Non-Hodgkin Lymphoma
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IRF4/DUSP22 Gene Rearrangement by FISH
IRF4/DUSP22 Gene Rearrangement by FISH The IRF4/DUSP22 locus is rearranged in a newly recognized subtype of non-Hodgkin lymphoma, large B-cell lymphoma with IRF4 rearrangement. These lymphomas are uncommon, but are clinically distinct from morphologically similar lymphomas, Tests to Consider including diffuse large B-cell lymphoma, high-grade follicular lymphoma, and pediatric- type follicular lymphoma. The IRF4/DUSP22 locus is also rearranged in a subset of ALK- IRF4/DUSP22 (6p25) Gene Rearrangement negative anaplastic large cell lymphomas (ALCL), where this rearrangement is associated by FISH 3001568 with a signicantly better prognosis. Method: Fluorescence in situ Hybridization (FISH) Test is useful in identifying ALK-negative anaplastic large cell lymphomas and large B- Disease Overview cell lymphoma with IRF4 rearrangement The rearrangement is associated with an improved prognosis Incidence See Related Tests Large B-cell lymphoma with IRF4 rearrangement accounts for <1% of all non-Hodgkin B-cell lymphomas overall More common in younger patients, with an incidence of 5-6% under age 18 IRF4/DUSP22 rearrangement is found in 30% of ALK-negative ALCLs Symptoms/Findings Large B-cell lymphoma with IRF4 rearrangement typically presents with limited stage disease in the head and neck, while the presentation of ALK-negative ALCLs is variable. Disease-Oriented Information Patients with large B-cell lymphoma with IRF4 rearrangement typically have a favorable outcome after treatment. Rearrangement of the IRF4/DUSP22 locus in ALK-negative ALCL is associated with a better prognosis than ALK-negative ALCL without this rearrangement. Test Interpretation Analytical Sensitivity The limit of detection (LOD) for the IRF4/DUSP22 probe was established by calculating the upper limit of the abnormal signal pattern in normal cells using the Microsoft Excel BETAINV function. -
Follicular Lymphoma
Follicular Lymphoma What is follicular lymphoma? Let us explain it to you. www.anticancerfund.org www.esmo.org ESMO/ACF Patient Guide Series based on the ESMO Clinical Practice Guidelines FOLLICULAR LYMPHOMA: A GUIDE FOR PATIENTS PATIENT INFORMATION BASED ON ESMO CLINICAL PRACTICE GUIDELINES This guide for patients has been prepared by the Anticancer Fund as a service to patients, to help patients and their relatives better understand the nature of follicular lymphoma and appreciate the best treatment choices available according to the subtype of follicular lymphoma. We recommend that patients ask their doctors about what tests or types of treatments are needed for their type and stage of disease. The medical information described in this document is based on the clinical practice guidelines of the European Society for Medical Oncology (ESMO) for the management of newly diagnosed and relapsed follicular lymphoma. This guide for patients has been produced in collaboration with ESMO and is disseminated with the permission of ESMO. It has been written by a medical doctor and reviewed by two oncologists from ESMO including the lead author of the clinical practice guidelines for professionals, as well as two oncology nurses from the European Oncology Nursing Society (EONS). It has also been reviewed by patient representatives from ESMO’s Cancer Patient Working Group. More information about the Anticancer Fund: www.anticancerfund.org More information about the European Society for Medical Oncology: www.esmo.org For words marked with an asterisk, a definition is provided at the end of the document. Follicular Lymphoma: a guide for patients - Information based on ESMO Clinical Practice Guidelines – v.2014.1 Page 1 This document is provided by the Anticancer Fund with the permission of ESMO. -
Circle) None Fever Night Sweats Wt Loss Laboratory Studies Hgb WBC Plate
