Involvement Of Mantle Cell Lymphoma Research Articles

Extranodal Mantle Cell Lymphoma Presented as Bilateral Adrenal Masses on 18 F-FDG PET/CT and 68 Ga-DOTATATE PET/CT.

Mantle cell lymphoma (MCL) is a rare type of B-cell non-Hodgkin lymphoma that commonly presents with constitutional symptoms, lymphadenopathy, and laboratory abnormalities. Although less common, extranodal involvement of MCL may be seen at initial presentation and most commonly occurs in the spleen, liver, bone marrow, and gastrointestinal tract. Adrenal involvement in MCL is extremely rare, and only a few cases have been reported in the literature. Herein, we present 18 F-FDG PET/CT and 68 Ga-DOTATATE PET/CT findings of a unique case of primary extranodal MCL with gastric and adrenal involvement.

Clinical efficacy and safety of chimeric antigen receptor T-cell therapy for mantle cell lymphoma with secondary central nervous system involvement.

Data describing outcomes of chimeric antigen receptor (CAR) T-cell therapy in patients with secondary central nervous system (SCNS) involvement of mantle cell lymphoma (MCL) are limited. We identified 10 patients with MCL and SCNS involvement treated with anti-CD19 CAR T-cell therapy at three US academic centres. Frequent objective responses were observed in the CNS (86%) and systemically (90%), and the 1-year progression-free survival was 47%. Seven patients developed immune-effector-cell-associated-neurotoxicity-syndrome (n = 2 Grade 1, n = 5 Grade 3). Our results suggest that anti-CD19 CAR T-cell therapy in this setting is feasible and additional data regarding neurotoxicity in this population may be warranted.

17th International Conference on Malignant Lymphoma, Palazzo dei Congressi, Lugano, Switzerland, 13 - 17 June, 2023.

Introduction: The ataxia–telangiectasia mutated (ATM) gene plays an important role in the cellular response to DNA damage. Whether different types of ATM aberrations (mutation and/or deletion of (del)11q) differentially affect survival in mantle cell lymphoma (MCL) has not yet been reported. Methods: We analyzed 160 consecutive MCL patients (pts) treated in two Czech university centers (Prague, Olomouc) from 11/2006 to 12/2021. Both fluorescence in situ hybridization (FISH) and next generation sequencing (NGS, Illumina), were performed in all pts to identify ATM and TP53 gene aberrations on tumoral tissue (peripheral blood, bone marrow); the cutoff value for ATM and TP53 mutation was 3% variant allele frequency. Peripheral blood and/or bone marrow had at least 5% MCL involvement as analyzed by flow cytometry. Variables were compared by chi-squared test. Kaplan-Meier method was used to calculate the probabilities of progression and overall survival (PFS, OS); the log rank test was used for univariate comparisons of survival curves. PFS and OS were calculated from the date of diagnosis. Results: The median age at diagnosis was 67 (30-87) years. All pts had advanced disease (IV). MIPI score was low, intermediate and high in 15.6%, 26.3% and 58.1% pts, respectively. Treatment approaches used were as follows: watch and wait in 5.0%, R-CHOP/R-CHOP-like in 50.6%, intensive R-HDAC-containing in 34.4% and non-anthracycline/palliative regimen in 10.0% of pts. Complete and partial response was achieved in 56.3% and 21.9% of pts, respectively. Stable and progressive disease was observed in 13.1% of pts. Almost 29% of pts underwent autologous stem cell transplant. Sixty percent of pts received rituximab maintenance. Overall, 71 (44.4%) and 68 (42.5%) pts had ATM and TP53 aberration(s) (del/mut/del+mut), respectively. Of these, 20 (12.5%) pts had aberrations in both genes. Disruption of ATM gene was related to B-symptoms (p≤0.005). Age, sex, ECOG PS, cytomorphology, Ki67 index, MIPI and treatment response did not correlate with ATM aberration. With a median follow-up of 3.8 years, 2-year PFS and 2-year OS in all pts was 58.1% and 74.6%, respectively. Median PFS in pts with ATM mutation, ATM deletion and ATM+TP53 aberration was not reached, 3.3 and 0.7 years, respectively (p ≤ 0.005). Median OS in pts with ATM mutation, ATM deletion and ATM+TP53 aberration was not reached, 5.6 and 0.9 years, respectively (p ≤ 0.005). No significant difference in OS was found between ATM mutated and wild-type pts. (Survival graph below) Keywords: aggressive B-cell non-Hodgkin lymphoma, diagnostic and prognostic biomarkers, indolent non-Hodgkin lymphoma The research was funded by: IGA_LF_2023_005, MH CZ DRO-VFN64165, IGA_LF_2023_010, MH_CZ-DRO (FNOL, 00098892) No conflicts of interests pertinent to the abstract.

