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A 65-year-old male patient was admitted for recurrent lymph node enlargement for 5 years and elevated creatinine for 6 months. This patient was diagnosed with angioimmunoblastic T-cell lymphoma 5 years ago and underwent multiple lines of anti-tumor therapy, including cytotoxic chemotherapy; epigenetic modifying drugs such as chidamide and azacitidine; the immunomodulator lenalidomide; and targeted therapy such as rituximab, a CD20-targeting antibody, and brentuximab vedotin, which targets CD30. Although the tumor was considered stable, multiple virus activation (including BK virus, JC virus, and cytomegalovirus) accompanied by the corresponding organ damage (polyomavirus nephropathy, cytomegalovirus retinitis, and progressive multifocal leukoencephalopathy) occurred during anti-tumor treatment. Anti-tumor therapy was suspended and ganciclovir was used. The serum viral load decreased and organ functions were stabilized. The purpose of this report was to raise clinicians′ awareness of opportunistic virus reactivation during anti-tumor treatment.
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Objective:To analyze the efficacy of second-line regimens and prognostic factors in patients with first-relapsed multiple myeloma (MM) treated with bortezomib, cyclophosphamide, and dexamethasone (BCD).Methods:A retrospective cohort study. Clinical data were collected in first-relapsed MM patients after BCD treatment from three tertiary hospitals in north China from July 2009 to October 2022. Patients were classified according to the second-line regimen into the immunotherapy group, single novel agent group [either proteasome inhibitor (PI) or immunomodulatory drug (IMiD)], combination treatment group (both PI+IMiD), and traditional treatment group. Responses to second-line regimens and survival data were analyzed. The Kaplan-Meier method was used for survival analysis and the Cox proportional risk model was used for univariate and multivariate analyses.Results:A total of 217 patients were enrolled including 8.8% (19/217) in the immunotherapy group, 48.4% (105/217) in the PI/IMiD group, 29.9% (65/217) in the PI+IMiD group, and 12.9% (28/217) in the traditional treatment group. The median age was 62 years (range 31-83 years) and 56.2% (122/217) were males. The overall response rates (ORRs) in the four groups were 94.7% (18/19) vs. 56.2% (59/105) vs. 73.8% (48/65) vs. 32.1% (9/28) ( χ2=24.55; P<0.001), respectively. The progression-free survival (PFS) of the second-line regimens (2ndPFS) was 17.7 vs. 9.0 vs. 9.2 vs. 4.6 months ( χ2=22.74; P<0.001), respectively, among which patients in the PI/IMiD and PI+IMiD groups had comparable 2ndPFS ( χ2=1.76; P=0.923). Patients with high-risk cytogenetic abnormalities (HRCAs) achieved the longest 2ndPFS of 22.0 months in the immunotherapy group ( χ2=15.03; P=0.002). Multivariate analysis suggested that immunotherapy ( HR=0.11, 95% CI 0.05-0.27), achievement of efficacy of partial response or better ( HR=0.47, 95% CI 0.34-0.66), and non-aggressive relapse ( HR=0.25, 95% CI 0.17-0.37) were independent prognostic factors of 2ndPFS. Conclusion:In this real-world study, immunotherapy was associated with a more favorable efficacy and PFS for first-relapsed MM patients after BCD treatment, with similar outcomes in patients with HRCAs.
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A 43-year-old female patient was admitted with recurrent thrombosis for more than 2 years and thrombocytopenia for more than 1 year. Both arterial and venous thromboses developed especially at rare sites even during anticoagulation therapy such as cerebral venous sinus thrombosis. Antinuclear antibody, anti-ENA antibody and antiphospholipid antibody were all negative. Platelet count elevated to normal after high dose glucocorticoid and intravenous immunoglobulin (IVIG). Immune thrombocytopenia was suspected. When 4 grade thrombocytopenia recurred, intravenous heparin, rituximab 600 mg, IVIG and eltrombopag were administrated. After 3 weeks, thrombocytopenia did not improve, and new thrombosis developed instead. Screening of thrombophilia related genes revealed PROS1 gene heterozygous mutation and MTHFR TT genotype. Low amount of serum IgG κ monoclonal protein was detected. Heparin-induced thrombocytopenia was differentiated and excluded. Finally, serum negative antiphospholipid syndrome was considered the most likely diagnosis. Dexamethasone 20 mg/day × 4 days combined with sirolimus 2 mg/day was prescribed. The patient was discharged with low molecular weight heparin. At one month, her headache was greatly relieved. The platelet count raised to 20-30×10 9/L, and no new thrombosis or bleeding was reported.
