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Mantle cell lymphoma is a B-cell malignancy with unique biological, pathological and clinical characteristics, accounting for about 5%-10% of non-Hodgkin lymphoma (NHL), and most patients are diagnosed at advanced stage. Mantle cell lymphoma has the aggressive characteristic of aggressive lymphoma and the refractory characteristic of indolent lymphoma, and the prognosis of patients is poor. In recent years, with the development of high-dose chemotherapy, autologous hematopoietic stem cell transplantation and new drug research, the survival time of patients has been significantly prolonged.
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Mantle cell lymphoma is characterized by t(11;14) with CCND1-IGH fusion and manifests with a spectrum of disease ranging from relatively indolent to aggressive. Here, we present a case of pleomorphic mantle cell lymphoma with three fusion signals that presented with lethal atraumatic splenic rupture. We discuss on the implications of variant CCND1 signal patterns as well as the epidemiology and pathophysiology of atraumatic splenic rupture.
Subject(s)
Humans , Male , Aged , Splenic Rupture/pathology , Lymphoma, Mantle-Cell/epidemiology , Splenomegaly/complications , Lymphoma, Mantle-Cell/physiopathology , Cyclin DABSTRACT
Mantle cell lymphoma (MCL) is an aggressive and rare B-cell lymphoma, accounting for around 6%-8% of non-Hodgkin's lymphoma (NHL). Up to now, there are a lot of studies and reports on the biological behavior, diagnostic criteria and treatments of MCL worldwide. However, due to its high invasiveness, there are still many problems to be solved in terms of prognosis and treatment. This article introduces MCL and its progress in the unsolved clinical problems.
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Mantle cell lymphoma (MCL) is a subtype of aggressive B-cell non-Hodgkin lymphoma(NHL) with a heterogeneous clinical characteristics, accounting for 3%-10% of adult-onset NHL. With the great advance of novel drugs, the therapeutic options for MCL are constantly updating. The 61st American Society of Hematology Annual Meeting reported the recent treatment progress of MCL including novel drugs,combinations of nonchemotherapeutic agents and chimeric antigen receptor T-cell therapy. This paper focuses on the treatment progress of MCL.
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Objective@#To investigate clinicopathologic features and prognostic factors of mantle cell lymphoma(MCL).@*Methods@#The clinical data of 349 MCL patients diagnosed at Beijing Friendship Hospital from January 2004 to January 2016 were retrospectively collected. Corresponding histological sections were reviewed. Additional studies included immunohistochemical staining using the MaxVision two-step method, IgH/CCND1 fusion gene detection by fluorescent in situ hybridization (FISH), and correlative statistical analysis.@*Results@#Of 349 patients with MCL, the median patient age was 61 years (range: 25-83 years, M∶F=2.7∶1.0) and the age of 243 patients ranged from 51 to 70 years (69.6%). Those with B symptoms accounted for 22.4% (70/313). Most of the patients presented with superficial lymphadenopathy and the clinical stage Ⅲ-Ⅳ accounted for 76.1% (235/309). Extranodal involvement was seen in 47.9% (148/309), among which the gastrointestinal tract accounted for 31.8% (47/148) and splenic involvement accounted for 15.4% (47/305). Three hundred and nine (88.5%) cases were of classical type and 40 (11.5%) cases were of aggressive variant type, and all were composed of proliferating lymphoid cells. All the tumors were positive for CD20 and cyclin D1, and 98.6% (344/349) tumors were weakly positive or positive for CD5. FISH test was positive in 12 cases that were CD5 negative and with cyclin D1 partial expression.Two hundred and forty-three (69.6%) patients had a median follow-up of 26 months (range: 3-108 months). The 3- and 5-year overall survival rates for patients were 63.0% and 34.8%, respectively. Single factor analysis showed that age of >60 years, splenic involvement, aggressive variant type, incompletely overlapping type [Based on the degree of overlap ≥90% and <90% between the follicular dendritic cell (FDC) meshwork and tumor cells, the tumors were divided into the completely overlapped type and incompletely overlapped type] and Ki-67 index >40% had poor prognosis (P<0.05). Multiple factor Cox proportional risk regression analysis after removing the aggressive variant type showed that age, splenic involvement, the degree of overlap between the FDC meshwork and tumor cells and Ki-67 index were independent prognostic factors for overall survival rate of MCL patients (P<0.05).@*Conclusions@#MCL is more commonly found among middle-aged and elderly men. Patient age, splenic involvement, degree of overlap between FDC meshwork and tumor cells and Ki-67 index are the independent prognostic indicators for MCL.