Endoscopic diagnosis of a colonic localisation of a mantle cell lymphoma.

Extra-nodal localisations of mantle cell lymphomas are most frequently found in the gastrointestinal tract. It is therefore important for an endoscopist to be familiar with the endoscopic image of a mantle cell lymphoma. In this case series of three patients with colonic involvement of mantle cell lymphoma, we discuss the endoscopic diagnosis.

Aggressive Mantle Cell Lymphoma with Neurolymphomatosis and Cutaneous Infiltration: First Case Report and Review of Literature

Mantle cell lymphoma accounts for 5-10% of cases of non-Hodgkin's lymphoma. Neurolymphomatosis(NL) and Cutaneous involvement of mantle cell lymphoma has been rarely described. When present, they are indicators of high-grade disease and poor prognosis. We present the first reported case of Neurolymphomatosis and concurrent cutaneous mantle cell lymphoma. A 73-year-old man with a recent diagnosis of mantle cell lymphoma presented with progressive weakness of the upper and lower extremities that began one month after his first round of chemotherapy. The Dermatologic exam revealed red-violaceous, edematous papules and plaques on the head, trunk, and upper extremities. Prior work-up including MRI brain and cervical spine, lumbar puncture and cytology were unremarkable. He received treatment with dexamethasone and PLEX with no improvement. MRI lumbar spine revealed abnormal linear and nodular contrast enhancement in the dural sac along the lumbosacral nerves with associated soft tissue thickening in epidural spaces, consistent with NL. Skin biopsy showed medium to large cells with atypical nuclei throughout the dermis. Immunohistochemical stains for CD20, CD5, and cyclin D1 were positive, while CD3 and CD23 were negative. These findings were consistent with cutaneous mantle cell lymphoma. MR-CHOP therapy was promptly initiated however the patient developed pancytopenia with worsening swallowing dysfunction and died of aspiration pneumonia and sepsis. We identified 13 cases of Mantle cell Neurolymphomatosis in the literature, including our case. Mean age on presentation was 57.5 with male predominance. Six cases had neuronal involvement on primary presentation. Most common symptom was painful sensorimotor neuropathy. Cranial nerve involvement was seen in four cases and leptomeningeal involvement was seen in two of the cases. Diagnosis was established with MRI in eight cases with 100% positivity. Seven cases had PET-CT on presentation, of which five were positive. Most cases were treated with systemic chemotherapy with R-CHOP and high dose Methotrexate. One case received stem cell transplant which was followed by relapse. One case reported successful treatment with CAR-T brexu-cel infusion with no evidence of relapse on 2-month follow up. Most patients died of progression of disease although survival data was not available for all cases. Rapid, aggressive infiltration of both the nerve roots and skin by mantle cell lymphoma represents unusually high-grade disease. This case highlights the importance of clinical suspicion of NL in any subtype of lymphoma showing aggressive features. There is a narrow window for treatment to prevent fatal outcomes. MRI is the diagnostic modality of choice, which was instrumental in establishing diagnosis in our case and further workup was not felt necessary. Novel CART-cell therapy and consolidative therapies such as high dose chemotherapy followed by stem cell transplant (HDC- ASCT) should be considered in appropriate cases, as we intended in our case after disease stabilization. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

Cutaneous Involvement of Mantle Cell Lymphoma: Report of Two Cases with Dermatoscopic Features.

Cutaneous Involvement of Mantle Cell Lymphoma: Report of Two Cases with Dermatoscopic Features.