LYMPHOMA STAGING DIAGRAM Instructions: boxed items must be completed History B Sx (Circle) none fever night sweats wt loss Laboratory studies Hgb WBC Plate ECOG performance status g/L x109/L x109/L LDH (patient/upper normal) / CERVICAL PRE-AURICULAR HIV antibody pos neg WALDEYER'S RING UPPER CERVICAL MEDIAN OR LOWER CERVICAL HBsAg pos neg POSTERIOR CERVICAL SUPRACLAVICULAR INFRACLAVICULAR MEDIASTINAL HBcoreAb pos neg PARATRACHEAL MEDIASTINAL AXILLARY Hep C Ab pos neg AXILLARY HILAR RETROCRURAL SPE monoclonal protein pos neg type____________ SPLEEN PARA AORTIC PARA AORTIC MESENTERIC For Hodgkin lymphoma only CELIAC COMMON ILIAC SPLENIC (HEPATIC) HILAR EXTERNAL ILIAC PORTAL Albumin g/L INGUINAL MESENTERIC Lymphs x 109/L INGUINAL FEMORAL OTHER EPITROCHLEAR POPLITEAL Treatment Largest tumor diameter (nearest whole cm) Treatment plan Initial biopsy site Date (d/m/y) / / Histologic diagnosis Doctor in Charge 1 _________________________________________ 2 Reason for referral Bone marrow pos neg not done New Recurrent Follow-up Completed by _______________ Date____________ List all other extranodal sites here 1 2 Complete if diagnosis or stage subsequently changed 3 4 Diagnosis/Stage amended to _________________________ 5 Reason__________________________________________ 6 By_______________________ Date______________ Stage (circle) 0 1 2 3 4 A B E This staging diagram can be found on NOTIFY PATIENT INFORMATION IF STAGE OR H:lym_docs\staging\lymphoma.doc DIAGNOSIS IS AMENDED Form #TH-41 Revised 26 March 2007 LYMPHOMA AND CHRONIC LYMPHOCYTIC LEUKEMIA STAGING SYSTEMS BCCA LYMPHOMA, HODGKIN AND CHRONIC LYMPHOCYTIC LEUKEMIA NON-HODGKIN 1982 STAGE FINDINGS STAGE INVOLVEMENT 0 Lymphocyte count > 5.0 x 109 /L 1 Single lymph node region (1) or one Bone marrow contains 40% extralymphatic site (1E). -
The Lymphoma and Multiple Myeloma Center
The Lymphoma and Multiple Myeloma Center What Sets Us Apart We provide multidisciplinary • Experienced, nationally and internationally recognized physicians dedicated exclusively to treating patients with lymphoid treatment for optimal survival or plasma cell malignancies and quality of life for patients • Cellular therapies such as Chimeric Antigen T-Cell (CAR T) therapy for relapsed/refractory disease with all types and stages of • Specialized diagnostic laboratories—flow cytometry, cytogenetics, and molecular diagnostic facilities—focusing on the latest testing lymphoma, chronic lymphocytic that identifies patients with high-risk lymphoid malignancies or plasma cell dyscrasias, which require more aggresive treatment leukemia, multiple myeloma and • Novel targeted therapies or intensified regimens based on the other plasma cell disorders. cancer’s genetic and molecular profile • Transplant & Cellular Therapy program ranked among the top 10% nationally in patient outcomes for allogeneic transplant • Clinical trials that offer tomorrow’s treatments today www.roswellpark.org/partners-in-practice Partners In Practice medical information for physicians by physicians We want to give every patient their very best chance for cure, and that means choosing Roswell Park Pathology—Taking the best and Diagnosis to a New Level “ optimal front-line Lymphoma and myeloma are a diverse and heterogeneous group of treatment.” malignancies. Lymphoid malignancy classification currently includes nearly 60 different variants, each with distinct pathophysiology, clinical behavior, response to treatment and prognosis. Our diagnostic approach in hematopathology includes the comprehensive examination of lymph node, bone marrow, blood and other extranodal and extramedullary tissue samples, and integrates clinical and diagnostic information, using a complex array of diagnostics from the following support laboratories: • Bone marrow laboratory — Francisco J. -
CD169+ Macrophages Take the Bullet
