interventions: The patient received the treatment of pegylated interferon-alpha weekly and glucocorticoid daily. Outcomes: The symptoms relieved significantly. Lessons: There are many similar features between POEMS syndrome and SM, probably leading to misdiagnosis. This study analyzed the different points between them which can provide help for differentiation.

Objectives Haploidentical hematopoietic stem cell transplantation (Haplo-SCT) and matched unrelated donor transplantation (MUD-SCT) are two important options when a matched sibling donor (MSD) is unavailable. Several studies comparing Haplo-SCT and MUD-SCT have reported inconsistent clinical outcomes. Therefore, it is necessary to synthesize the existing evidence regarding outcomes of stem cell transplantations comparing Haplo-SCT with MUD-SCT. Methods We searched for titles of articles in MEDLINE (PubMed), Cochrane library, EMBASE database that compared transplantation with Haplo-SCT versus MUD-SCT. To compare clinical outcomes between Haplo-SCT and MUD-SCT, we performed a meta-analysis of 17 studies and reported the pooled odd ratios (OR) of 6 endpoints including overall survival (OS), progression free survival (PFS), non-relapse mortality (NRM), relapse rate (RR), acute graft-versus-host disease (aGVHD) and chronic graft- versus-host disease (cGVHD). Results We found that Haplo-SCT was associated with a comparable OS (pooled OR of 0.99, 95% Confidence Interval (CI) 0.86-1.14), PFS (OR 1.00, 95% CI 0.88-1.15), NRM (OR 0.83, 95% CI 0.65-1.04) and RR (OR 1.08, 95% CI 0.95-1.22) compared to MUD-SCT. We also found a significantly decreased risk of aGVHD (OR 0.74, 95% CI 0.62-0.88) and cGVHD (OR 0.50, 95% CI 0.38-0.66) in Haplo-SCT group. Conclusion Results of this meta-analysis demonstrates that Haplo-SCT achieves comparable clinical outcomes compared to MUD-SCT in terms of OS, PFS, TRM and RR, but is better than MUD-SCT in terms of decreased aGVHD and cGVHD risk. Haplo-SCT is a valid option for patient needing urgent transplantation.

Objective To investigate the expression of S100A6 in AML and its influence no curative effect.Methods Collecting 42 AML patients and 12 normal persons,the expression of S100A6 mRNA in these AML patients was determined by real time quantitative PCR (RQ-PCR).The expressions in AML patients and normal persons,different types AML patients,the patients before and after the therapy were compared.The expression differences before and after the therapy between the effective treatment group and the ineffective treatment group of AML-M3,M4,M5 patients were compared.The expression differences before the therapy between the recurrence group within two years and the no recurrence group within two years of AML-M3,M4,M5 patients were compared.The expression differences of CR1,CR2 between the high expression group and the low expression group of AML-M3,M4,M5 patients were compared.The expression differences of OS in two years between the high expression group and the low expression group of AML-M3,M4,M5 patients were compared.The expression differences of relapse between the high expression group and the low expression group of AML-M3,M4,M5 patients were compared.All the results were analyzed by statistic.Results Compared with normal per sons,the expression lever of S100A6 in the AML-M3,M4,M5 patients was higher(P ＜ 0.05),the expression lever in the AML-M1,M2,M6,M7 patients was not different(P ＞ 0.05).The expression of S100A6 in the AML-M3,M4,M5 patients who have effective treatment has difference between before and after therapy (P ＜ 0.05),the expression of S100A6 in these patients who have no effective treatment has no difference between before and after therapy(P ＞ 0.05).The expression of S100A6 in the AML-M3,M4,M5 patients who relapsed in two years was higher than that in the patients who have not relapsed in two years(P ＜0.05).The rate of CR1 and CR2 in the S100A6 high expression group is no statistical difference with the rate of the low expression group of AML-M3,M4,M5 patients(P ＞0.05).The rate of OS in two years in the S100A6 high expression group is no statistical difference with the low expression group of AML-M3,M4,M5 patients (P ＞ 0.05).The rate of relapse in the S100A6 high expression group is higher than that of low expression group of AML-M3,M4,M5 patients (P ＜ 0.05).Conclusion The expression of S100A6 in AML patients is related to the patients' styles,curative effect and prognosis.It might be the diagnosis molecular markers of AML.