Mantle cell lymphoma with gastrointestinal involvement and the role of endoscopic examinations.

Studies on gastrointestinal (GI) tract involvement in mantle cell lymphoma (MCL) are lacking. We investigated the clinical characteristics and prognosis of MCL with GI tract involvement. We retrospectively analyzed 64 patients diagnosed with MCL from January 2009 to April 2017. At the time of MCL diagnosis, patients who were identified to have GI involvement by endoscopic or radiologic examination were assigned to the GI-MCL group. The other patients were assigned to the non GI-MCL group. The GI-MCL group included 28 patients (43.8%). The most common endoscopic finding of MCL was lymphomatous polyposis (20/28, 71.4%). The GI-MCL group had higher stage and International Prognostic Index status (P = 0.012 and P = 0.003, respectively). Among the total 51 GI lesions in the GI-MCL group, 31.4% (16/51) were detected only by endoscopic examinations and were not detected on CT or PET-CT. The cumulative incidence of recurrence was higher in the GI-MCL group compared with the non GI-MCL group but the difference was not statistically significant (P = 0.082). Stage (HR 1.994, 95% CI 1.007-3.948) and auto PBSCT (HR 0.133, 95% CI 0.041-0.437) were identified as independent predictive factors for recurrence. Recurrences at GI tract were identified in 59.1% (13/22) and 11.1% (2/18) of the GI-MCL and non GI-MCL group, respectively. Among 15 GI tract recurrences, five recurrences were detected only with endoscopic examinations. Endoscopy can reveal the GI involvement of MCL that is not visualized by radiological imaging. Endoscopic examinations are recommended during staging workup and the follow-up period of MCL patients.

1817 Blastic Mantle Cell Lymphoma of the Gastroesophageal Junction: A Unique Presentation

INTRODUCTION: Though vast, the medical literature is deficient on reports of gastroesophageal junction (GEJ) involvement of mantle cell lymphoma (MCL). We present a unique case of a 76-year-old male who was found to have a blastic variant of MCL in the GEJ. This case provides additional distinct features to the various clinical presentations associated with this rare proliferative disorder, thereby enhancing the medical literature on MCL. CASE DESCRIPTION/METHODS: A 76-year-old male with a history of prostate and bladder cancer presented with worsening dysphagia, weight loss, and heartburn for over 6 months. He had extensive workup in the past for an ulcerative, gastric/GEJ lesion, found on 4 separate esophagogastroduodenoscopies (EGD) and 2 esophageal ultrasounds (EUS), whose biopsies ranged from mild gastritis to intestinal metaplasia, but were repeatedly negative for malignancy. Physical exam and labs were unremarkable except for anemia. Computerized tomography (CT) scan showed an irregular mass extending from the proximal fundus to the GEJ, and diffuse lymphadenopathy (LAD). EGD on admission showed a large friable, ulcerated lesion with heaped up margins involving the cardia, with extension into the GEJ. GEJ biopsies showed histological and immunohistochemical (IHC) findings consistent with blastic MCL. Further workup of a positron emission tomography (PET) scan showed a fluorodeoxyglucose (FDG) avid mass involving the GEJ and stomach as well as FDG avid regional LAD. A bone marrow biopsy showed minimal involvement (<5%) of CD5+/CD23+ B cells and was negative for both Cyclin D1 and t (11;14). He was started on a chemoimmunotherapy regimen (CITR) with a good response and is currently progression free after 2 years. DISCUSSION: The EGD biopsy was diffusely infiltrated by atypical lymphocytes with prominent nucleoli and IHC stains positive for CD-20, Cyclin D1, BCL-2, and BCL-6, and a Ki-67 proliferative index >90%; all consistent with blastic MCL; a rare and aggressive subtype of MCL. He was not a candidate for stem cell transplant due to his age/comorbidities, so he was started on guideline-based CITR of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) followed by rituximab maintenance therapy. PET/EGD done 1 year later showed improvement of the LAD and GEJ mass, with negative biopsies for lymphoma. To the best of our knowledge, there have been no reported cases of GEJ involvement of blastic MCL appearing as a large friable lesion, illustrating the unique aspect of this case.