nEWs and ViEWs 1. Fazilleau, N. et al. Immunity 30, 324–335 could sustain the presence of NKTFH cell– assessed mainly for their ability to induce strong (2009). dependent germinal centers. T helper type 1 or 2 or tolerogenic immune 2. Nutt, S.L. et al. Nat. Immunol. 12, 472–477 The new insights gained from these studies responses according to the therapeutic objec- (2011). could lead to the design of complex antigen for- tives. It will be important to assess the effect 3. Galli, G. et al. Proc. Natl. Acad. Sci. USA 104, 3984–3989 (2007). mulations that combine lipid-protein antigen of iNKT cell agonists on inducing IL-21 pro- 4. Chang, P. et al. Nat. Immunol. 13, 35–43 (2012). with T cell epitopes to prime not only B cells duction by iNKT cells to promote not only 5. King, I. et al. Nat. Immunol. 13, 44–50 (2012). 6. Cerundolo, V. et al. Nat. Rev. Immunol. 9, 28–38 but also T cells. This strategy would boost not B cell help but also a different spectrum of (2009). only iNKT cells but also conventional T cell cytokines such as IL-4 or interferon-γ, which 7. Tupin, E. et al. Nat. Rev. Microbiol. 5, 405–417 responses through the activation of dendritic are known to drive isotype switching to (2007). 8. Diana, J. et al. Eur. J. Immunol. 39, 3283–3291 cells. This strategy would combine the antigen- IgG1 and IgG2a, respectively. Modifying the (2009). specific iNKT cell cognate help provided to lipid covalently coupled to protein antigen 9. -
Quantitative Analysis of Bcl-2 Expression in Normal and Leukemic Human B-Cell Differentiation
Leukemia (2004) 18, 491–498 & 2004 Nature Publishing Group All rights reserved 0887-6924/04 $25.00 www.nature.com/leu Quantitative analysis of bcl-2 expression in normal and leukemic human B-cell differentiation P Menendez1,2, A Vargas3, C Bueno1,2, S Barrena1,2, J Almeida1,2 M de Santiago1,2,ALo´pez1,2, S Roa2, JF San Miguel2,4 and A Orfao1,2 1Servicio General de Citometrı´a, Universidad de Salamanca, Salamanca, Spain; 2Departamento de Medicina and Centro de Investigacio´n del Ca´ncer, Universidad de Salamanca, Salamanca, Spain; 3Servicio de Inmunodiagno´stico, Departamento de Patologı´a Clı´nica, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru´, ; and 4Servicio de Hematologı´a, Hospital Universitario, Salamanca, Spain Lack of apoptosis has been linked to prolonged survival of results in the overexpression of the bcl-2 protein and it malignant B cells expressing bcl-2. The aim of the present represented the first clear example of a common step in study was to analyze the amount of bcl-2 protein expressed 9,10 along normal human B-cell maturation and to establish the oncogenesis mediated by decreased cell death. Currently, frequency of aberrant bcl-2 expression in B-cell malignancies. the exact antiapoptotic pathways through which bcl-2 exerts its In normal bone marrow (n ¼ 11), bcl-2 expression obtained by role are only partially understood, involving decreased mito- quantitative multiparametric flow cytometry was highly vari- chondrial release of cytochrome c, which in turn is required for þ À able: very low in both CD34 and CD34 B-cell precursors, high the activation of procaspase-9 and the subsequent initiation of in mature B-lymphocytes and very high in plasma cells. -
Follicular Lymphoma with Leukemic Phase at Diagnosis: a Series Of
Leukemia Research 37 (2013) 1116–1119 Contents lists available at SciVerse ScienceDirect Leukemia Research journa l homepage: www.elsevier.com/locate/leukres Follicular lymphoma with leukemic phase at diagnosis: A series of seven cases and review of the literature a c c c c Brady E. Beltran , Pilar Quinones˜ , Domingo Morales , Jose C. Alva , Roberto N. Miranda , d e e b,∗ Gary Lu , Bijal D. Shah , Eduardo M. Sotomayor , Jorge J. Castillo a Department of Oncology and Radiotherapy, Edgardo Rebagliati Martins Hospital, Lima, Peru b Division of Hematology and Oncology, Rhode Island Hospital, Brown University Alpert Medical School, Providence, RI, USA c Department of Pathology, Edgardo Rebaglati Martins Hospital, Lima, Peru d Department of Hematopathology, MD Anderson Cancer Center, Houston, TX, USA e Department of Malignant Hematology, H. Lee Moffitt Cancer Center & Research Institute, Tampa, FL, USA a r t i c l e i n f o a b s t r a c t Article history: Follicular lymphoma (FL) is a prevalent type of non-Hodgkin lymphoma in the United States and Europe. Received 23 April 2013 Although, FL typically presents with nodal involvement, extranodal sites are less common, and leukemic Received in revised form 25 May 2013 phase at diagnosis is rare. There is mounting evidence that leukemic presentation portends a worse Accepted 26 May 2013 prognosis in patients with FL. We describe 7 patients with a pathological diagnosis of FL who presented Available online 20 June 2013 with a leukemic phase. We compared our cases with 24 additional cases reported in the literature. Based on our results, patients who present with leukemic FL tend to have higher risk disease. -