Objective:To investigate the expressions of S100A6,annexin A2 (AnxA2) and c-myc in patients with multiple myeloma (MM) and their clinical significance.Methods:The expressions of S100A6,AnxA2 and c-myc mRNAs in bone marrow mononuclear cells from 28 cases of MM before and after chemotherapy and 20 controls (whose peripheral blood white cell count and the platelet count were a little lower than the normal values,but the result of bone marrow aspiration was normal) were detected by real-time fluorescent quantitative PCR.The relationships among the expressions of S100A6,AnxA2 and c-myc mRNAs were analyzed.The expressions of S100A6,AnxA2 and c-myc mRNAs and proteins in MM U266 cells after transfection with S100A6 siRNA were detected by real-time fluorescent quantitative PCR and Western blotting,respectively.Results:The expression levels of S100A6,AnxA2 and c-myc mRNAs in bone marrow mononuclear cells from patients with MM were higher than those from the controls (all P ＜ 0.05).The expression levels of S100A6,AnxA2 and c-myc mRNAs in bone marrow mononuclear cells from patients with MM before chemotherapy were higher than those after chemotherapy (all P ＜ 0.05).The expression levels of S100A6,AnxA2 and c-myc mRNAs in bone marrow mononuclear cells from MM patients with extramedullary metastasis were higher than those from MM patients not having extramedullary metastasis (all P ＜ 0.05).The expression of S100A6 mRNA was positively correlated with the expressions of AnxA2 and c-myc mRNAs (r =0.585,P=0.001;r =0.540,P =0.004).The expression levels of S100A6,AnxA2 and c-myc mRNAs and proteins in MM U266 cells after transfection with S100A6 siRNA were down-regulated (all P ＜ 0.05).Conclusion:The expression level of S100A6 in patients with MM is higher,and it is positively associated with AnxA2 and c-myc.S100A6 may be involved in the development,progression and extramedullary metastasis of MM.

Objective: To investigate the expression levels of S100A6, Notch1 in multiple myeloma (MM) patients and its clinical significance. Mathods: The expression levels of S100A6, Notch1 in 28 MM cases and 20 healthy controls were determined by real time quantitative PCR (RQ-PCR) , and their relationships with clinical features and outcomes were analyzed. Immunohistochemical was used to analysis the levels of S100A6 and Notch1 in bone marrow biopsy samples and intramedullary metastases soft tissues. RQ-PCR and Western blot were used to test the changes of Notch1 mRNA and Notch1 protein in U266 MM cells after S100A6 silenced by siRNA. Results: ①The expression levels of S100A6, Notch1 in primary MM patients was 2.19±1.25, 2.98±0.64, significantly higher than those in controls (0.71±0.20, 0.58±0.39, P<0.05) and patients in platform status (0.85±0.26, 0.72±0.40, P<0.05) . 8 cases with intramedullary metastasis had significantly higher levels of S100A6 (3.36±1.23) and Notch1 (5.71±3.96) , as compared to those without extra medullary metastases. ②S100A6 expression was positive correlation with Notch1 (r=0.505, P=0.007) . ③S100A6 and Notch1 proteins were positive in plasma cells of bone marrow biopsy samples and intramedullary metastases soft tissues. ④The Notch1 mRNA and Notch1 expression decreased significantly after 48 hours treatment by S100A6 siRNA in U266 cells. Conclusion: S100A6 and Notch1 were closely associated with MM progress and intramedullary metastasis. They have significant correlation and might be as two prognostic molecular markers in MM.

Objective To evaluate the curative effect of VAD,improved M2 and MP program combined with thalidomide in treatment of multiple myeloma (MM).MethodsAccording to the patient’s condition,economic situation and individual will,39 cases of patents with MM were divided into three groups.26 patients were treated with VAD,which included 20 cases with newly diagnosed MM,21 patients were treated with improved M2 and MP program (Chemotherapy regimens including melphalan),which included 13 cases with newly diagnosed newly diagnosed MM.14 patients were treated with improved M2,which included 4 cases with newly diagnosed MM and 10 cases with recurrently diagnosed MM.15 patients were treated with MP,which included 10 cases with newly diagnosed MM and 5 cases with recurrently diagnosed MM.8 patients were treated with MP and M2 program repetition.8 patients were treated with successively VAD and improved M2 or MP program repetition.Results The total effective rate of VAD group and Melphalan (M) group (OR)(≥PR) respectively were 80% and 76.9% (P>0.05).The total effective rate of patients with newly diagnosed MM of VAD group and Melphalan (M) group (OR)(≥VGPR) respectively were 60.0% and 46.2% (P>0.05).The total effective rate of patients with newly and recurrently diagnosed MM of VAD group and Melphalan (M) group (OR)(≥PR) respectively were 76.9% (26 cases in VAD group) and 57.1% (21 cases in M group) (P>0.05).The total effective rate of patients with newly diagnosed MM of VAD group and Melphalan (M) group (OR)(≥VGPR) respectively were 53.8% and 42.9% (P>0.05).There are 4 cases of VGCR or more curative effect among the 10 cases of newly diagnosed MP.There are 3cases of VGCR or more curative effect among the 4 cases of newly diagnosed improved M2.The effect of 3 cases of patients with newly diagnosed VAD was poor.After switching to M2 program, it was up to VGPR efficacy.Overall survival (OS) time of VAD group and M group respectively were 39 months and 25 months for patients with new diagnosis (P>0.05),39 months and 50 months for patients with new&nbsp;and recurrent diagnosis (P>0.05).Progression free survival (PFS) time of VAD group and M group respectively were 31 months and 18 months for patients with new diagnosis (P>0.05),29 months and 31 months for patients with new and recurrent diagnosis (P>0.05).Conclusion VAD,improved M2 and MP program combined with thalidomide chemotherapy is an economical and effective solution to individually doctor elderly patients with MM.

