Hematogenous extramedullary relapse in multiple myeloma - a multicenter retrospective study in 127 patients

The current study assesses the characteristics and outcomes of multiple myeloma (MM) patients, treated with novel agents for hematogenous extramedullary (HEMM) relapse. Consecutive patients diagnosed with HEMM between 2010-2018 were included. Patients' characteristics at diagnosis and at HEMM presentation, response to treatment, survival and factors predicting survival were recorded and analyzed. A group of 127 patients, all diagnosed with HEMM by imaging (87.3%) and/or biopsy (79%), were included. Of those, 44% were initially diagnosed with ISS3, 57% presented with plasmacytomas, and 30% had high-risk cytogenetics. Median time to HEMM was 32 months. In multivariate analysis, ISS3 and bone plasmacytoma predicted shorter time to HEMM (P = .005 and P = .008, respectively). Upfront autograft was associated with longer time to HEMM (P = .002). At HEMM, 32% of patients had no BM plasmacytosis, 20% had non-secretory disease and 43% had light-chain disease. Multiple HEMM sites were reported in 52% of patients, mostly involving soft tissue, skin (29%), and pleura/lung (25%). First treatment for HEMM included proteasome inhibitors (50%), immunomodulatory drugs (IMiDs) (39%), monoclonal antibodies (10%), and chemotherapy (53%). Overall response rate (ORR) was 57%. IMiDs were associated with higher ORR (HR 2.2, 95% CI 1.02-4.7, P = .04). Median survival from HEMM was 6 months (CI 95% 4.8-7.2). Failure to achieve ≥VGPR was the only significant factor for worse OS in multivariate analyses (HR = 9.87, CI 95% 2.35 - 39, P = .001). In conclusion, HEMM occurs within 3 years of initial myeloma diagnosis and is associated with dismal outcome. The IMiDs might provide a higher response rate, and achievement of ≥VGPR predicts longer survival.     

Analysis of clinical characteristics and prognostic factors of multiple myeloma: a retrospective single-center study of 787 cases

Objective: This study aims to explore the clinical features of multiple myeloma (MM) and the influence of various prognostic factors on survival.

Methods: A retrospective analysis, consisting of clinical characteristics analysis and laboratory examinations, was performed on 787 MM patients. Clinical and laboratory parameters were analyzed by multivariate process and compared across different groups.

Results: Of the 787 patients enrolled (median age, 61 years old, range 29–89 years old), 491 (62.4%) were male. Two most common complaints were bone pain (51.2%) and fatigue (48.0%). Anemia (hemoglobin (Hb) ≤100?g/L in female, Hb ≤110?g/L in male) was present initially in 69.4% patients. IgG was the most common type (46.6%). 52.2% of the patients were diagnosed on stage IIIA according to Durie–Salmon (D–S) system, 44.6% are on stage III according to International Staging System (ISS). Multivariate analysis suggested that age, serum calcium, LDH, percentage of abnormal plasma cells in bone marrow were all independent prognostic factors for OS.

Conclusion: The MM patients in China are relatively younger, have higher rate on stage III according to D–S system. Older age, high serum calcium, high LDH, high percentage of abnormal plasma cells in bone marrow were highly related to poor prognosis.     

Late and localized extramedullary relapse of a light chain kappa myeloma after syngeneic bone marrow transplantation

We report the case of a 54-year-old female patient with stage IIIA kappa light chain myeloma (MM) who relapsed 7 years after syngeneic bone marrow transplantation (BMT). The relapse occurred as a voluminous soft tissue plasmacytoma in the leg, developing after local trauma. The patient was successfully treated with local radiotherapy and has remained progression-free for more than 2 years. This case represents one of the longest survivors, in complete remission, after syngeneic transplantation for MM. The presentation of recurrent disease as localized plasmacytoma with extramedullary growth is unusual in the post-transplant setting. Bone Marrow Transplantation (2000) 25, 115-117.     

Central nervous system involvement in patients with granulomatosis with polyangiitis: a single-center retrospective study

Clinical Rheumatology - The aims of this study were to estimate the frequency of central nervous system (CNS) involvement in Greek patients with granulomatosis with polyangiitis (GPA) and describe...     

