The Clinical-Biological Features and Prognosis of Patients with Mantle Cell Lymphoma / 中国实验血液学杂志

OBJECTIVE@#To analyze the clinical-biological features and outcomes of patients with mantle cell lymphoma (MCL).@*METHODS@#The clinical and laboratory data of 104 patients with newly diagnosed MCL who were admitted to the Department of Hematology, Fujian Medical University Union Hospital from January 2011 to December 2019 were retrospectively analyzed, and the efficacy was observed through survival analysis.@*RESULTS@#Among 104 MCL patients, 88 were male and 16 were female. The median age was 54 (25-79) years old, 93.0% (93/100) of the patients with advanced stage (III and IV stages) and 48.08% (50/104) of the patients with bone marrow infiltration. Patients with Ki-67≥50% had higher WBC counts and LDH levels. Univariate analysis showed that the patients with WBC≥15×109/L, bone marrow involvement, high LDH, high β2-MG levels, Ki-67≥50%, SOX11-, had lower OS and EFS rates (P =0.005, 0.049, 0.033, 0.025, 0.042, 0.018 and 0.001, 0.021, 0.024, 0.035, 0.014, 0.026). The OS rate and EFS rate of patients in R-CHOP and R-Hyper-CVAD treatment groups were significantly higher than those in other treatment groups (P =0.02, 0.002 and P =0.001, 0.001). Patients with autologous stem cell transplantation (ASCT) had higher OS rate and EFS rate (P =0.037, 0.013). Multivariate COX analysis showed that only WBC count, SOX11 expression and whether achieved CR after 4 courses treatment were the independent factors affecting the prognosis.@*CONCLUSION@#MCL mainly occur in elderly men. There are many factors affecting patients' survival, while WBC≥15×109/L, negative expression of SOX11 and failure to achieve CR after 4 courses of treatment are adverse factors for MCL patients.

Clinicopathological and molecular genetic features of cyclin D1-negative mantle cell lymphoma / 中华病理学杂志

Objective: To investigate the clinicopathological features and molecular genetics of cyclin D1-negative mantle cell lymphoma (MCL). Methods: The clinicopathological features and molecular genetics of CyclinD1-negative MCL diagnosed between January 2016 and July 2021 at the First Affiliated Hospital of Zhengzhou University were analyzed using immunohistochemistry and fluorescence in situ hybridization. Clinical information was collected and analyzed. Results: A total of five Cyclin D1-negative MCL cases from all 212 MCL patients (5/212, 2.4%)were included. There were three male and two female patients,age ranged from 59 to 70 years (median 64 years). All patients presented with nodal lesions. None of the patients had B symptoms but four had bone marrow involvement. Histopathologically, four cases were classic MCL and one case was pleomorphic variant type. All five cases were negative for Cyclin D1 but SOX-11 were positive in all cases. CD5 was positive in four cases and one case was weakly positive for CD23. CD10 and bcl-6 were negative in all cases. CCND1 translocation was identified in three cases and CCND2 translocation in one case by FISH analysis. However,CCND3 translocations were not found in the five cases. Conclusions: Cyclin D1-negative MCL are uncommon, its accurate diagnosis needs combined analysis with morphologic and immunophenotypic characteristics and genetic changes. It may be particularly difficult to distinguish from other small cell type B cell lymphomas. FISH analyses for CCND1/CCND2/CCND3 translocations and immunohistochemistry for SOX-11 are helpful to resolve such a difficult distinction.

Application of modified Matutes score containing CD200 in diagnosis of chronic lymphocytic leukemia / 中南大学学报(医学版)

OBJECTIVES@#Immunophenotyping technique is a powerful tool for the diagnosis and differential diagnosis of chronic lymphocytic leukemia (CLL) and other B-cell chronic lymphoproliferative diseases (B-CLPD). CD200 is strongly expressed in CLL. This study aims to analyze the clinical value of modified Matutes score (MMS) containing CD200 in the diagnosis of CLL.@*METHODS@#We retrospectively analyzed 103 B-CLPD patients diagnosed from January 2020 to July 2021, including 64 CLL patients, 11 follicular lymphoma (FL) patients, 14 mantle cell lymphoma (MCL) patients, 6 marginal zone lymphoma (MZL) patients, 1 hairy cell leukemia (HCL) patient, and 7 lymphoplasmic lymphoma/Waldenstrom macroglobulinemia (LPL/WM) patients. The expression of CD markers between the CLL group and the non-CLL group was compared, and the sensitivity, specificity, and clinical consistency of MMS and Royal Marsden Hospital (RMH) immunophenotyping score system were analyzed.@*RESULTS@#There were significant differences in the expressions of CD5, CD23, FMC7, CD22, CD79b, CD200, and sIg between the CLL group and the non-CLL group (χ2 values were 37.42, 54.98, 30.71, 11.67, 55.26, 68.48, and 17.88, respectively, all P<0.01). When the RMH immunophenotyping score≥4, the sensitivity was 79.7%, and the specificity was 100%. When the MMS≥3, the sensitivity was 95.3%, and the specificity was 100%. The Kappa coefficient of RMH immunophenotyping system was 0.677, and the Kappa coefficient of MMS system was 0.860.@*CONCLUSIONS@#The MMS system containing CD200 has better sensitivity and same specificity compared with RMH immunophenotyping system, and MMS system may be more useful in the diagnosis of CLL.