Analysis of Clinical Characteristics and Prognosis of Mantle Cell Lymphoma With Gastrointestinal Involvement

Introduction: Mantle cell lymphoma (MCL) is an aggressive B-cell lymphoma characterized by rapid disease progression. Studies on gastrointestinal (GI) involvement of MCL are lacking. In this study, we investigated the clinical features and prognosis of MCL with GI involvement. Methods: We retrospectively analyzed 64 patients who diagnosed MCL from January 2009 to April 2017 consecutively. At the time of MCL diagnosis, patients who were identified to have GI involvement by endoscopy or radiologic examination were assigned to the GI-MCL group. And those who were confirmed to have no GI involvement were assigned to the non GI-MCL group. The clinical characteristics and the outcomes of both groups were compared. Results: The GI-MCL group included 29 patients (45.3%). There were no significant differences in age and gender between the GI and non GI-MCL group. The stage of GI-MCL group was higher compared to the non GI-MCL group and all of 29 patients were stage IV. The mean of International Prognostic Index (IPI) score was also significantly higher in GI-MCL (2.97 vs. 1.97, p=0.047). Bone marrow involvement in the GI-MCL group was much greater than non GI-MCL group (69.0% vs. 40.0%, p=0.021). R-CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone combined with rituximab) was the most abundant in 1st line chemotherapy (44/64, 69.8%). After 1st line chemotherapy, 56 of 64 patients showed complete remission (87.5%). Although there was no difference in complete remission rate between the two groups, recurrence rate was much higher in GI-MCL group (72.% vs. 41.2%, p=0.024). A total of 15 patients received bone marrow transplantation (15/64, 23.4%). The median follow up period was 37 months and 11 out of 64 patients died during this period (17.2%). There was no significant difference in mortality between the two groups. Conclusion: The patients with MCL invading GI tract tended to have advanced stage, higher IPI score, more bone marrow involvement and more recurrence than MCL not involving the GI tract. Endoscopic examination to rule out GI involvement in patients with MCL is warranted.

Recurrent Mantle Cell Lymphoma Presenting as Colonic Mass

Mantle cell lymphoma (MCL) is a rare and aggressive subtype of B cell Non-Hodgkin's Lymphoma. MCL frequently involves extranodal sites and gastrointestinal tract is involved in 80% of cases. MCL is usually resistant to standard chemotherapy regimens and associated with high recurrence rate. Recurrent disease is very aggressive and resistant to treatment. We present a case of recurrent MCL involving the colon despite intensive chemotherapy. A 58 year old woman presented to our hospital with complaints of left sided abdominal pain and hematochezia since one day. Abdominal pain was described as severe and continuous with no radiation. She was diagnosed with MCL two years ago when she had axillary lymph node biopsy. She was treated with standard chemotherapy regimen and stem cell transplantation at that time. She had a relapse one year after initial treatment for which she was again treated with chemotherapy. On examination, no lymphadenopathy was noted. Abdominal examination was significant for left lower quadrant tenderness without guarding, rigidity or hepatosplenomegaly. Laboratory tests were normal. Computed tomography scan of abdomen showed ascending colon wall thickening. Colonoscopy showed mass like lesion in the ascending colon and polyps in the ascending colon as well as splenic flexure. The biopsy of all the three lesions showed atypical lymphocytes infiltrating the lamina propria. The tumor cells were positive for cyclin-D1, CD 5, CD 20, CD 43 and CD 79A consistent with the diagnosis of MCL. Patients with MCL and gastrointestinal involvement could be asymptomatic or may present with abdominal pain, obstruction, diarrhea, or hematochezia. The most common site of involvement in the GI tract is ileocecal region, but any other area may be involved from the stomach to the rectum. Involvement of the gastrointestinal tract may occur with or without the appearance of polyps. Even though there are no standard guidelines for endoscopic screening of gastrointestinal tract in asymptomatic patients, one should be aware of involvement of gastrointestinal tract in the early course of disease or recurrent disease. Although mantle cell lymphoma is initially responsive to chemotherapy, it eventually becomes refractory with median survival of 3 to 5 years. Patients with obstructive tumor masses require surgical therapy, but the mainstay of treatment is chemotherapy.Figure: A polypoid non-obstructing medium-sized mass. Polyp with normal looking mucosa was found at the splenic flexure.Figure: A frond-like/villous, fungating, infiltrative, polypoid and ulcerated nonobstructing large mass was found in the ascending colon.