274例青少年和成人传染性单核细胞增多症患者肝损伤临床特征分析

目的分析青少年和成人传染性单核细胞增多症(IM)患者肝损伤的临床特征及影响因素。方法回顾性分析2005年1月1日—2018年12月31日北京大学第一医院住院的青少年和成人IM患者的肝损伤特征,按照年龄、血清EBV DNA水平、是否合并黄疸、是否合并巨细胞病毒及HEV感染分为不同亚组,比较亚组间的各项指标的差异。计量资料符合参数分析条件者2组间比较采用t检验,不符合参数分析条件者2组间比较采用Mann-Whitney U检验;计数资料2组间比较采用χ2检验或Fisher精确概率法。多因素分析采用logistic回归分析。结果共纳入274例患者,其中男154例(56.2%),女120例(43.8%);发病平均年龄为(22.3±6.7)岁。肝损伤(定义为ALT>50 U/L和/或AST>40 U/L)发生率为97.4%(267/274),黄疸发生率27.6%(74/268)。年龄≥20岁者GGT更高(Z=2.070,P=0.038)。167例患者检测了血清EBV DNA,其中阳性组90例,阴性组77例。血清EBV DNA阳性组比阴性组的GGT(Z=3.005,P=0.003)及LDH(Z=2.162,P=0.031)水平更高。合并巨细胞病毒感染组ALP更高(Z=2.351,P=0.019);合并HEV感染组GGT(Z=1.988,P=0.047)更高。AST(OR=1.006,95%CI:1.002~1.010,P=0.005)和ALP(OR=1.012,95%CI:1.005~1.020,P=0.001)为影响黄疸发生的独立危险因素。结论青少年和成人IM患者肝损伤发生率很高,其中年长者和血清EBV DNA阳性者的肝损伤更严重。     

老年患者侵袭性念珠菌病特点和治疗研究

目的:探讨老年患者侵袭性念珠菌病(IC)的临床及真菌学特点、治疗现状与预后。方法:回顾性收集2010年1月至2019年12月10年间依据真菌培养确诊为IC的老年住院患者(≥65岁)的临床资料,对其感染菌株进行鉴定及耐药性实验,分析老年IC患者的临床、真菌学、治疗与预后特点,并与非老年人患者进行比较。结果:共纳入99例老...     

Features of extramedullary and extraosseous multiple myeloma: a report of 19 patients from a single center

Extramedullary (EM) localizations at diagnosis or during the course of multiple myeloma (MM) are rare. We conducted a large retrospective study to more accurately describe the clinical and laboratory features of this entity, and the outcome of these manifestations. The charts of 19 eligible patients out of 432 patients with MM were retrieved from the hematology department of the Institut Paoli-Calmettes Cancer Center. Median age was 61 (range: 39-79) with a female/male sex ratio of 8/11. Ten patients were found to have EM and extraosseous tumor at the time of MM diagnosis, and nine patients developed EM tumor during the course of the disease. Neither the stage of the disease, the LDH level, or the type of immunoglobulin (Ig) was found to be associated with the development of EM disease. Patients who developed EM tumor during the course of MM had a lower serum Ig and a higher monoclonal Bence-Jones proteinuria at the diagnosis of MM than patients who presented with EM tumor at diagnosis. Multiple sites were usually involved. Resistance to chemotherapy was frequent and response to thalidomide was poor. Eight out of the 19 patients responded to high-dose chemotherapy. The remaining 11 patients progressed while on therapy. With a median follow-up of 13 months (range: 2-65), six patients are alive, four patients are in partial remission and two patients in present progressive disease. In conclusion, EM tumors are a rare manifestation of MM, with a cumulative incidence of 4.6% of MM. Multiple sites are usually involved. The response to chemotherapy is very poor with a very low response rate to thalidomide. The prognosis is very poor, especially when the diagnosis of EM tumor is concurrent with the diagnosis of MM.     