Primary Splenic and Nodal Marginal Zone Lymphoma
J. Clin. Exp. Hematopathol Vol. 45, No. 1, Aug 2005 Review Article Primary Splenic and Nodal Marginal Zone Lymphoma: Jacques Diebold, Agne`s Le Tourneau, Eva Comperat, Thierry Molina and Jose´ e Audouin Primary splenic and nodal marginal zone (MZ) lymphomas are rare small B cell lymphomas presenting with similar histopathologic features. The neoplastic cell population mostly consists of monocytoid B cells organized in a MZ pattern, associated with centrocytoid cells colonizing follicles. About 50% of cases have a monotypic plasma cell component. The different histopathologic patterns and differential diagnosis are discussed here. Both diseases share a similar immunophenotype, with the expression of B-cell associated antigens and restriction of immunoglobulin light chain. The only difference is the more frequent expression of IgD in splenic than in nodal lymphomas. The most recent findings in genetics and molecular biology are presented and discussed. The main clinical and biological symptoms are described and the similarity of some cases with Waldenstro¨ms macroglobulinemia is stressed. Both lymphomas present with the same type of bone marrow involvement with a high frequency of intravascular infiltrates, which can be associated with interstitial and nodular infiltrates. Transformation into diffuse large B cell lymphoma occurs in about 10 to 15% of the cases. The outcome in many splenic MZ lymphomas is characterized by a lengthy survival after splenectomy (9 to 13 years or longer), despite the absence of a consensus on the optimal treatment. Nodal MZ lymphoma has a more aggressive evolution and seems to only be curable at an early stage. Further studies are needed of both lymphomas to improve treatment and prognosis. -
Non-Hodgkin's Lymphoma Questions and Answers
Non-Hodgkin's Lymphoma Questions and Answers What is Non-Hodgkin's Lymphoma? Non-Hodgkin’s lymphoma (NHL) is a cancer of the lymphocytes, a type of white blood cell. When lymphocytes become cancerous (malignant), they multiply and become tumors. Lymphocytes are normally found in the blood stream and lymph nodes. Lymph nodes are found throughout the body and are identified by their location. They are a part of the body’s immune system, which includes the lymphatic system, spleen and lymphocytes. Some lymph nodes can be found just by feeling them in the neck, groin or under the arms. Some cannot be felt, but they can be seen on X-rays. NHL is the fifth most common cancer in the United States. Men are at slightly higher risk than women. It is more common in adults than children. The average age at diagnosis is 45 to 55 years. The cause of NHL remains unknown. What Are the Types and Symptoms of Non-Hodgkin’s Lymphoma? There are more than 30 different types of NHL. The specific types of NHL are associated with different symptoms. Low-grade or indolent NHL is usually associated with painless swelling of lymph nodes (usually in the neck or over the collarbone), but patients are otherwise healthy. The swelling may go away for a while, but then return. If the low-grade NHL has spread outside of the lymph nodes, such as to the stomach, there may be discomfort in the affected area. Low-grade lymphomas grow slowly. Examples of low-grade NHLs include: Marginal zone lymphomas. -
Non-Hodgkin Lymphoma Booklet