Perirenal Involvement of Mantle Cell Lymphoma: Imaging Features

Perirenal lymphoma is a rare disease and accounts for less than 10% of all malignant lymphomas. Mantle cell lymphoma (MCL) is the rarest but 1 of the most aggressive non-Hodgkin's lymphoma subtype. The perirenal involvement of MCL has not been reported previously. A 69-year-old male, who had been diagnosed as having MCL 1 year ago, presented with recent-onset right back pain. Herein we present the key imaging findings of perirenal soft tissue manifestation of MCL.

Prognostic relevance of the Ki-67 proliferation index in patients with mantle cell lymphoma.

BackgroundA high Ki-67 proliferation index (PI) in neoplastic cells is associated with poor survival in mantle cell lymphoma (MCL). We aimed to determine the cut-off values for the Ki-67 PI as a prognostic factor in MCL according to bone marrow findings.MethodsImmunohistochemical (IHC) staining for Ki-67 was performed on formalin-fixed paraffin-embedded biopsy tissues from 56 patients with MCL. Patients were grouped based on their Ki-67 PI values. Survival analyses were carried out and the cut-off value for the Ki-67 PI was determined.ResultsOf the 56 patients, 39 (69.6%) showed bone marrow involvement of MCL; 21 of these patients had leukemic manifestations at the time of diagnosis. The results of the Ki-67 IHC staining were as follows: ≤10% in 22 patients, 11–20% in 14 patients, 21–30% in 3 patients, 31–40% in 4 patients, 41–50% in 4 patients, and >50% in 9 patients. A cut-off value of 20% revealed significantly different survival rates with mean survival times of 69.8 months (Ki-67 PI≤20%) and 47.9 months (Ki-67 PI>20%), irrespective of bone marrow findings (P=0.034). Clinical outcomes did not differ, regardless of bone marrow findings. However, in cases with bone marrow involvement, the Ki-67 cut-off value of 30% for overall survival was required to yield statistical significance (P=0.033).ConclusionThe 20% cut-off value for the Ki-67 PI was clinically meaningful, regardless of bone marrow involvement of MCL. For patients with bone marrow involvement, the statistically significant cut-off value increased to 30%.

Interim Analysis of a Phase 2 Study of Bortezomib Plus Rituximab Maintenance Therapy in Patients with Mantle Cell Lymphoma Status Post Autologous Stem Cell Transplantation

Interim Analysis of a Phase 2 Study of Bortezomib Plus Rituximab Maintenance Therapy in Patients with Mantle Cell Lymphoma Status Post Autologous Stem Cell Transplantation

Disseminated Peritoneal Mantle Cell Lymphoma Mimicking Peritonitis

Mantle cell lymphoma (MCL) is an aggressive subtype of B-cell lymphoma that represents 7% of non-Hodgkin cases, composed of CD5-positive naive B-cell of mantle zone surrounding germinal center follicles. MCL is clinically challenging to manage, with an ominous prognosis. MCL involves lymph nodes, spleen, bone marrow, and gastrointestinal tract organs. MCL involvement of peritoneal …

Skin Involvement of Mantle Cell Lymphoma May Mimic Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg Type.