Treatment of hemophagocytic lymphohistiocytosis with chemotherapy and bone marrow transplantation: a single-center study of 22 cases

Twenty-two children with hemophagocytic lymphohistiocytosis were treated with a chemotherapy regimen consisting of VP16-213, corticosteroids, and intrathecal methotrexate. A sustained clinical and biologic complete remission was obtained in 15 children and a partial remission in one child; six children died early of opportunistic infection (n = 4) or of disease progression (n = 2). Of the 16 children who were placed in first remission, 10 received maintenance chemotherapy alone, while six underwent bone marrow transplantation (HLA matched in five, HLA mismatched in one). Of the children who received chemotherapy alone, only two are in long-term remission after cessation of treatment. The remaining eight patients relapsed after a mean period of 5.4 months (range 2 to 8 months). Further treatment using the same regimen induced second remissions of short duration; death occurred after a median period of 2.3 months (range 0.5 to 6 months). A total of nine patients received allogeneic bone marrow transplantation (BMT). Among the six children transplanted in remission, four are in long-term unmaintained remission, 1 to 6 years after HLA-matched BMT. However, the relapse that occurred in one patient 1 year post BMT is difficult to interpret because the donor, the patient's 5-year-old sister, also developed the disease 1 year later. An HLA-nonidentical BMT resulted in unmaintained remission for 1 year, with autologous hematologic reconstitution followed by disease relapse. HLA-nonidentical BMT failed in three other patients with active disease at time of transplant. The poor long-term results of chemotherapy alone justify the use of related HLA-matched BMT in complete remission.     

Primary esophageal adenosquamous carcinoma: a retrospective analysis of 24 cases

Primary adenosquamous carcinoma (ASC) of the esophagus is a rare kind of malignancy characterized by mixed glandular and squamous differentiation as well as a propensity for aggressive clinical behavior. Data on the evaluation of the clinicopathological features and the prognosis of patients suffering from this malignancy are few because of the rarity of this disease. We conducted a retrospective review of 24 patients with primary esophageal ASC among 6546 esophageal cancer patients who underwent transthoracic esophagectomy in our hospital. The clinicopathological presentation, diagnosis, treatment, and prognostic factors of the patients were respectively investigated. The Kaplan–Meier method and the log rank test were used to calculate and compare overall survival (OS). The Cox proportional hazards model was employed to identify independent prognostic factors. There were 18 males and 6 females with a median age of 60 years (range: 40–78 years). The clinical symptoms, macroscopic type, as well as the radiological and endoscopic features of esophageal ASC were similar to those of esophageal squamous cell carcinoma. Sixteen (88.9%) of the 18 cases who underwent preoperative esophagoscopic biopsy were misdiagnosed as adenocarcinoma or squamous cell carcinoma. The overall median follow‐up period was 36 months, and the median survival time was 32 months. The 1, 3, 5‐year OS rates were 75.0%, 48.5%, and 19.4%, respectively. Univariate analysis showed that gender (P = 0.047), lymph node metastasis (P = 0.007), and TNM stage (P = 0.037) were important factors associated with OS of the 22 patients who underwent radical resection. Multivariate analysis showed that the pathological N stage was the only independent prognostic factor (P = 0.031, hazard ratio [HR], 5.369, 95% confidence interval [CI], 1.167–24.700). These results suggest that esophageal ASC is an uncommon disease prone to be misdiagnosed by endoscopic biopsy. Surgical resection is the primary treatment, but the prognosis of ASC is usually poorer than conventional squamous cell carcinoma. Lymph node metastasis is an independent prognostic factor after radical resection.     