Case report of a metachronous multiple tumor: Mantle cell lymphoma in the orbital region associated with epithelial malignancies at other sites / Tumor múltiplo metacrônico: linfoma do manto na região orbital associado a neoplasias malignas epiteliais em outros sítios - relato de caso

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.

Morphological spectrum of leukemic mantle cell lymphoma.

Background: Leukemic involvement in mantle cell lymphoma (MCL) is common, and can be secondary to nodal or extranodal disease or can be de-novo. There is paucity of literature that describes the morphological spectrum. Aim: This study was aimed at studying the morphological spectrum of leukemic MCL and to correlate the morphology with other features. Materials and Methods: Twenty six such cases diagnosed over a period of four years were studied. Peripheral blood and bone marrow aspiration smears stained with Wrights stain were examined by three hematopathologists. Immunophenotyping was done using multicolor flow cytometry. Fluorescence in situ hybridization (FISH) done in 12 cases showed t(11;14)(q13:q32). Results: Six cases had de-novo leukemic involvement; while 20 cases had secondary involvement. Morphologically, the cells were small (less than twice the size of red blood cell) or large. Small cell morphology in turn showed irregular nuclear border (n=13) or round nuclear contour (n=6). Large cells had blastic morphology (n=5) or had central prominent nucleoli resembling prolymhphocytes (n=2). Twenty cases showed characteristic immunophenotype of CD5+/CD19+/CD20+/FMC7+/CD10-/CD23- and light chain restrictions. Three cases expressed CD23 and two cases were negative for FMC7. Five out of 12 cases, where FISH was done, showed cytogenetic abnormalities in addition to t(11;14)(q13;q32). Conclusion: Morphological spectrum of leukemic MCL ranges from small cells resembling chronic lymphocytic leukemia (CLL) or follicular lymphoma (FL) to large cell mimicking prolymphocytic leukemia (PLL) or acute leukemia. Large cell morphology was associated with more frequent additional cytogenetic abnormality as well as a poorer outcome.

Linfoma de células del manto: manifestación extranodal colorrectal / Mantle-cell lymphoma: extranodal manifestation colorectal

Antecedentes: Los linfomas gastrointestinales agrupan diferentes lesiones y son objeto de permanente revisión. Se los considera de acuerdo a su histología y el estadio evolutivo, hechos que suelen guardar estrecha relación con la clínica, las conductas terapéuticas y el pronóstico. El tubo digestivo está afectado entre un 5 a 15 por ciento en los pacientes que padecen Linfoma no Hodking. El colon y recto son los sitios de menor frecuencia. Representa entre 0,2 a 0,6 por ciento de los tumores malignos colorrectales. El linfoma de células del manto es un subtipo de linfoma de células B, con una frecuencia del 5 al 10 por ciento dentro de los linfomas no Hodking. Objetivo: Presentar el caso de una manifestación colorrectal de linfoma de células del manto, sus características fisiopatológicas y sus alternativas terapéuticas. Pacientes y método: Paciente de 82 años consultó por proctorragia, diarrea crónica, pérdida de peso y mucorrea de más de tres meses de evolución. Se realizó videocolonoscopía constatándose a 10 cm del margen anal lesión mamelonada, friable, ulcerovegetante, extendida hasta sigma con múltiples pólipos sesiles y subpediculados de gran tamaño en cantidad mayor de 100, extendidos en todo el colon. El hallazgo en correlación con la clínica y la endoscopia es compatible con linfoma de células del manto en su forma de afectación extranodal gastrointestinal, y poliposis linfomatosa múltiple. Resultados: Se realizó seis esquemas de quimioterapia bajo la modalidad CHOOP (ciclofosfamida, doxirrubicina, vincristina, prednisona) conjuntamente la aplicación al comienzo de los ciclos con Rituximab (Anticuerpo monoclonal). Se realizó un control endoscópico y tomográfico demostrando remisión completa del compromiso colorrectal, ganglios mesentéricos y retroperitoneales. Se observó persistencia de masas mediastinicas intercavo-aorticas derrame pleural bilateral y dilatación cardiaca...