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Objective To evaluate the safety and efficacy of lenalidomide plus rituximab in treatment of the patients with relapsed/refractory B-cell non-Hodgkin lymphoma (B-NHL). Methods The clinical data of the patients with relapsed/refractory B-NHL after the varieties of treatment methods in Peking Union Medical College Hospital between January 2015 and December 2017 were retrospectively analyzed. All the patients were treated with R2 regimen: oral lenalidomide (25 mg/d for day 1-day 21) and rituximab (375 mg/m2 of intravenous infusion on day 1, 28-day of each cycle); the efficacy was evaluated after three cycles. After this induction phase, the patients achieving complete response (CR), partial response (PR), or stable disease (SD) were given R2 regimen until the end of 8 cycles. The major end point was overall response rate (ORR) defined as CR + PR. Secondary end point included 1-year progression free survival (PFS), 1-year overall survival (OS) and grade 3-4 adverse events. T cell and B cell subsets of 7 patients at baseline were measured, and T cell and B cell subsets of 13 patients with good efficacy were dynamically observed. Results A total of 49 patients who received 1-4 chemotherapy regimens were included. The ORR after the R2 treatment for 3 courses was 65% (32/49). Thirty-six patients (9 cases of CR, 22 cases of PR, 5 cases of SD) were enrolled in R2 maintenance treatment. The median follow-up time was 13 months, 1-year PFS rate was 61% and 1-year OS rate was 84% . The most common adverse event was bone marrow suppression, including grade 3-4 neutropenia (27% ), grade 3-4 thrombocytopenia (6% ) and grade 4 anemia (4% ), most of which could be controlled by prolonging interval cycles or reduced lenalidomide dosage. The decreased number of CD19+B cell after treatment could be seen in 13 patients who obtained good efficacy under the dynamic observation. Conclusion Lenalidomide plus rituximab is well tolerated and highly active in the treatment of relapsed/refractory B-NHL.
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Objective@#To evaluate the feasibility and potential value of comprehensive geriatric assessment (CGA) in elderly (≥60 years) patients with newly diagnosed acute myeloid leukemia (AML) in China.@*Methods@#The CGA results of 83 newly diagnosed AML (non-APL) patients from 16 hospitals in Beijing and Tianjin between March 2016 and December 2017 were prospectively collected and analyzed. The clinical data, treatment and follow-up information were also collected.@*Results@#Of 83 newly diagnosed elderly AML patients, 81 patients (97.6%) completed all designated CGA assessment. The median number of impaired scales of the CGA assessment in the studied population was 2(0-6). Sixteen patients (19.3%) showed no impairments according to the geriatric assessment scales implem ented by this study. The distributions of impaired scales were as follows: impairment in ADL, 55.4%; IADL impairment, 42.2%; MNA-SF impairment, 48.2%; cognitive impairment, 15.7%; GDS impairment, 31.7%; HCT-CI impairment, 19.5%, respectively. In patients with "good" ECOG (n=46), the proportion of impairment for each CGA scale ranged from 6.5% to 37.0% and 32 patients (68.9%) had at least one impaired CGA scale. Survival analysis showed that the number of impaired scales of the CGA was significantly correlated with median overall survival (P=0.050).@*Conclusions@#CGA was a tool with feasibility for the comprehensive evaluation in elderly AML patients in China. Combined with age and ECOG, CGA may be more comprehensive in assessing patients’ physical condition.
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Immune checkpoint inhibitors are able to reactivate the immune system therefore enhance the anti-tumor effects. However, over-activated T cells may induce immune related adverse events (irAEs). Hematological irAEs are rarely reported, which mainly represent as mono-lineage cytopenia or pancytopenia, including autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP), neutropenia and aplastic anemia, sometimes even lethal, such as hemophagocytic lymphohistiocytosis. The clinical manifestations of hematological irAEs will be summarized and recommendations of diagnosis and treatment are proposed.