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Objective@#To investigate the clinicopathologic features and prognosis of gastrointestinal mantle cell lymphoma (GI-MCL).@*Methods@#Clinical data of 38 GI-MCL patients diagnosed at Beijing Friendship Hospital from January 2002 to January 2016 were retrospectively reviewed morphologically and immunophenotypically. IgH/CCND1 gene fusion was assessed by fluorescent in situ hybridization (FISH). For comparison, 60 cases of non-GI-MCL were randomly selected to extract the differences inclinicopathological features and patient survival between the two groups.@*Results@#Of 38 patients with GI-MCL, the median age was 62 years (range: 35-78 years, 23 males and 15 females), of which patients of 60 years of age or older accounted for 55.3%. Patients with clinical course of less than 6 months accounted for 81.1%(30/37). The main symptoms included abdominal pain, diarrhea, anorexia and hematochezia. Those with B symptoms accounted for 32.4%(12/37). The tumor most often involved lleocecal region (57.9%, 22/38), followed by rectum (36.8%, 14/38) and sigmoid colon (28.9%, 11/37), and the stomach accounted for 18.4%(14/38). Endoscopic polypoid lesions were found in 33 cases (86.8%, 33/38), of which 22 cases (66.7%, 22/33) were multiple. Five cases (13.2%, 5/38) presented with local protuberant neoplasm. According to Ann Arbor staging, 3 cases (7.9%, 3/38) were at stage Ⅰ, 4 cases (10.5%, 4/38) were at stage Ⅱ, and 31 cases (81.6%, 31/38) were at stage Ⅳ. The number of patients with tumor involvement of abdominal and retroperitoneal lymph nodes accounted for 45.7%(16/35), including 41.7%(15/36) involving the superficial lymph node, 17.1%(6/35) involving extranodal sites, and 23.5%(8/34) having splenomegaly. All of the 38 cases were classic MCL, and the tumor was composed of uniform lymphoid cells and effacing normal mucosal structure. All tumors were positive for CD20 and CD5. 97.4% (37/38) tumors were positive for cyclin D1, and 92.0% (23/25) tumors were positive for SOX11. FISH test was positive in 1 case of cyclin D1 negative tumor. Twenty-eight patients (73.7%) had a median follow-up of 25.0 months (range: 3-79 months). The 3-year survival rate for stage Ⅰ-Ⅱ and stage Ⅲ-Ⅳ of patients were 80.0% and 69.1%, respectively (P> 0.05). The 3-year survival rate for GI-MCL and non-GI-MCL patients were 71.7% and 72.5%, respectively (P>0.05). Single factor analysis showed that age of >60 years and splenomegaly were correlated with a worse overall survival rate (P<0.05).@*Conclusions@#Gastrointestinal malaise is the most common presenting symptom in GI-MCL patients. GI-MCL more commonly involves colorectum with more frequent multiple polypoid lesions. Patients of age >60 years and with splenomegaly have poor prognosis. There is no difference in the prognosis between GI-MCL and non-GI-MCL patients.
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Overexpression of CCND1 and t (11;14) (q13;q32) chromosomal translocation are important markers in mantle cell lymphoma (MCL). However, part of MCL lacks the expression of CCND1. SOX11 can be used as a biomarker for its overexpression in CCND1-negative MCL. SOX11 is a neurogenic transcription factor, and its overexpression is closely related to histone modification and DNA methylation. Differential expression of SOX11 in MCL is closely related with the plasma cell differentiation, and there is a relationship between the prognosis and survival time of MCL. SOX11 cDNA is more sensitive than conventional methods for the detection of minimal residual disease (MRD). With further research of SOX11, it will become an important basis of diagnosis and prognosis in MCL, and can provide a new method for targeted therapy of MCL.