Background: Gastrointestinal Lymphomas different injuries and are grouped under constant review. They are considered according to their histology and stage of development, events that are closely related to the clinic, the therapeutic behavior and prognosis. The gastrointestinal tract is affected between 5 to 15 per cent in patients with non-Hodgkin lymphoma. The colon and rectum are less frequent sites. It represents between 0.2 and 0.6 per cent of malignant colorectal tumors. The mantle cell lymphoma is a subtype of B-cell lymphoma, with a frequency of 5 to 10 per cent in non-Hodgkin lymphoma. Objective: To present the case of a manifestation colorectal mantle cell lymphoma, pathophysiological characteristics and treatment options. Patients and methods: Patient 82 years consulting with bloody diarrhea, chronic diarrhea, weight loss and mucorrea more than three months of evolution. A colonoscopy was performed, confirmed a 10 cm from the anal injury swellings, extended to multi-sigma subpediculados sessile polyps and large in quantity greater than 100, spread throughout the colon. The finding in correlation with clinical and endoscopy is compatible with mantle cell lymphoma, as extranodal gastrointestinal involvement and multiple lymphomatous polyposis. Results: we performed six schemes in the form CHOOP chemotherapy (cyclophosphamide, doxirrubicina, vincristine, prednisone) combined the application at the beginning of the cycles with Rituximab (monoclonal antibody). We undertook a endoscopy and CT demonstrated complete remission of colorectal disease, commitment, mesenteric and retroperitoneal lymph nodes. Showed persistence of mediastinal masses, bilateral pleural effusion and cardiac dilatation. Today is free from colorectal disease and active monitoring by their serial mediastinal and their heart failure...

Immunohistochemical analysis for CD21, CD35, Caldesmon and S100 protein on dendritic cells types in oral lymphomas

OBJECTIVE: Follicular dendritic cells (FDCs) and interdigitating dendritic cells (IDCs) are dendritic cells found in lymphoid follicles, reactive follicles and in lymphomas. The goal of this study was to evaluate the presence and distribution of FDCs and IDCs in oral lymphomas. MATERIAL AND METHODS: Immunohistochemistry reactions were applied to 50 oral lymphomas using the antibodies anti-CD21, anti-CD35 and anti-caldesmon to FDCs, and anti-S100 protein to IDCs. Caldesmon+/FDCs and S100+/IDCs were quantified in Imagelab® software. RESULTS: FDCs revealed by CD21 and CD35 were positively stained in two cases of diffuse large B-cell lymphoma, one MALT lymphoma, and in one case of mantle cell lymphoma. FDCs were immunopositive to caldesmon in all cases, as well as IDCs to S100 protein. Burkitt lymphoma presented a lower amount of caldesmon+/FDCs and S100+/IDCs than diffuse large B-cell lymphoma and plasmablastic lymphoma of the oral mucosa type. CONCLUSIONS: The microenvironment determined by neoplastic lymphoid cells in oral lymphomas is responsible by the development and expression of dendritic cells types.

Fatal disseminated mucormycosis in a patient with mantle cell non-hodgkin's lymphoma: an autopsy case

A patient with mantle cell non-Hodgkin's lymphoma presented herself with fever, nausea, right upper quadrant pain on the 7th day of R-CHOP chemotherapy. After hospitalization with the suspicion of acute cholecystitis, she received antibiotherapy with G-CSF because of emerging neutropenia at the 10th day of chemotherapy. Abdominal computed tomography revealed small infarcts in the spleen and kidneys. The echymotic lesion which developed on her right lateral malleolus, became bullous in the following days and treated as ecthyma gangrenosum. Altough the patient was afebrile with a normal neutrophil count on the third day of antibiotherapy, she developed acute renal failure and deteriorated rapidly. The patient underwent hemodialysis but expired on the 10th day of hospitalization. Post mortem autopsy findings showed ischemic infarction and necrosis of parenchyma due to mycotic thrombosis of arteries and veins of many organs (heart, lung, diaphgram, kidneys, spleen, gut mucosa) as well as invasion of vessel walls and parenchyma by mucor. We reviewed mucormycosis in the light of this case.