OBJECTIVE To investigate the expression level of S100A6 mRNA in MM and to its clinical significance, and to evaluate its significance. METHODS The expression level of S100A6 mRNA in MM patients was determined by real time quantitative PCR(RQ-PCR), and its relationship with the clinical features and outcomes of patients was analyzed by statistic method. RESULTS S100A6 mRNA was detected in 20 MM patients. Compared with normal persons, the S100A6 mRNA expression in MM patients was higher. In different groups, the S100A6 mRNA expression in MM patients of 3 stages was higer than that in patients of 1 and 2 stages. MM patients with higher S100A6 mRNA expression had poor prognosis and higer extramedullary metastasis rate. CONCLUSION The high expression of S100A6 mRNA is associated with poor prognosis and may be a prognostic molecular marker of MM.

Objective:To investigate the clinical characteristics of adult patients with hemophagocytic lymphohisto-cytosis(HLH).Methods:The clinical and experimental data of 21 adult patients with HLH from Department of He-matology,The Second Affiliated Hospital of Nanjing Medical University,were collected retrospectively from January 2005 to January 2015.Results:All patients presented high fever,hepatosplenomegaly,hematostatic abnormality,and cytopenia at first visit.Seven cases(33.3%)were diagnosed as non -Hodgkins'lymphoma.10(47.6%)were diag-nosed as infection,3 cases as fever with thrombocytopenia syndrome among them.1(4.8%)was malignancy -associ-ated HLH,3(14.3%)without pathogenesis.Seventeen cases were dead during follow -up and two was still in follow-up(the longest was 5 years and 7 months till now).Two cases lost follow -up.The median survival time was 183 days(range from 4 days ~5.7 years).Conclusion:HLH is an uncommon fatal disease and farely occurred in adults. The clinical presentation is complex,usually with multi -organ dysfunction,aggressive course and poor prognosis.The pathogenesis and treatment of HLH should be further studied.

Multiple myeloma (MM) with intraspinal invasion is very rare in clinic, which is difficult to treat. No stan-dard treatments are recommended at home and abroad, and only case report can be found, which is poor prognostic. Chemotherapy, operation and radiotherapy are the basic treatments. To further deepen the understanding of the diagno-sis and treatment of MM complicated by spinal infiltration, clinical practice, curative effect and course of disease pro-gression of two cases of MM complicated with spinal infiltration are summarized and analyzed, and pertinent literature is reviewed. Early detection, diagnosis and individualized treatment are thought to be critical in improving prognosis. Once paraplegia happened in patients, the therapeutic efficacy and prognosis would be very poor, and the disease pro-gression would be rapid.

Objective To investigate the effect of silenced c-FLIP mRNA level by small interfering RNA (siRNA) on adriamycin-resistance of K562/ADR cells. Methods c-FLIP siRNA and negative siRNA were transfect-ed into K562 and K562/ADR cell lines respectively, and mRNA expression of c-FLIP and multi-drug resistance gene 1 (MDR1) were detected by quantitative PCR. Cell proliferation rate was detected by MTT assay, and cell apoptosis rate was assayed by Annexin V/7-ADD double-staining method. Results Compared with negative siRNA transfection group, siRNA transfection significantly decreased c-FLIP mRNA expression in K562 cells (P<0.05) and slightly inhib-ited the proliferation of K562 cell (not enough to induce K562 cell apoptosis). The 48 h proliferation rates of K562 cells were (69.14 ± 1.82)% and (60.69 ± 2.23)% in negative siRNA transfection group and siRNA transfection group, and the apoptosis rate were (1.7±0.3)%and (1.8±0.2)%, respectively. c-FLIP siRNA transfection significantly inhibit-ed K562/ADR proliferation and induced cell apoptosis (P<0.05). The 48 h proliferation rate of K562/ADR cells were (-6.07 ± 0.71)% and (-37.45 ± 3.53)% in negative siRNA transfection group and siRNA transfection group, and the apoptosis rate were (5.2 ± 0.4)% and (9.2 ± 0.4)%, respectively. In addition, MDR1 mRNA expression of K562/ADR was significantly decreased (P<0.05) upon c-FLIP siRNA transfection (P<0.05). Conclusion c-FLIP siRNA transfec-tion significantly decreases mRNA expression of MDR1 and inhibites the adriamycin-resistance of K562/ADR cells.