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Objective@#To Evaluate the efficacy and safety of posaconazole as primary prevention of invasive fungal disease (IFD) in patients with severe aplastic anemia (SAA) treated with anti-thymus/lymphocyte immunoglobulin (ATG/ALG) combined with cyclosporine intensive immunosuppressive therapy (IST).@*Methods@#A retrospective analysis of clinical data of 58 SAA patients who received IST of anti-thymocyte immunoglobulin combining cyclosporine and antifungal prophylaxis during April 2013 to May 2017 in Peking Union Medical College Hospital was performed. The patients were divided into posaconazole prophylaxis group and the control group (itraconazole or fluconazole). The disease characteristics, IFD prevention effect and adverse drug reaction, curative effect and prognosis of the two groups were compared.@*Results@#Posaconazole was used to prevent fungal infection in 20 patients. The other 38 patients were used as the control group. Retrospective analysis showed comparable characteristics (gender, age, disease severity, etiology, interval between the onset of disease to treatment, ATG/ALG type) of both groups. The incidence of IFD were 0 and 15.8% in posaconazole prophylaxis group and the control group, respectively (P=0.084). In the control group, there were 6 cases diagnosed as IFD. Of them, 2 were confirmed, 2 suspected and 2 not identified. Five of the 6 cases were pulmonary infection, 1 bloodstream infections. Of the 6 IFD cases, 5 were very severe aplastic anemia (VSAA). There was no obvious adverse reaction in posaconazole prophylaxis group.@*Conclusion@#Posaconazole is safe and effective for primary prevention of fungal infection of SAA patients receiving IST, especially for the VSAA.
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Objective@#To analyze the clinical effects and safety of combination treatment of thalidomide, stanozolol, and prednisone (TSP regimen) in patients with myelofibrosis (MF) who experienced hematological adverse reactions during ruxolitinib treatment.@*Methods@#Ten MF patients with anemia or thrombocytopenia during ruxolitinib treatment from January 2016 to July 2017 in Peking Union Medical College Hospital were retrospectively analyzed. All patients were treated with a combination of low dose of thalidomide (around 75 mg/d), stanozolol (2 mg/time, 3 times per day), and low dose of prednisone (0.5 mg·kg-1·d-1). The hematological response and safety were assessed.@*Results@#There were 9 cases of anemia and 7 cases of thrombocytopenia during ruxolitinib treatment. After TSP regimen treatment, there were 5 cases of hemoglobin response including 1 case of complete remission (CR). There were 4 cases of platelet response and all achieved CR. Totally, the hematological overall response was seen in 7 cases. The median time from taking medicine to getting response was 27 d (13-89 d). 3 patients lost efficacy, while the median duration of response didn't reach (28-207 d).@*Conclusion@#TSP regimen can improve anemia or thrombocytopenia during ruxolitinib treatment in patients with MF.
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Objective@#To evaluate the response of oral melphalan plus high-dose dexamethasone (MDex) for patients with primary light chain amyloidosis (pAL).@*Methods@#Clinical data, hematological and organ responses, and survival of 76 patients with pAL who had received MDex from January 2009 to July 2017 were retrospectively analyzed.@*Results@#Of 76 patients (47 males and 29 females with the median age of 56 [range, 20-74] years old), 19.70% patients were defined as Mayo 2004 stage 3, involvement of more than or two organs was presented in 65 (85.53%) patients. Among 60 response evaluable patients, overall hematological response was 48.33% with complete response of 20.00% and very good partial response of 20.00%, respectively. The median time to the hematological response was 5 (range, 1-15) months. 36.67% patients achieved organ response. After the median follow up of 23(range, 1-113) months for surviving patients, median progression-free survival (PFS) and overall survival (OS) were 34 and 43 months, respectively. In a three months landmark analysis, the median rates of PFS and OS were 46 and 65 months, respectively. The median OS rates of patients with Mayo 2004 stage 3 and non Mayo 2004 stage 3 were 5 and 65 months (P=0.001), respectively.@*Conclusions@#MDex was an effective treatment for patients with early stage pAL, but was not suitable for those with severe cardiac involvement.
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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.
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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.