Linfoma de células da zona do manto em anexos oculares: relato de caso / Mantle-cell lymphoma of the ocular adnexa: a case report

Os tumores linfóides dos anexos oculares são neoplasias de crescimento lento que acometem principalmente idosos. O objetivo deste trabalho é relatar um caso de linfoma não-Hodgkin de células da zona do manto, um subtipo raro de linfoma de células B nos anexos oculares. RELATO DO CASO: Paciente masculino de 62 anos com tumoração em pálpebras superiores, região maxilar e cavidade oral, tendo o diagnóstico inicial de doença de Mikulics, evoluiu com acentuado aumento das lesões, apesar da terapêutica imunossupressora. Biopsias da pálpebra superior esquerda e medula óssea revelaram linfoma não-Hodgkin de células B da zona do manto. Apesar da disseminação (estadiamento grau IV), quimioterapia e transplante de medula óssea conduziram à remissão da doença. COMENTARIOS CONCLUSIVOS: Apesar da disponibilidade de avançados métodos diagnósticos complementares como a imunofenotipagem e a análise genética molecular, o linfoma representa, para clínicos e patologistas, um desafio quanto ao diagnóstico e ao prognóstico.

Multiple intestinal lymphomatous polyposis in a Jindo dog

A male, 5-year-old Jindo dog underwent enterectomy and enteroanastomosis due to ileus of the intestine at a local veterinary hospital. Grossly, the excised intestine showed markedly thickened multinodular masses in the serosal layer of the upper part, and soft-to-firm, creamcolored neoplastic masses that displayed extensive nodular mucosal protuberances into the lumen. The neoplastic masses were filled with large round cells that were ovoid in shape and they had pale and/or hyperchromatic nuclei. The neoplastic cells had mainly infiltrated into the mucosal and submucosal layers, and they had diffusely invaded the muscular and serosal layers. Therefore, the diagnosis of canine multiple intestinal malignant lymphomatous polyposis was made based on the gross and histopathological findings. The origin of these tumor cells was determined to be B-cells since they were positive for anti-CD20.

Linfoma del manto vs. leucemia linfatica crónica atipica: utilización de inmunohistoquímica, citometría de flujo y biología molecular para su correcta tipificación / Mantle cell lymphoma vs. atypical chronic lymphocytic leukemia: use of immunohistochemistry, flow cytometry and molecular biology for their adequate typing

The differential diagnosis of certain B CD5+ lymphoproliferative processes, such as mantle cell lymphoma (MCL) and atypical chronic lymphocytic leukemia (ACLL), is difficult. The aim of this study was to correlate morphological findings, cyclin D1 (cD1) detection by immunohistochemistry (IHC) and immunophenotype by flow cytometry (FC) with the results obtained by molecular biology in this type of neoplasias. We analyzed 20 samples classified as B CD5+ lymphoproliferative processes by FC. PCR was used for t(11;14) bcl-1/IgH determination. Histopathological and IHC studies for cD1 were done in 14 cases. Twelve cases were diagnosed as MCL, with positive cD1 in 5 (5/9), five as ACLL and three as B lymphoproliferative process. PCR revealed t(11;14) in 6/12 MCL and negative results in the other groups (0/8). Molecular biology evidenced translocation in 4/5 MCL positive for cD1 with IHC. The presence of translocation could be demonstrated by IHC and PCR in 7/12 MCL: 4 with both techniques, 2 with PCR alone, and 1 with IHC alone. These findings show a significant association between cD1 by IHC and bcl-1/ IgH gene detection by PCR, which implies that both techniques are complementary for MCL typing.

Algunos procesos linfoproliferativos B CD5+ son de difícil diagnóstico diferencial como es el casodel linfoma del manto (LM) y la leucemia linfocítica crónica atípica (LLCA). El motivo del presenteestudio fue correlacionar los hallazgos morfológicos, la detección de ciclina D1 (cD1) por inmunohistoquímica(IHQ) y el inmunofenotipo por citometría de flujo (CF) con los resultados obtenidos por biología molecular en este tipo de neoplasias. Se estudiaron 20 muestras clasificadas como procesos linfoproliferativos B CD5+ por CF. Se realizó la determinación de t(11;14) bcl-1/IgH por PCR. El estudio histopatológico e IHQ para cD1 se efectuó en 14 casos. Doce casos fueron diagnosticados como LM, con cD1 positiva en 5 (5/9); cinco como LLCA y tres como proceso linfoproliferativo B. Con PCR se observó t(11;14) en 6/12 LM y negatividad en losrestantes grupos (0/8). Se pudo demostrar la presencia de traslocación en 7/12 LM mediante IHQ Y PCR: 4con ambas técnicas, 2 con PCR exclusivamente y 1 con IHQ, evidenciando una alta asociación entre cD1 por IHQ y la detección del gen bcl-1/IgH por PCR, ambas técnicas complementarias en la tipificación de LM.