Clinicopathological study on peripheral T-cell non-Hodgkin lymphoma with bone marrow involvement: a retrospective analysis from China

We reviewed 173 patients with an initial diagnosis of peripheral T-cell non-Hodgkin lymphoma (PTCL) and compared the patients with bone marrow involvement (BMI) to those without to have a better understanding of the clinical characteristics, treatments, survival and prognosis of PTCLs with BMI. We found that 40% (70/173) of the patients had BMI, and its frequency was 64% in angioimmunoblastic T-cell lymphoma (TCL), 46% in PTCL unspecified, 29% in anaplastic large T-cell lymphoma, 23% in extranodal NK/T-cell lymphoma and 13% in enteropathy-type TCL. In the BMI group, 36% of patients had lymphoma-associated hemophagocytic syndrome (LAHS), compared with 8% of the patients without BMI (8/103, P < 0.001). The estimated 1-year overall survival (OS) rates of patients with LAHS in the BMI and non-BMI groups were 5 and 49%, respectively. The increased levels of lactate dehydrogenase, fasting triglycerides and β₂-microglobulin between the BMI and non-BMI groups were not significantly different, but ferritin increased significantly and liver dysfunction-related diseases were seen more in the BMI group. As much as 51% of patients of the BMI group had anemia, compared with 27% of the patients without BMI (P = 0.001). The estimated 2-year OS rates in the two groups were 10 and 34%. The estimated 2-year OS rate of the 67 patients with BMI, who did not lose to follow-up, was 22%, compared with 38% in the non-BMI group. The median survival times of the 2 groups were 120 and 356 days. The estimated 2-year OS rate of patients treated by CHOP regimen was 9%, compared with 51% of those with intensive chemotherapy, with a significant difference (log rank P = 0.0008). The median survival time of the 14 patients subjected to chemotherapy combined with l-asparaginase was 365 days and that of the 7 patients undergoing hemopoietic stem cell transplantation (HSCT) was 575 days. A total of 3 patients in a critical condition underwent plasmapheresis as initial therapy and achieved stable condition. We conclude that patients with PTCLs with BMI on initial diagnosis usually have hemaphagocytic syndrome and poor prognosis. BMI without lymphadenopathy is a patent clinical feature in most PTCLs. Patients with anemia on initial diagnosis in the BMI group usually have poor prognosis than those without. Intense chemotherapy, addition of l-asparaginase in chemotherapy and HSCT are comparatively efficient treatments of PTCLs. For patients in critical conditions, plasmapheresis before chemotherapy would lower the risk and improve the tolerance to chemotherapy.     

Efficacy and safety of bortezomib-based retreatment at the first relapse in multiple myeloma patients: A retrospective study

Abstract

Objective

Multiple myeloma remains incurable and retreatment with available therapies is of substantial interest.

Methods

This retrospective observational study included data from 35 patients treated initially and at the first relapse with bortezomib-containing regimens.

Results

Bortezomib retreatment provided a similar depth and time to response as first-line therapy; however, as could be expected, the duration of response was shorter with retreatment. The tolerability profile was similar with bortezomib as the first- and second-line therapy, with no evidence of cumulative toxicity.

Conclusion

These findings support bortezomib retreatment after a treatment-free interval of ≥6 months in patients who achieved at least a partial response to the first-line bortezomib-based therapy.     

Clinical characteristics of intravascular large B cell lymphoma: a single-center retrospective study

<正>Objective To investigate clinic-pathological characteristics,diagnosis,treatment and prognosis of intravas-cular large B cell lymphoma (IVLBCL) in China.Methods Clinical and pathological records were analyzed from 12 IVLBCL patients diagnosed between Jan 2010and Jun 2016. Kaplan-Meier method was used to estimate overall survival (OS),and univariate analysis was performed to identify prognostic factors. Results A series     

Articular involvement in human brucellosis: a retrospective analysis of 304 cases

