Mantle cell lymphoma mcl is a rare subtype of nonhodgkins lymphoma, comprising approximately 7% of all cases. We previously described the outcome of a cohort of 1031 young patients pts with mcl. Mantle cell lymphoma mcl is a mature b cell lymphoma associated with the hallmark translocation t11. Outcome of young patients with blastoid variant mantle. Mantle cell leukemia, blastoid variant researchgate. Ibrutinibinduced egression of largeblastoid mantle cell. Pdf cd5negative blastoid variant mantle cell lymphoma. Blastoid variant mantle cell lymphoma expressing aberrant. All three drugs target a protein on cancer cells known as cd19. The book will be accompanied by an online image bank containing numerous additional fullcolor images of the various disorders. The high proliferation on ki67 and the blastoid cytomorphology are both markers of aggressive biology.
It is named after denis parsons burkitt, the irish surgeon who first described the disease in 1958 while working in equatorial africa. Diagnosis is based on lymph node, bone marrow, or tissue morphology of centrocytic lymphocytes, small cell type, or blastoid variant. Lymphoma and plasma cell neoplasms mantle cell lymphoma mcl, classic variant. Dlbcl, follicular lymphoma fl, mantle cell lymphoma including blastic variant mcl, burkitt lymphoma leukemia bl, marginal zone b. The blastic variant bv form of mantle cell lymphoma mcl is considered to be a very aggressive subtype of nonhodgkins lymphoma nhl. Overt peripheral blood involvement leukemic presentation in patients with adenopathy is. The median age of patients at diagnosis is 65 and males represent 75% of cases. Mantle cell lymphoma mcl is a heterogeneous disease with a broad. The main treatment for mantle cell lymphoma is chemotherapy. The classic cytologic appearance of mcl is a monomorphic proliferation of small to mediumsized lymphoid cells with irregular nuclear contours and inconspicuous nucleoli. Mantle cell lymphoma is now recognised as a rare but distinct entity in the revised who classification. Men get mantle cell lymphoma more often than women. Outcome of young patients with blastoid variant mantle cell. The other approved cart cell therapies for cancer are tisagenlecleucel kymriah for acute lymphoblastic leukemia and axicabtagene ciloleucel yescarta for diffuse large b cell lymphoma.
Approximately 20% of mantle cell lymphomas mcl present with the blastoid variant associated with poor prognosis. Abstract, mantle cell lymphoma mcl is a bcell neoplasm that usually carries. Lactic acidosis complicated with hematologic malignancies is seen infrequently and is associated with a poor outcome. Mantle cell lymphoma usually presents with advanced stage and rapid progression, and historically, it has been considered an aggressive disease.
Mantle cell lymphoma an overview sciencedirect topics. Cyclin d1 expression is also observed in a subset of patients with myeloma, hairy cell leukemia, and splenic lymphoma, and sox. Blastoid variant of mcl american society of hematology. Car tcell therapy approved by fda for mantle cell lymphoma. Clinicopathologic features and management of blastoid. Immunophenotypic variations in mantle cell lymphoma. Biallelic amplification of atm gene in blastoid variant of mantle cell. Four cytologic variants of mcl are recognized in biopsy, small cell variant, mantle zone variant. A heterogeneous group that includes an unusual variant of mantle cell lymphoma. A rare case of leukemic presentation of blastoid variant.
Leukemic mantle cell lymphoma limited to blood and bone marrow is an indolent variant characterized by mildmoderate lymphocytosis, interstitial lowlevel. Mantle cell lymphoma mcl is a unique lymphoma subtype, both biologically and clinically. Mantle cell lymphoma, blastoid variant presenting as acute leukemia a rare case report. Some cases may represent a mantle cell lymphoma counterpart to chronic lymphocytic leukemia phenotype monoclonal b cell lymphocytosis. Mantle cell lymphoma may be slow growing indolent, but there can also be fastgrowing aggressive variations, called the blastoid variant and the pleomorphic variant. The average age of people with this form of cancer is the early 60s. Jul 29, 2020 mantle cell lymphoma mcl is a type of nonhodgkin lymphoma nhl. Leukemic phase of mantle cell lymphoma, blastoid variant. Prognostic factors guide mantle cell treatment decisions.