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Objective To evaluate the safety and efficacy of Hyper-CVAD intensive chemotherapy regimen in patients with newly diagnosed aggressive T-cell lymphoma. Methods The efficacy, side effects and survival status were retrospectively analyzed in 34 patients with newly diagnosed aggressive T-cell lymphoma who received Hyper-CVAD regimen as induction therapy in Peking Union Medical College Hospital from September 2009 to December 2010. Results Of 34 patients, 28 cases (82.4 ％) showed treatment response, including 10 cases (29.4 ％) of complete response (CR). Eleven patients underwent stem cell transplantation, including 1 case of human leukocyte antigen-identical siblings allogeneic stem cell transplantation. The median follow-up was 16 months (1-82 months), and the overall survival (OS) rate of 1 or 3-year was 70.2 ％ and 41.1 ％ respectively, and progression-free survival (PFS) rate of 1 or 3-year was 49.3 ％ and 31.6 ％ respectively. The major adverse reaction was myelosuppresion, including 18 cases (52.9％) of myelosuppresion with grade Ⅳ. Three patients died of serious infection. Cox regression multifactor analysis showed CR was the only influencing factor for PFS (HR=6.118, 95％CI 1.327-28.206, P=0.020). Marrow involvement (HR= 0.270, 95 ％CI 0.101-0.722, P= 0.009) and CR (HR= 6.669, 95 ％CI 1.754-25.354, P= 0.005) were independent influencing factors for OS. Conclusions Hyper-CVAD regimen has a high response rate for aggressive T-cell lymphoma, but the lasting effectiveness and the short-term efficacy show unfavorable performances. Meanwhile, myelosuppression is serious and infection incidence is high. Autologous hematopoietic stem-cell transplantation after remission may improve the outcome.
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Objective@#To evaluate the clinical characteristics and outcomes of very high risk patients with primary immunoglobulin light-chain amyloidosis (pAL) at a single center in China.@*Method@#Clinical data, treatment and outcome of 205 pAL patients in Peking Union Medical College Hospital from January 2009 to February 2016 were retrospectively analyzed. A 'very high risk’ group includes patients with Mayo 2004 stage Ⅲb and Mayo 2012 stage 4.@*Results@#Of 205 patients, 34 (16.6%) were defined as very high risk pAL patients. The median age at diagnosis was 57 (20-84) years, and 22 patients (64.7%) were male. All 34 patients were diagnosed with cardiac involvement, multi-organ involvement was observed in 15 patients (44.1%) , and 27 (81.8%) had New York Heart Association Class Ⅲ or Ⅳ. Median values of serum cTnI, NT-proBNP, and free light chains difference were 0.25 μg/L, 11 733 ng/L, and 403 mg/L, respectively. Eight (24.2%) had more than 10% plasma cell on the bone marrow aspirate. Sixteen (47.1%) patients received bortezomib based chemotherapy and overall hematologic response rate was 58.3%. Median overall survival (OS) was 4 months. The estimated OS at 3, 6, 12, and 24 months was 51.3%, 44.0%, 35.2%, and 29.6%, respectively. Fourteen (41.2%) patients died within 3 months after the diagnosis. The estimated 1-year survival rate for the patients who got hematologic response, without hematologic response, and palliative treatment was 90.9%, 11.1%, and 0, respectively (P<0.001) .@*Conclusion@#Patients with very high risk pAL had very poor prognosis and the early death rate remained high. Those patients who obtained hematologic remission would have significantly better outcomes.
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Objective@#To deepen the knowledge of HIV-negative plasmablastic lymphoma (PBL) .@*Methods@#Medical records from 8 HIV-negative PBL patients diagnosed in Peking Union Medical College Hospital from January 1997 to May 2015 were collected, and the clinical features and prognosis of these patients were analyzed.@*Results@#All of these 8 patients were diagnosed as HIV-negative PBL, 3 of 8 patients were males, and others were female. The median age was 60 (43-80) year. Among these patients, 4 cases had underlying immunosuppressive state. These patients all had extra-nodular involvement, and 6 cases of them were at stage Ⅳ according to Ann Arbor Staging, 5 patients had bone marrow involvement. CD38 and CD138 were diffusely positive for all patients, while the positive rate of B cell marker including PAX-5 and Bcl-6 were relative low. 5 of 8 patients had been detected for EBV-DNA, and all of them were negative. The median follow-up for the 7 patients receiving chemotherapy and regular follow-ups was 36 (11-57) months, the median progression-free survival (PFS) was 15 (6-52) months, and the median overall survival was 36 (2-52) months. Among these patients, 4 cases had received chemotherapy combined with Bortezomib, showing 3 cases of effective, but it seems to be difficult to keep the long term efficacy, and disease progression occurred in 2, 9, and 21 months after treatment. 2 patients at stageⅠ-Ⅱ were treated effectively, without disease progression and survival, 5 patients at stage Ⅳacquired the efficacy unsustainably, with a median PFS of 10 (2-21) months and a median overall survival of 12 (6-52) months.@*Conclusion@#HIV-negative PBL is relatively prevalent in elderly patients, and presenting with high invasiveness in clinical, extremely prone to extra-nodular involvement, especially the bone marrow. The immunophenotype of PBL is more resemble to that of plasmacytoma. Patients who were in late stage at diagnosis show poor prognosis.