Mantle cell lymphoma (MCL) is a B-cell neoplasm with a variable and generally aggressive clinical course. So far our knowledge of skin involvement of MCL is limited. To understand the clinical and histopathologic features of MCL with skin involvement, the files of the Lymph Node Registry Kiel were screened for MCL diagnosed in the skin. Over a period of 13 years, 1321 biopsy specimens were diagnosed as MCL; among them, 14 patients (1%) showed skin involvement. Of these, skin was the initial site of manifestation in 6/11 (55%) cases. One patient presented with a skin-limited lymphoma. Furthermore, 7/12 (58%) patients presented with lesions on the leg. The lymphomas were highly proliferative with blastoid cytology in 12/14 (86%) cases. Moreover, the immunophenotype with expression of BCL2 (100%), MUM-1/IRF4 (83%), and IgM (82%) and lack of CD10 (25%) and BCL6 (0%) closely resembled the features of primary cutaneous diffuse large B-cell lymphoma, leg type. Solely the expression of cyclin D1 (100%) and the presence of t(11;14) (100%) allowed a distinction from cases of primary cutaneous diffuse large B-cell lymphoma, leg type. Only 2 MCL cases with skin involvement presented with classical cytology. Interestingly, in these 2 cases skin involvement occurred simultaneously in a lesion of coexisting primary cutaneous marginal zone lymphoma. Our data suggest that clinical presentation on the leg and blastoid cytology along with high proliferation and expression of Bcl2, Mum-1/IRF4, and IgM are typical for MCL involving the skin. Lymphomas with these features might be erroneously diagnosed as diffuse large B-cell lymphoma, leg type, if cyclin D1 staining is not performed.

MDCT of extranodal mantle cell lymphoma: a single institute experience.

To study the pattern of extranodal and particularly gastrointestinal (GI) involvement of mantle cell lymphoma (MCL) on MDCT MATERIALS AND METHODS: In this IRB-approved, HIPAA compliant retrospective study, MDCT features of 78 patients (62 males and 16 females, mean age 57 years) with MCL including 28 patients at presentation were reviewed. Clinical and histopathological (blastoid vs. non-blastoid) data were noted from medical records. Extranodal involvement was present in overall 51/78 patients on CT (65%), 18/28 (64%) patients at presentation. Spleen (21/78-27%) and bowel (19/78-24%) were the most common sites of extranodal involvement by MCL on imaging, followed by lungs (10/78-13%) and skin/subcutaneous tissue (9/78-12%). Bowel involvement was either in the form of endophytic polypoidal lesions (n = 11, mean size 3.1 cm), as focal mild bowel wall thickening (n = 5, mean thickness 1.4 cm), or as combination of the two (n = 3). Blastoid histology was present in 14/78 (24%) patients and was statistically associated with skin/subcutaneous involvement (p < 0.05; Fisher's exact t test). Median follow-up was 72 months during which 21 patients died with median survival of 48 months (26 months for blastoid histology vs. 47 months for non-blastoid histology). There was no statistical correlation between sites of involvement and survival. MCL has a predilection for extranodal disease, predominantly involving the spleen, bowel, lungs, and subcutaneous tissue. GI involvement on CT is in the form of endoluminal polypoidal lesions and mild bowel wall thickening. Skin/subcutaneous involvement was statistically more common with blastoid histology in our study.

An Unusual Case of Mantle Cell Lymphoma Involving the GI Tract

Introduction: Mantle cell lymphoma is an aggressive form of non-Hodgkin’s lymphoma. Involvment of MCL in the gastrointestinal tract (GI) has been reported to occur in 15-30% of patients. MCL can involve any region of the GI tract and typically presents as lymphomatous polyposis representing 2% of GI tract lymphomas. We present an interesting case of MCL involving the GI tract. Case Report: A 69-year-old female initially presented with an abnormal serum protein electrophoresis (SPEP) in 2008 and underwent further evaluation. She had a biclonal gammopathy with IgM (monoclonal) and IgG (polyclonal) disease. Bone marrow examination revealed lymphoplasmocytic lymphoma (20-25%) as well as another clonal population (5%) consistent with mantle cell lymphoma. She was treated with fludarabine and rituximab and post-treatment bone marrow biopsy only showed hypocellular marrow, consistent with treatment effect and complete remission. Five years later she developed newonset watery diarrhea and a 30-lb weight loss, prompting an EGD and colonoscopy. Linear ulcerations were seen in the antrum and pyloric channel. There was erythema and loss of vascularity throughout the colon with some areas of normal intervening mucosa, and multiple biopsies were obtained. Biopsy results showed patchy dense lymphoid infiltrate and immunostains revealed CD20, Cyclin D1, CD5, and CD43 positivity, consistent with mantle cell lymphoma. A follow-up bone marrow biopsy revealed mantle cell lymphoma occupying 70% of marrow cellularity. Treatment was then initiated with bendamustine and rituximab. Conclusion: Unusual features of our case include pan-gastrointestinal infiltration of lymphoma as well as the lack of endoscopic polyposis, which is typical in GI involvement of mantle cell lymphoma. Additionally, the patient’s mantle cell lymphoma represented a minority clonal population, but the recurrent disease occupied the majority of her marrow cellularity. Recent studies have shown a higher frequency of mantle cell lymphoma involving the GI tract than previously reported. In a prospective study, Romageura et al identified mantle cell lymphoma in the lower GI tract in 88% of patients and in the upper GI tract in 43% of patients.[1] Only 26% of patients initially presented with GI symptoms. National Comprehensive Cancer Network (NCCN) guidelines suggest that upper endoscopy and colonoscopy may be useful in certain circumstances in patients diagnosed with mantle cell lymphoma and is necessary for confirmation of stage I-II disease as well as response assessment to initial therapy.[2]