It is now well recognised that mcl represent a broad spectrum of different histopathological subtypes. The grade of malignancy is intermediate between that of follicular lymphoma and that of diffuse large b cell lymphoma, hence the previous designations lymphoma of intermediate differentiation and intermediate lymphoma. Blastoid mantle cell variant of richters transformation. In article crossref 7 bernard m, gressin r, lefrere f, et al. Blastoid variant of mantle cell lymphoma with leukemic. Mantle cell lymphoma, blastoid variant basicmedical key.
Blastoid variant of mantle cell lymphoma with lactic acidosis. Hairy cell leukemia is defined in almost all cases by the braf v600e mutation, which is not detected in hcl. In situ, mantle zone, nodular and diffuse patterns are commonly seen 2. Isolated primary presentation on the palatine tonsils is rare, and prognosis and outcome are. Jun 14, 2004 a 76yearold man presented with leukostasis syndrome, including oculodynia, blurred vision, and visual field defects, due to mantle cell lymphoma, prolymphocytoid variant, with marked. Multiple lymph nodes may be affected as well as other sites of the body including the spleen, bone marrow, blood, tonsils and adenoids, liver, brain, spinal cord and gastrointestinal tract. Mcl cells generally overexpress cyclin d1 due to a t chromosomal translocation in the dna. Mature b cell leukemias with more than 55% prolymphocytes. Mantle cell lymphoma mcl is a bcell nonhodgkin lymphoma with heterogeneous outcomes. Biopsy of a lymph node, tissue, or bone marrow shows the typical morphology of monomorphic small to medium sized. It can grow in several different architectural patterns including mantle zone, mantle cell nodular, follicular colonization, and diffuse. Chronic lymphocytic leukemia and mantle cell lymphoma. Small studies have shown that the blastoid variant has a particularly poor prognosis.
Leukemic mantle cell lymphoma limited to blood and bone marrow is an indolent variant characterized by mildmoderate lymphocytosis, interstitial lowlevel bone marrow involvement, simple karyotype, kappa light chain expression, cyclin d1 expression with lack of sox11, and slow or absent clinical progression. Hairy cell leukemia hcl hairy cell leukemia may be cyclin d1 positive refer to flow cytometry section for more detail chronic lymphocytic leukemia small lymphocytic lymphoma cllsll cd23 expression in noncll is often weak or subset positive cd200 is rarely expressed in mantle cell lymphoma prolymphocytic. How i treat mantle cell lymphoma blood american society. Many cell lines derived from mature b and t cell lymphoproliferative disorders have been used in the past two to three decades. Mantle cell lymphoma mcl is a relatively rare nonhodgkins lymphoma nhl with a heterogeneous clinical presentation comprising about 3% to 10% of nhl cases. Dec 27, 2018 the term blastoid mantle cell lymphoma mcl describes a morphological subgroup of lymphomas with blastic features. Two commonly distinguished cytological variants share blastic morphology and high proliferation, the pleomorphic and blastoid variants.
Blastoid variant of mantle cell lymphoma with lactic. Cytogenetic analysis of hematologic malignant diseases. Fs4 mantle cell lymphoma facts i page 1 revised november 2014 introduction lymphoma is the general name for many related subtypes of cancer that arise from a type of white blood cell called a lymphocyte. The coexistence of ccnd1igh and myc rearrangements in mantle cell lymphoma mcl is a rare finding associated with a very poor prognosis. Hairy cell leukemia variant, a new entity of the who 2008. Splenic b cell lymphoma leukemia, unclassifiable is a newly recognized disease group defined by the recent who classification of 2008. Some cases may represent a mantle cell lymphoma counterpart to chronic lymphocytic leukemia phenotype monoclonal b cell. Abstract the blastic variant bv form of mantle cell lymphoma mcl is considered to be a very aggressive subtype of nonhodgkins lymphoma nhl.