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Objective@#To study the clinical features, therapeutic effects, prognostic factors of 140 patents with mantle cell lymphoma (MCL).@*Methods@#Clinical data of 140 MCL patients admitted from June 2009 to January 2016 in our hospital were retrospectively analyzed.@*Results@#The median age of 140 patients was 59 years with a ratio of 6∶1 for men and women. There were 134 cases (95.7%) in Ann-Arbor stage Ⅲ-Ⅳ, 37 cases (26.4%) with B symptoms, 61 cases (43.6%) with bone marrow involvement and 38 cases (27.1%) with enlarged spleen. The overall response rate (ORR), 3-year survival rate and progression-free survival rate in the treatment group with rituximab were 87.1%, 68.1% and 59.5% respectively, which were significantly higher than those in the rituximab-free treatment group (66.6%, 51.5% and 31.7%, respectively). The difference was statistically significant (all P<0.05). Among patients treated with rituximab, the complete remission rates (70.8% and 77.8%) of R-HyperCVAD/MA and VcR-CAP regimens were higher than those of R-CHOP regimen (39.0%, both P<0.05). However, there was no significant difference in the overall response rate, overall survival rate and progression-free survival rate (all P>0.05). Univariate analysis showed that age, Ki-67 index, B symptoms, bone marrow invasion, platelet count, LDH, β2-MG and MIPI scores were associated with overall survival (all P<0.05). Multivariate analysis showed that age (HR=4.940, 95% CI: 2.347 to 10.397), B symptom (HR=2.900, 95% CI: 1.517-5.544), β2-MG (HR=2.945, 95% CI: 1.656-5.238), Ki-67 index (HR=4.915, 95% CI: 2.554-9.456) and treatment with rituximab-containing regimen (HR=2.450, 95% CI: 1.352-4.440) were independent factors for OS.@*Conclusions@#Most patients with MCL were older adults and usually had bone marrow involvement and spleen involvement. Rituximab combined with chemotherapy (especially R-HyprCVAD/MA and VcR-CAP) had better clinical efficacy than conventional chemotherapy.
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Objective@#To investigate clinicopathological features and prognosis of tonsillar mantle cell lymphoma(TMCL).@*Methods@#Clinical data of 25 patients with TMCL at Beijing Friendship Hospital, Capital Medical University from 2002 to 2016 were included. All the cases were reviewed microscopically. Various immunohistochemical stains were performed using the MaxVision two-step method. IgH/CCND1 gene fusion was detected by fluorescent in situ hybridization(FISH). Additionally, randomly selected 40 cases of non-tonsil MCL of the same period were compared.@*Results@#Among all mantle cell lymphomas (MCL), TMCL accounted for 5.6%(25/449). The median age of the patients was 60 years(range: 44-82 years) with a M∶F ratio of 5.3 to 1.0. The main symptoms were sore throat and foreign body sensation and patients usually presented with enlargement or mass of tonsil. At the early stage of the disease, 18 cases(72.0%) were clinically misdiagnosed as tonsillitis. Lymph node involvement was present in 76.0%(19/25) of the patients. There were 4 cases(16.0%)with current splenic involvement, 11 cases(44.0%) with pharyngeal focal recidivism, and 3 cases(12.0%) with involvement of other non-lymphoid organs. Morphologically, tonsillar architectures were effaced at various degrees. Eighteen MCL cases showed classical type and 7 cases were blastoid variant. All tumors were positive for CD20 and cyclin D1. 92.0%(23/25) tumors showed weakly positive or positive expression for CD5. FISH test that IgH/CCND1 gene fusion was positive in two CD5 negative classical cases. 18 patients(72.0%) had a median follow-up time of 26 months(range: 6-81 months). The difference of survival rate between stage Ⅰ-Ⅱ and stage Ⅲ-Ⅳ patients was not statistically significant(P>0.05). Compared with NTMCL, TMCL was found to have higher proportion of stage Ⅰ-Ⅱ disease (χ2=12.789, P<0.01), lower the proportion of non-lymphatic organ involvement (χ2=8.125, P<0.01), and better prognosis (χ2=4.351, P=0.037).@*Conclusion@#The incidence of TMCL is low and prone to be misdiagnosed as tonsillitis. Patients with TMCL are more likely at stage Ⅰ-Ⅱ at presentation and the prognosis is better than that of NTMCL.