Brucellosis is a zoonosis which in humans is caused by one of four species of the Brucella genus: B. melitensis, B. abortus, B. suis and B. canis. B. abortus is the species prevalent in North America and Europe and B. melitensis in most developing countries.Differences in disease manifestations may be accounted for either by differences in the species or by differences in the host. Articular involvement in brucellosis, although recognized since 1904, has been variably emphasized.Three hundred and four cases of human Brucellosis caused by B. melitensis, the prevalent species in Perú, were seen during a 12-yr period in one Lima hospital. Fever, malaise and hepatomegaly were the most frequent findings. Diagnosis was greatly improved when cultures were done in the biphasic Ruiz-Castañeda medium, rather than in trypticase soy broth. Serologie diagnosis is still important, and it should include standard tube testing, detection of IgG blocking antibodies and fractionation with 2-ME in chronic cases.The disease may take one of three courses: acute, (<8 wk), chronic (>8 wk) or undulant (periods of remissions and exacerbations). Four syndromes were recognized in a total of 33.8% of patients with Brucellosis. The most frequent pattern (in ~46.6% of patients with arthritis) was sacroiliitis, usually non-destructive and either uni-or bilateral. The second most frequent articular syndrome was peripheral arthritis (38.8%), manifested either as a single large lower extremity joint or as an asymmetric pauciarthritis. Rarely patients presented with a rheumatoidlike arthritis. Mixed arthritis (7.8%) was a combination of the first two. The above forms occurred in patients with an acute or undulant course. Spondylitis was the least common form of arthritis (6.8%), and differed significantly from the other forms of arthritis in the duration of symptoms (chronic course), age of patients (older individuals) and the paucity of fever and malaise. It also tended to be destructive.The arthritis usually resolved with the combined regimen of tetracycline (2 g p.o. for 21 days) and streptomycin (1 g i.m. for 21 days) without sequelae. Illustrative cases of these syndromes are presented.The relatively benign nature of most of the patients with brucellar arthritis lead us to postulate that they are for the most part reactive arthritides. Host factors are thought to be important in determining the response to the infection, but they are yet to be identified. Our own genetic studies have failed to identify an increased frequenty of B27 or CREG antigens in the patients with sacroiliitis. The demonstrations of circulating immune complexes in these patients however favors an immunopathogenic mechanism as responsible for some of the manifestations of this disease.     

Allogeneic bone marrow transplantation in multiple myeloma: a report of four cases

Allogeneic bone marrow transplantation offers a new and promising form for treatment of multiple myeloma incurable with chemotherapy. We present four cases of advanced multiple myeloma given bone marrow transplantation from HLA-identical and MLC-negative sibling donors. One patient had a recurrent plasmacytoma 8 months later and one died 12 days after the transplantation whereas the other two are in good clinical remission 15 and 19 months post-transplantation.     

Superior vena cava syndrome caused by a malignant tumor: a retrospective single-center analysis of 124 cases

Purpose

Superior vena cava syndrome (SVCS) results from compression of the superior vena cava. SVCS is an emergency requiring immediate diagnosis and treatment. We hypothesized that the outcome of patients (pts.) admitted during regular work hours may differ from that of pts. admitted on weekends.

Methods

From 1992 to 2011, we analyzed all pts. admitted with SVCS due to a malignancy. Clinical outcome was analyzed, focusing on the work-up of pts. hospitalized on a weekend compared with those hospitalized during the week.

Results

One hundred and twenty-four pts. with malignant causes of SVCS were analyzed. Causes were as follows: small cell lung cancer (SCLC) 28.2 %, non-small cell lung cancer 25 %, non-Hodgkin’s lymphoma 25 %, metastasis of other malignant tumors 19.4 % and Hodgkin’s disease 2.4 %. Sixty-five percent of pts. were admitted during the week and 35 % on a weekend. Sixty-one percent received chemotherapy, 24 % radiation, 4 % radiochemotherapy, 9 % palliative treatment and 2 % no treatment at all. No difference in choice of treatment between pts. admitted on a weekday versus weekend was seen. Response was as follows: 7 pts. complete remission, 20 pts. partial response, 38 pts. progressive disease, 3 pts. NC and 15 pts. died. Overall response rate was as follows: Hodgkin’s disease 100 %, non-Hodgkin’s lymphoma 29 %, SCLC 22.8 %, non-small cell lung cancer 9.6 % and metastatic cancer 16.6 %. Only 2 of the 34 pts. with relapsing carcinoma responded. None of the pts. died due to SVCS.

Conclusion

The outcome of pts. with SVCS is not dependent on the day of admission (weekend or weekday) but is related to underlying disease in the setting of a tertiary care center.