Pdf mantle cell lymphoma, blastoid variant, diagnosed on. Diagnosis is based on lymph node, bone marrow, or tissue morphology of centrocytic lymphocytes, small cell type, or blastoid variant cells. There are only about 15,000 patients presently in the united states with mantle cell lymphoma. Proposal for a novel scoring system for the diagnosis f cll. Nov 25, 20 fs4 mantle cell lymphoma facts i page 1 revised november 2014 introduction lymphoma is the general name for many related subtypes of cancer that arise from a type of white blood cell called a lymphocyte. Niklas vogt, wolfram klapper, variability in morphology and cell proliferation in sequential biopsies of mantle cell lymphoma at diagnosis and relapse. Diffuse architectural effacement can be seen, composed of small to mediumsized lymphoid cells.
Sep 28, 2018 weve all heard about indolent mantle cell so theres this leukemic nonnodal variant thats been described, she said, noting that this variant has a chronic lymphocytic leukemia like presentation no nodal disease, blood and marrow involvement, and splenic involvement. Mantle cell lymphoma, blastoid variant bmcl, is a very rare type of nonhodgkins lymphoma exhibiting an aggressive clinical course. Nov 06, 2001 abstract the blastic variant bv form of mantle cell lymphoma mcl is considered to be a very aggressive subtype of nonhodgkins lymphoma nhl. Immunohistochemistry and fluorescence in situ hybridization confirmed the identity of both tumors. Mcl is a subtype of b cell lymphoma, due to cd5 positive antigennaive pregerminal center b cell within the mantle zone that surrounds normal germinal center follicles. Pubmed a leukemic or blastoid phase is a finding of unusual high amounts of lymphocytes in the peripheral blood lymphocytosis suggesting that the lymphoma cells are in the blood in high concentrations. The ebmtemcl consensus project on the role of autologous and allogeneic stem cell transplantation in mantle cell lymphoma. Blastoid variant, which predominantly involves men in their sixth decade, has frequent extranodal involvement 4060%, stage iv disease up to 85% and central nervous. Cytogenetic technologist ii retired western connecticut health network, danbury hospital campus, danbury, connecticut, usa.
Usually the first sign of mcl is a painless swelling in the neck, armpit andor groin. To the best of our knowledge, atm gene amplification is not previously reported in bmcl and our case suggests a novel mechanism of atm. Lymphoma is a type of cancer that starts in your white blood cells. In order to determine its clinicobiological features and response to therapy we studied 33 patients 17% out of 187 suffering from mcl who were diagnosed with a bv of mcl. In this study, a patient with blastoid variant mcl is. The blastoid variant of mantle cell lymphoma mcl accounts for less than onethird of all mcl cases. An unusual occurrence of blastoid variant of mantle cell lymphoma arising in a patient with untreated chronic lymphocytic leukemia small lymphocytic lymphoma is described. S transition 3 by overcoming the suppressor effect of the retinoblastoma protein rb 4 and p27. May 23, 2018 large granular lymphocyte lgl leukemia is an indolent lymphoproliferative disorder that belongs to mature t and natural killer nk cell neoplasms and is recognized as cytotoxic t and nk cell lymphomas and leukemia in the 2016 world health organization classification. Burkitt lymphoma is a cancer of the lymphatic system, particularly b lymphocytes found in the germinal center. These correspond to both lowintermediate processes eg, chronic lymphocytic leukemia cll, mantle cell lymphoma mcl, hairy cell leukemia hcl and highly aggressive disorders eg, dlbcl and alcl.