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In recent years, the incidence of non-Hodgkin lymphoma (NHL) is on the rise. Among the newly diagnosed NHL patients, 3 %-10 % are mantle cell lymphoma (MCL), which is common in middle-aged and elderly men, and the incidence of extranodal aggression is more in stage Ⅲ or Ⅳ. MCL has clinical and pathological features of the invasiveness in invasive lymphoma and the incurability in indolent lymphoma. Although the application of new drugs has made more progress in the treatment of MCL, the overall survival rate is not good; the majority of patients relapse after treatment, and there are no standard treatment regimens. The progresses in MCL will be summarized in this paper based on the reports in the 60th American Society of Hematology (ASH) Annual Meeting.
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Mantle cell lymphoma (MCL) is a malignant B-cell neoplasm, which comprises monomorphic and small- to medium-sized mantle zone-derived lymphoid cells. It is characterized by chromosomal translocation t(11;14)(q13;q32) and CCND1 truncation, resulting in cell cycle deregulation. It is an aggressive type of non-Hodgkin lymphoma with a propensity to present with extranodal involvement. This study shows the case of an 80-year-old Caucasian male who complained of a 2-month progressive swelling on the right side of his face. The magnetic resonance imaging exam showed multifocal involvement of the head and neck, including oral manifestations, bilateral parotid glands, palate, tongue, and floor of the mouth. An incisional biopsy of the tumor mass was performed. The morphological and immunophenotypic findings were consistent with the diagnosis of MCL. The patient died 4 months later, without any chance of undergoing a therapeutic approach. Although MCL is a rare condition, it should be subjected to a differential diagnosis when affecting the maxillofacial area. Imaging exams and both immunohistochemical and morphological analyses are needed to reach the correct diagnosis. Here, we present an unusual MCL with multifocal involvement of the head and neck.
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Humans , Male , Aged, 80 and over , Head and Neck Neoplasms/diagnosis , Lymphoma, Mantle-Cell/diagnosis , Oral Manifestations , Diagnosis, Differential , Fatal Outcome , Head and Neck Neoplasms/pathologyABSTRACT
El linfoma de células del manto hace parte del subgrupo de linfomas no Hodgkin (LNH). Este se manifiesta con adenopatías, esplenomegalia, síntomas B y compromiso cutáneo asociado a enfermedad diseminada. El compromiso esplénico se presenta como: Esplenomegalia sin lesión focal; lesiones sólidas únicas o múltiples e infiltración del bazo sin cambios morfológicos ni lesiones focales. La aparición de linfoma con lesiones quísticas es extremadamente rara, se encuentran solo unos cuantos casos en la literatura, ninguno de ellos en el bazo. Se expone el caso de una paciente de 59 años de edad, quien consultó por dolor abdominal intermitente. En los estudios diagnósticos se observó esplenomegalia con lesiones sólidas y quísticas. Se realizó esplenectomía con estudio histopatológico que confirmó compromiso por linfoma de células del manto.
Mantle cell lymphoma is a Non-Hodgkin Lymphoma (NHL). In cases of disseminated disease, lymphadenopathy, splenomegaly, B-symptoms and skin disease are present. Lymphoma affecting the spleen has several radiologic presentations, ranging from normal appearance, to splenomegaly or multiples focal solid lesions. Cystic presentation of lymphoma is rare and few cases have been reported, none of them involving the spleen. We report a case of a 59-year old female patient with cystic spleen lesions that after splenectomy were diagnosed as Mantle cell lymphoma.