目的 探讨T-大颗粒淋巴细胞白血病(T-LGLL)的临床和实验室检特征,以提高对T-LGLL的的认识.方法 回顾性分析2009年5月至2013年10月南京医科大学第二附属医院收治的5例T-LGLL患者的临床资料.结果 5例均为老年女性患者,3例免疫表型为CD3+ CD4-CD8+ TCRαβ+,伴有纯红细胞再生障碍(PRCA);2例免疫表型为CD3+CD4+ TCRαβ+.结论 T-LGLL为少见疾病,临床进展慢,预后较好;以CD3+CD4-CD8+TCRαβ+亚型为主,常伴PRCA,可采用免疫抑制剂治疗;CD3+CD4+ TCRαβ+亚型更为少见且多无明显临床表现.

目的:探讨华氏巨球蛋白血症(WM)的临床特征,提高利妥昔单抗治疗WM的认识.方法:报告1例采用预防性血浆置换后给予利妥昔单抗序贯治疗血清IgM＞50g/L的WM并结合文献进行复习.结果:患者为老年男性.无淋巴结和肝脾肿大,主要表现为血清IgM明显增高(IgM 63.1g/L)、伴有高黏滞血症表现、贫血,骨髓示淋巴细胞弥漫性浸润,免疫分型符合WM.经预防性血浆置换后给予利妥昔单抗为基础的方案治疗,出现轻微IgM反跳,未发生高黏滞血症加重和其他并发症.接受治疗5月后获得主要反应(IgM下降＞50%)、23月后接近非常好的部分缓解水平(IgM下降≥90%).结论:WM属于慢性B淋巴细胞增殖性肿瘤,临床少见,多发生于老年,有症状者需接受治疗.接受以利妥昔单抗为基础的治疗后,可发生IgM反跳,严重时可使病情加重,治疗前IgM＞50g/L者尤为明显,IgM反跳并不意味利妥昔单抗治疗失败,预防性血浆置换对降低反跳发生及IgM增高的程度有积极意义.

This study was aimed to investigate the normalization of serum free light chain ratio (sPLCR) after treatment of multiple myeloma (MM) and its influence on the prognosis of MM patients. The clinical data of 42 patients with MM were analyzed retrospectively from January 2009 to November 2013 in out department. According to sPLCR consecutive normalization for more 4 weeks or not after treatment, the patients were classified in patients with mormalized sPLCR and patients with abnormalized sPLCR, then the influence of traditional prognostic factors of MM on sPLCR and effect of sPLCR on overall survival (OS) time of MM patients were analyzed. The results showed that the influence of age, ISS stage displayed statistical difference between sFLCR normalization group and abnormalization group, the age ≥ 65 years and ISS stage III negatively impacted on sFLCR normalization (P < 0.05). The response rates of patients with normalized sFLCR were as follows: CR - 60%, VGPR - 38.89%; PR - 28.57%; 17 patients (40.48%) with sFLCR normalization showed superior OS, as compared with patients with sPLCR abnormalization (P < 0.01). It is concluded that the sFLCR normalization is the independent prognostic factor for MM, suggesting that the MM patients with sPLCR normalization after treatment have superior prognosis.

Objective To analyze the diagnostic process of a rare case diagnosed of myeloid/nature killer cell precursor acute leukemia( M/NKPAL) and to improve the recognition of M/NKPAL. Methods Cell morphology was analyzed by marrow smear and re-lated cell chemical staining. Immunophenotyping of blast cells was performed by flow cytometry. Cytogenetics technique was used for karyotype analysis. PCR was applied for detection of T-cell recepter gene rearrangement or fusion gene associated with leukemia. The related articles were reviewed. Results With regard to morphology, most of leukemic cells demonstrated that 90?4% blasts in the bone marrow were generally L2-shaped with negative reactivity of myeloperoxidase staining and Auer?s rods were occasionally found. Expres-sion of CD34, HLA-DR,CD33,CD7,CD56,CD38 and cyCD3( dim) ,but no other markers including cyMPO,CD3 and CD4 were ob-served. Cytogenetics examination revealed abnormal karyotype. Clonal T-cell recepter gene rearrangement and fusion gene were not de-tected. Conclusion M/NKPAL is considered extremely rare and diagnosis is difficult depending merely on the morphology. It is im-portant to be distinguished from T-cell acute lymphoblastic leukemia with expression of myeloid-antigen, acute myeloid leukemia with minimal differentiation, mixed-phenotype acute leukemia with T/myeloid lineage and blastic NK cell leukemia/lymphoma.