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Objective@#To evaluate the clinical characteristics, MYD88L265P mutation, CXCR4WHIM mutation and prognosis in patients with Waldenström macroglobulinemia (WM).@*Methods@#The clinical characteristics, International Prognostic Scoring System for symptomatic WM (WPSS) , and overall survival (OS) were retrospectively assayed in 93 patients with newly diagnosed WM at Peking Union Medical College Hospital during January 2000 to August 2016. The MYD88L265P mutation and CXCR4WHIM mutation were tested among 34 patients.@*Results@#The median age of the 93 patients was 64 years (range, 33-85 years) with a male-to-female ratio of 2.44. According to WPSS, we included 16 (17.2%) low-risk, 44 (47.3%) intermediate-risk and 33 (35.5%) high-risk patients. Eight patients had secondary amyloidosis. With a median follow-up of 44 (1-201) months, the median OS was 84 months. Cox regression multifactor analysis showed WPSS risk group (HR=2.342, 95% CI 1.111-4.950, P=0.025) , whether patients had secondary amyloidosis (HR=5.538, 95% CI 1.958-15.662, P=0.001) and whether patients received new drugs (HR=3.392, 95% CI 1.531-7.513, P=0.003) were independent factors associated with OS. We have investigated the presence of the MYD88L265P and CXCR4WHIM mutation in 34 patients and found that MYD88L265P mutation was occurred in 32 patients (94.1%) and CXCR4WHIM mutation was occurred in 8 patients (23.5%). Seven of 8 patients who harbored CXCR4WHIM-mutated also exhibited the MYD88L265P mutation. Patients with MYD88L265PCXCR4WHIM vs MYD88L265PCXCR4WT presented with more severe anemia, lower platelet level, higher M protein level and more hyper-viscosity syndrome.@*Conclusion@#WPSS risk group, whether patients had secondary amyloidosis or received new drugs are independent factors for OS in WM. MYD88L265P and CXCR4WHIM mutation, the most common somatic variants in WM, often occur together and impact the clinical presentation.
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Objective@#To explore the clinical characteristics, treatment, and prognosis of patients with blastic plasmacytoid dendritic cell neoplasm.@*Method@#Clinical records of 6 patients diagnosed with blastic plasmacytoid dendritic cell neoplasm in our hospital from January 2008 to May 2016 were collected and retrospectively analyzed.@*Results@#Six patients manifested with initial symptoms of skin lesions, other common symptoms included bone marrow involvement (5/6) , lymphadenectasis (4/6) , splenomegaly (4/6) , and hepatomegaly (3/6) . In addition, extra-nodal involvement except skin was also observed, including breast (1/6) , maxillary sinus (1/6) , vertebrae (1/6) , and central nervous system (1/6) . Characteristic immunophenotype, CD4, CD56, and CD123 were all positive. All these patients were treated with acute lymphoblastic leukemia type (ALL-type) chemotherapy and complete remission (CR) were reached in 4 patients. The median follow-up was 9.5 (7-37) months, median progression free survival was 7 months; while median overall survival was 9 months. A total of 3 patients died during the follow-up, which were all happened in the first year after diagnosis, and all resulted from the relapse or disease progression.@*Conclusion@#Blastic plasmacytoid dendritic cell neoplasm is highly aggressive, in which the skin lesions are always manifested as initial symptoms, and bone marrow involvement, lymphadenectasis, splenomegaly, and hepatomegaly is also common. Characteristic immunophenotype include the positivity of CD4, CD56, and CD123. Effective and standard therapy is limited in this disease, which indicates the poor prognosis.