Clinicopathological studies on bone marrow involvement of mantle cell lymphoma

Mantle cell lymphoma is a rare lymphoma and involvement of bone marrow is more rare. Here, we concluded the features, diagnosis and other points of the involvement to illustrate the diagnostic main point of mantle cell lymphoma on pathology. Objective To explore clinicopathological features, diagnosis and differential diagnosis of bone marrow involvement of mantle cell lymphoma (MCL). Methods 55bone marrowinvolvementofMCLwith bone marrow biopsy from 2006to2012 were analyzed retrospectively.There were 15 patients with lymph node specimens and available 8 spleen specimens in the 55 patients. We found some features of bone marrow involvement of MCL on the basis of WHO classification. Results The median age at diagnosis is 55 years, M:F = 2.67:1 (male 40 and female 15). The type of involvement includes interstitial, intrasinusoidal, diffuse and mixed infiltration; the last is common. MCL usually consists of small to medium-sized lymphoid elements and characterized by expression of the B-cell markers. The characteristic expression of MCL is CD5 and cyclinD1. The MCL has four cytological variants. Among these, the classic variant is most commonly found. The prognosis has two major factors, the number of mitotic figures and Ki-67 making can divide different groups on prognosis. Conclusion The infiltration of MCL is rare and has specific features on bone marrow. It must be distinguished from other mature B cell lymphomas.

Mantle Cell Lymphoma of Blastoid Variant With Skin Lesion and Rapid Progression

Mantle cell lymphoma (MCL) is a type of aggressive mature B-cell neoplasm. Skin involvement of MCL is extremely rare, with only 21 cases reported so far. We report a case of MCL with blastoid variant, presenting as skin lesion initially. A 53-year-old man presented with increasing petechiae and ecchymosis after slight hit. Biopsy of skin showed tumor cells densely infiltrated dermal and subcutis with blastoid cytological features and numerous mitotic figures. Immunohistochemistry study showed tumor cells were positive for CD20, CD79a, BCL2, CyclinD1, and MUM1 but lost CD5 expression. Fluorescence in situ hybridization (FISH) studies demonstrated t(11;14). Although received chemotherapy after the diagnosis established, the patient deteriorated rapidly and died 5 weeks after presenting with skin lesions.

Duodenal involvement of mantle cell lymphoma observed by magnified endoscopy with narrow-band imaging

A 67-year-old man with mantle cell lymphoma (MCL) in remission for 3 years underwent follow-up positron emission tomography-computed tomography that showed cervical and abdominal lymph node enlargement. EGD was performed, and on duodenoscopy, multiple polypoid lesions of varying sizes were observed (A). Magnified chromoendoscopy with crystal violet staining of the smaller lesions showed a concentric circled structure and dilatation of the surrounding villi (B), and narrow-band imaging revealed looped and elongated microvessels on the surface of the lesions (C). The same endoscopic findings were emphasized on the surface of the larger lesions (D). Endoscopic biopsy specimens taken from the small lesions showed infiltration of neoplastic lymphoid cells (E), which were positive for CyclinD1 (F), CD20, and Bcl-1 but negative for CD3, CD5, and CD10. The patient received a diagnosis of MCL involving the duodenum, and chemotherapy with bendamustine was begun.