Splenic b cell leukemia, hairy cell leukemia variant. The 2016 updated who classification of lymphoid neoplasias. Clinicopathologic features and management of blastoid variant. Mantle cell lymphoma, blastoid variant presenting in the tonsils of a 63 year old man. The overall cure rate for burkitt lymphoma in developed countries is about 90%, but worse in lowincome countries. Six patients with mantle cell lymphoma, blastoid variant, involving the blood are described. A localized site of intense uptake on positron emission tomography suggested richters transformation.
Other treatments include targeted therapy, biological therapy, radiation therapy and stem cell transplant. Biopsy of a lymph node, tissue, or bone marrow shows the typical morphology of. Brexucabtagene is the first fdaapproved car t cell therapy for mantle cell lymphoma. Burkitt lymphoma can be divided into three main clinical variants.
Mantle cell lymphoma is a type of nonhodgkins lymphoma, comprising about 6% of nhl cases. Mantle cell lymphoma pathology update in the 2016 who. Virtually all cases are characterized by a common genetic lesion. The lymphoid cells are medium in size, resembling lymphoblasts with dispersed chromatin and a high mitotic rate. Two cytologic variants of mcl have been identified, classical and blastoid, with the last including pleomorphic and blastic subtypes. It may transform to a more aggressive blastoid or pleomorphic variant, with or without acquisition of chromosomal abnormalities. Mantle cell lymphoma mcl is an aggressive nonhodgkin b cell lymphoma typically expressing cd19, cd20, cd5, fmc7, cyclind1, and sox11 and harboring the ighccnd1 translocation. On bone marrow examination 43% abnormal lymphoid cells were. Here we report the case of a patient with the blastoid variant of mcl complicated by lactic acidosis and who achieved complete remission through chemotherapy. Mantle cell lymphoma atlas of genetics and cytogenetics. Blastoid and pleomorphic variants see mantle cell lymphoma mcl. Mantle cell lymphoma mcl is a type of mature b cell nonhodgkin lymphoma with characteristic morphology and immunophenotype, which typically presents in elderly patients with advanced disease and shows a poor prognosis.
The term blastoid mantle cell lymphoma mcl describes a morphological subgroup of lymphomas with blastic features. Blastoid variant of mantle cell lymphoma a rare case report. Mantle cell lymphoma mcl, a b cell nonhodgkin lymphoma with a median survival of 35 years has a relatively aggressive variant characterized by blastoid appearance of lymphoma cells. Peripheral smear showed thrombocytopenia along with 53% abnormal cells. On the molecular level, mcl is characterized by the translocation t11. Blastoid variant, which predominantly involves men in their sixth decade, has frequent extranodal involvement 4060%, stage iv disease up to 85% and central nervous system cns involvement. Most people with mantle cell lymphoma have cancer cells in more.
Mantle cell lymphoma is a b cell neoplasm that affects adults. Mantle cell lymphoma with plasma cell differentiation. The circulating blastlike cells suggested the possibility of acute leukemias, chronic lymphoproliferative disorders or peripheralized lymphomas. Dec 16, 2020 mantle cell lymphoma mcl is a relatively rare type of cancer of the lymphoid cells. Recently a novel molecular assay based on the gene expression of 16. The term blastic or blastoid variant is generally used to describe cases with a homogeneous population of cells displaying lymphoblastic morphology. Leukemic involvement is a common feature in mantle cell. Blastoid variant mantle cell lymphoma expressing aberrant cd3.
Mcl is an aggressive b cell type of nonhodgkins lymphoma, and the rare disease accounts for about 2%10% of nonhodgkins lymphomas. Hairy cell leukemia 149 hairy cell leukemia variant 150. We report a blastoid variant of mantle cell lymphoma mcl involving an inguinal lymph node that, in addition to classical phenotypic and genetic findings, also aberrantly coexpresses surface cd10 and cytoplasmic. Role of cd11c expression in the diagnosis of mantle cell. Monoclonal gammopathy of undetermined significance.
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