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Objective@#To explore the clinical characteristics, treatment and prognosis in 17 patients with primary cold agglutinin disease (CAD) .@*Methods@#Clinical data, treatment and survival status of 17 patients diagnosed with primary cold agglutinin disease in Peking Union Medical College Hospital during April 2007 to October 2016 were retrospectively analyzed. The MYD88L265P mutation was tested in 4 patients.@*Results@#The median age of 17 patients was 67 years (range, 51-86 years) , and male- to female ratio was 1.1∶1. Seven patients were diagnosed with indolent lymphoma, including 3 Waldenstrom macroglobulinemia/lymphoplasmacytic lymphoma (WM/LPL) , 2 small lymphocytic lymphoma/chronic lymphocytic leukemia (SLL/CLL) , and 2 splenic marginal zone lymphoma (SMZL) . 15 patients experienced anemia. The median HGB level was 67 (35-127) g/L. 11 patients had cold agglutinin (CA) titers ≥1∶64, with median CA of 1∶1 024. MYD88L265P mutation was detected in 1 patient. 12 patients received drug therapy: 7 were treated with glucocorticoid-based therapy and 1 patient responded to treatment; 5 received rituximab-based therapy and 3 patients responded to treatment. With a median follow-up of 14 (0.5-96) months, the median overall survival was not reached.@*Conclusion@#Clinical manifestations of CAD are various, and diagnosis is dependent on CA testing. The efficacy of glucocorticoid-based therapy is limited, and rituximab is recommended for CAD treatment.
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Objective@#To evaluate the clinical characteristics, diagnosis criteria, treatment and prognosis in patients with Bing-Neel Syndrome (BNS) .@*Methods@#The clinical characteristics, lab data, treatment and outcomes of 3 Bing-Neel syndrome patients diagnosed at Peking Union Medical College Hospital were collected.@*Results@#The clinical presentation was heterogeneous without any specific common signs or symptoms. One patient was diagnosed with BNS 42 months after diagnosis of Waldenström macroglobulinemia (WM) by cerebrospinal fluid (CSF) cytology and flow cytometry, but dead of infection during the first course of chemotherapy. BNS was the first manifestation of WM in the other 2 cases. They were diagnosed by flow cytometry and cytology of CSF. The detection of MYD88L265P mutation in CSF contributed to diagnosis and to sequential monitoring of minimal residual disease. They received systemic chemotherapy of FC (fludarabine + cyclophosphamide) ± rituximab and intrathecal therapy, followed by maintenance therapy of chlorambucil or R2 (rituximab + lenalidomide) . They were followed 17 and 20 months respectively without progression of disease.@*Conclusion@#The diagnosis approach of BNS should be based on a combination of CSF cytology, flow cytometry and detection of the MYD88L265P mutation. The detection of MYD88L265P mutation may be useful in the monitoring of minimal residual disease.
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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.
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<p><b>OBJECTIVE</b>To evaluate the usage of Mayo staging system in Chinese patients with primary light chain (LC) amyloidosis.</p><p><b>METHOD</b>Clinical data, treatment and outcome of 162 primary LC amyloidosis patients with Mayo Clinic staging in Peking Union Medical College Hospital from January 2009 to June 2015 were retrospectively analyzed.</p><p><b>RESULTS</b>The median age of 162 patients with Mayo Clinic 2004 stage was 57 (20-81) y, of them 62.3% were male. The number of patients with stage I to III were 44 (27.2%), 69 (42.6%), and 49 (30.2%), respectively. The median overall survival was not reached, 23 months and 12 months in patients with Mayo Clinic 2004 stage I, II, and III, respectively (P<0.001). Among 128 patients with Mayo Clinic 2012 stage, 48 patients (37.5%), 32 patients (25.0%), 32 patients (25.0%) and 16 patients (12.5%) were staged as Mayo Clinic 2012 stage 1 to 4, and the median OS was not reached, not reached, 13 months and 3 months, respectively (P<0.001).</p><p><b>CONCLUSION</b>Mayo Clinic staging systems had important prognostic value in patients with primary LC amyloidosis.</p>