Multiple lymphomatous polyposis of the gastrointestinal tract

CONTEXTO: A polipose linfomatosa múltipla gastrintestinal é tipo raro de linfoma maligno de comportamento biológico agressivo, com disseminação sistêmica precoce e prognóstico sombrio. A polipose linfomatosa múltipla gastrintestinal é considerada manifestação do linfoma não-Hodgkin e representa a contraparte gastrintestinal do linfoma nodal das células do manto. OBJETIVO: O presente estudo descreve caso de polipose linfomatosa múltipla gastrintestinal e discute os aspectos anatomopatológicos, clínicos, diagnósticos e terapêuticos desta inusitada neoplasia. RELATO DE CASO: Homem de 59 anos, branco, apresentou-se ao nosso serviço com queixa de astenia, suores noturnos, alteração do hábito intestinal e emagrecimento há dois meses. O exame físico mostrou mucosa descorada e massa palpável no epigástrio e mesogástrio. A endoscopia digestiva alta revelou pólipos gástricos e duodenais. O enema opaco mostrou lesões polipóides múltiplas, principalmente no ceco. A biópsia retal revelou infiltração da mucosa e submucosa por linfoma difuso de células pequenas clivadas. O estudo imuno-histoquímico mostrou linfócitos que expressavam o anticorpo CD20 (L26) e imunoglobulina de cadeia leve kappa (k), porém não expressava imunoglobulina de cadeia leve lambda (l). O doente apresentou abdome agudo obstrutivo devido a massa mesentérica formada por linfonodos aglutinados ao redor do íleo proximal e obstruindo seu lúmen. O enfermo foi submetido a enterectomia segmentar e gastrotomia com biópsias excisionais das lesões polipóides gástricas. Após dois ciclos de quimioterapia, houve piora do estado geral, com aumento das dimensões das massas abdominais e sepse, acompanhada por insuficiência respiratória progressiva e óbito.

Intracavitary metastatic non-Hodgkin's lymphoma simulating an atrial myxoma.

We describe a case of intracavitary metastasis of Mantle Cell Lymphoma of gastrointestinal tract, which mimicked an atrial myxoma. The gastrointestinal lymphoma led to widespread metastasis and a large metastatic tumor deposit at the hilum of the right lung caught up the hilar vessels. This led to infiltration into the pulmonary veins and intracavitary metastatis to the left side of the heart.

Imaging Findings of Mantle Cell Lymphoma Involving Gastrointestinal Tract

Clinical manifestations and imaging findings of mantle cell lymphoma involving gastrointestinal tract were evaluated. The subjects were 7 cases of mantle cell lymphoma involving the gastrointestinal tract. All cases were pathologically confirmed in our hospital during the period from April 1994 to July 2000. Five patients were male and 2 were female, and their age ranged from 49 to 63 years (average 57.4). The objectives were: 1) characteristics and distribution of multiple polyposis, 2) presence, location and enhancement pattern of bowel wall thickening or mass formation, 3) presence of splenomegaly, 4) presence and location of abdominal lymph node enlargement, 5) involved extra-abdominal organs, 6) combined cancer and location, and 7) other findings. All mantle cell lymphomas occurred in elderly persons, over 40 years, and most showed multiple polyposis (6/7), bowel wall thickening or mass formation (6/7), lymph node enlargements (6/7) and extra- abdominal involvement (5/7). All cases of polyposis involved the small bowel and colon, and the size of the polyps ranged from 0.1-4.0cm. Four of 6 patients showed combined sessile and polypoid polyps, while the other 2 showed only sessile polyps. Most of or some of the polyps in 3 patients showed small central ulcerations. Most of the patients (5/6) showed an uncountable number of polyps. Polyposis was predominant in the rectum, ascending colon, rather than other sections in the colon, and the ileum were almost always involved by polyposis. Bowel wall thickening or mass formation developed exclusively in the ascending colon, rectum or ileum. Extra- abdominal involvement developed either simultaneously or nonconcurrently with gastrointestinal involvement. Some of patients showed splenomegaly (3/7), appendiceal enlargement (2/7), and intussusception (1/7), and some had associated adenocarcinomas (3/7).

Mantle cell lymphoma presenting as solitary polypoid colonic lesions.

Mantle cell lymphoma of the intestine is rare, usually presenting as multiple small polyps. We report three men with colonic mantle cell lymphoma in the form of single large polypoid mass. The clinical picture suggested adenocarcinoma; the diagnosis was made at histology and immunohistochemistry of the colectomy specimens.