Subject(s)
Humans , Lymphoma, Mantle-Cell , Tomography, X-Ray Computed , LymphomaABSTRACT
Objective To study the clinical features, effects of therapeutic regimen and prognosis of patents with mantle cell lymphoma (MCL). Methods Clinical data of 27 MCL patients admitted in Tianjin Medical University Cancer Institute&Hospital from January 2008 to December 2014 were retrospectively analyzed. Cox regression analysis was used to analyze influencing factors of prognosis of MCL. Results The median age was 68 years old for 27 patients, and the male-to-female ratio was 4.4∶1. Ann Arbor staging showed that 25 cases were stageⅢ-Ⅳ(92.6%), 8 cases were heptosplenomegaly (29.6%), 7 cases showed extranodal involvement (25.9%). ECOG scoring showed that 4 cases with scores of 2-4 (14.8%), 8 cases were 0-3 (29.6%), 14 cases were 4-5 (51.9%) and 5 cases were 6-11 (18.5%). The Ki-67 index≤30%was found in 9 cases (33.3%), and>30%was found in 18 cases (67.7%). Patients with B symptom was found in 10 (37.0%). The elevated lactate dehydrogenase (LDH) was found in 17 cases (63.0%). The increased Beta 2- microglobulin was found in 8 cases (29.6%). Seven patients were found with bone marrow involvement. The total effective rate (ORR) was 81.8%in group with R-CHOP method, and the ORR was 68.8%in group with CHOP method. Multivariate analysis showed that age, LDH and Ki-67 were independent factors influencing the prognosis of MCL (P60 years, elevated LDH and Ki-67 index>30%are with poor prognosis.
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Mantle cell lymphoma (MCL), is a disease with high heterogeneity, which is insensitivity to the chemotherapy and presents poor prognosis. Appearance of the Bruton tyrosine kinase (BTK) targeted inhibitors, such as ibrutinib, provides novel treatment strategy for MCL. This review focused on the latest achievements of BTK inhibitors in MCL treatment.
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Mantle cell lymphoma (MCL) is a group of highly aggressive non-Hodgkin lymphoma (NHL) in small B-cell lymphoma, accounting for 6 % of NHL incidence. MCL is characterized with its concealed onset, strong aggression, high malignancy and poor prognosis. Therefore, more attention should be paid to the diagnosis and differential diagnosis of MCL in clinic. Recently, diagnostic models of molecular pathology, researches on cyclin D1 protein negative MCL, staging prognosis and stratification treatment of MCL are worthy of attention.
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Mantle cell lymphoma (MCL) is a rare subtype of non-Hodgkin lymphoma.The MCL is classified into two subtypes, some patients have a chronic/indolent course and the others have a more aggressive course and short survival.In recent years, some novel therapeutic target agents for MCL have reports.These target agents achieve efficacy comparable to conventional chemotherapy in relapsed/refractory MCL.Therapeutic targents for MCL include the surface antigens of B-cell lymphoma, B-cell receptor signaling, DNA damage response pathway and the cellular microenviroment.This review focused on the recent research progress of target agents in the treatment of MCL.
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Here we report the case of a 73-year-old man who was diagnosed with metachronous, multiple primary tumors with non-Hodgkin B-cell mantle cell lymphoma involving the orbit on the basis of biopsy and immunohistochemistry in 2012. The patient had been diagnosed with non-Hodgkin small cell lymphoma and basal cell skin carcinoma in 2010 and intestinal adenocarcinoma with metastasis to the regional lymph nodes in 2011, thus representing a typical case of metachronous, multiple primary tumors. Mantle cell lymphoma is a rare disease and its prognosis is quite poor, particularly when it is associated with other metachronous malignancies. Therefore, physicians should consider mantle cell lymphoma as a differential diagnosis for neoplasms of the orbit.
Relatamos um caso de linfoma não-Hodgkin de células B subtipo células do manto acometendo região orbital, com o diagnóstico confirmado pela biópsia e testes imuno-histoquímicos em 2012. O paciente apresentava história de linfoma não-Hodgkin de pequenas células em 2010, carcinoma basocelular de pele no mesmo ano e adenocarcinoma intestinal com metástase para linfonodos regionais em 2011; caracterizando um caso de tumor primário múltiplo metacrônico. O linfoma de células do manto é uma doença rara, ainda mais quando associado a outras neoplasias metacrônicas, apresentando prognóstico bastante reservado. Dessa forma, deve estar entre os diagnósticos diferenciais de neoplasias da órbita.
Subject(s)
Aged , Humans , Male , Lymphoma, B-Cell/pathology , Lymphoma, Mantle-Cell/pathology , Neoplasms, Multiple Primary/pathology , Orbital Neoplasms/pathology , Biopsy , Immunohistochemistry , Magnetic Resonance ImagingABSTRACT
Objective To investigate the clinical and pathological characteristics,treatment response and clinical follow-up for patients with mantle cell lymphoma (MCL).Methods Clinical data,treatment efficacy and outcomes of 25 cases of MCL enrolled from Beijing Friendship Hospital were retrospectively analyzed.Results Median age of onset was 65 years old.Male to female ratio was 3.4:1,15 cases (60 ％) had bone marrow invasion,Ann Arbor clinical stage Ⅲ were 6 cases (24 ％),stage Ⅳ were 17 cases (68 ％).10 cases (40 ％) of patients had symptoms B.5 cases (20 ％) patients had elevation of lactate dehydrogenase (LDH).17 cases (64 ％) had elevation of β2 microglobulin (β2-MG).11 eases (64.71％) had complete remission (CR) after rituximab combined chemotherapy in 17 patients,2 years overall survival (OS) rate was 69.6 ％,2 years progression-free suvival (PFS) rate was 45.1％,significantly higher than those in traditional chemotherapy group (P ＜ 0.005).Outcomes analyses showed that variants of plasma cells,bone marrow invasion,LDH elevation,Ki-67 level,sMIPI ＞ 5 scores and increased leukocyte number were negative prognostic factors.IPI score,splenomeagly,age,symptoms B and elevation of [β2-MG had no predicative value.Conclusion MCL has higher invasion and poor prognosis.Rituximab combined chemotherapy could significantly improve CR,PFS and OS rates.
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Mantal cell lymphoma (MCL) is a rare and special type of non-Hodgkin lymphoma.MCL is classified as an aggressive variety of lymphoma and yet it sometimes behaves as an indolent lymphoma.Although the first-line therapy of MCL is highly effective,but it is incurable through conventional chemotherapy.Patients with MCL have a poor prognosis.This review focus on a new therapeutic approach,mammalian target of rapamycin (mTOR) inhibitor.Temsirolimus,a derivation of rapamycin,in MCL cell lines inhibited mTOR.Temsirolimus could inhibits tumor cell proliferation through cell cycle arrest and able to down-regulation of cyclin D1 and Ki-67 in lymphomacell.Clinical trials showed that temsirolimus induced patients with MCL response rate and a prolong progression-free survival.In addition,preclinical studies demonstrated the use of a combination of temsirolimus with monoclonal antibodies or conventional chemotherapy to improve the efficacy of single agent use.Altogether,temsirolimus shows promising results in the therapy of patients with heavily MCL,and especially responses in chemotherapy refractory patients,providing a novel approaches to treatment of MCL.
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Objective To study the biological features and prognostic factors of mantle ceil lymphoma (MCL).Methods The clinical data of 39 cases of MCL were analyzed prospectively.The fellow-up informations were also studied.Age,sex,B symptoms,Ann-Arbor staging,bone marrow and lympho node biopsies were assessed.Serum lactate dehydrogenates(LDH) and β2-microglobulin (β2-MG) level,Cyclin D1 expression and immunophenotype were investigated.Results The median age of patients was 62 years old (range 43-80).The male-to-female ratio was 5.5:1,a clear predominante of male patients.Thirty-five (89.7 ％)cases presented with advanced stage disease (Ann Arbor stage Ⅲ to Ⅳ) at initial diagnosis.CD20 was positive expressed in 39 patients,that all cases expressed B-cell markers.Majority of cases were positive for Cyclin D1 (84.7 ％) and CD5 (64.1％).Conclusion MCL is a special type of B-cell originated non-Hodgkin lymphoma (NHL).The clinical data suggest considerable clinical heterogeneity and it has characteristics of both indolent and aggressive NHL that some patients show a chronic/indolent course,while others have a more fulminant course and short survival.LDH,β2-MG and Cyclin D1 arc the important prognostic factors.