Understanding Non-Hodgkin Lymphoma A Guide for Patients, Survivors, and Loved Ones October 2017 Lymphoma Research Foundation (LRF) Helpline and Clinical Trials Information Service CONTACT THE LRF HELPLINE Trained staff are available to answer questions and provide support to patients, caregivers and healthcare professionals in any language. Our support services include: • Information on lymphoma, treatment options, side effect management and current research fi ndings • Financial assistance for eligible patients and referrals for additional fi nancial, legal and insurance help • Clinical trial searches based on patient’s diagnosis and treatment history • Support through LRF’s Lymphoma Support Network, a national one-to one volunteer patient peer program Monday through Friday, Toll-Free (800) 500-9976 or email [email protected] Understanding Non-Hodgkin Lymphoma A Guide For Patients, Survivors, and Loved Ones October 2017 This guide is an educational resource compiled by the Lymphoma Research Foundation to provide general information on adult non- Hodgkin lymphoma. Publication of this information is not intended to replace individualized medical care or the advice of a patient’s doctor. Patients are strongly encouraged to talk to their doctors for complete information on how their disease should be diagnosed, treated, and followed. Before starting treatment, patients should discuss the potential benefits and side effects of cancer therapies with their physician. Contact the Lymphoma Research Foundation Helpline: (800) 500-9976 [email protected] Website: www.lymphoma.org Email: [email protected] This patient guide is supported through unrestricted educational grants from: © 2017 Lymphoma Research Foundation. Information contained herein is the property of the Lymphoma Research Foundation (LRF). -
Essential Requirement for Nicastrin in Marginal Zone and B-1 B Cell Development
Essential requirement for nicastrin in marginal zone and B-1 B cell development Jin Huk Choia,b,1,2, Jonghee Hanc,1, Panayotis C. Theodoropoulosa,d, Xue Zhonga, Jianhui Wanga, Dawson Medlera, Sara Ludwiga, Xiaoming Zhana, Xiaohong Lia, Miao Tanga, Thomas Gallaghera, Gang Yuc, and Bruce Beutlera,2 aCenter for the Genetics of Host Defense, University of Texas Southwestern Medical Center, Dallas, TX 75390; bDepartment of Immunology, University of Texas Southwestern Medical Center, Dallas, TX 75390; cDepartment of Neuroscience, Peter O’Donnell Jr. Brain Institute, University of Texas Southwestern Medical Center, Dallas, TX 75390; and dDepartment of Internal Medicine, Physician Scientist Training Program, Washington University in St. Louis, Barnes Jewish Hospital, St. Louis, MO 63110 Contributed by Bruce Beutler, January 7, 2020 (sent for review September 24, 2019; reviewed by Douglas J. Hilton and Ellen V. Rothenberg) γ-secretase is an intramembrane protease complex that catalyzes peritoneal cavity, and are maintained by self-renewal throughout the proteolytic cleavage of amyloid precursor protein and Notch. the life of the organism (10). It is well established that the spleen is Impaired γ-secretase function is associated with the development also required for B-1 (especially B-1a) cell development (11); of Alzheimer’s disease and familial acne inversa in humans. In a however, the underlying mechanism(s) that mediate B-1 cell dif- forward genetic screen of mice with N-ethyl-N-nitrosourea-induced ferentiation remain largely unknown. mutations for defects in adaptive immunity, we identified animals The γ-secretase protease complex cleaves multiple type I mem- within a single pedigree exhibiting both hypopigmentation of brane proteins, including amyloid precursor protein (APP) and the fur and diminished T cell-independent (TI) antibody responses. -
MINI-REVIEW Large Cell Lymphoma Complicating Persistent Polyclonal B
Leukemia (1998) 12, 1026–1030 1998 Stockton Press All rights reserved 0887-6924/98 $12.00 http://www.stockton-press.co.uk/leu MINI-REVIEW Large cell lymphoma complicating persistent polyclonal B cell lymphocytosis J Roy1, C Ryckman1, V Bernier2, R Whittom3 and R Delage1 1Division of Hematology, 2Department of Pathology, Saint Sacrement Hospital, 3Division of Hematology, CHUQ, Saint Franc¸ois d’Assise Hospital, Laval University, Quebec City, Canada Persistent polyclonal B cell lymphocytosis (PPBL) is a rare immunoglobulin (Ig) M with a polyclonal pattern on protein lymphoproliferative disorder of unclear natural history and its electropheresis. Flow cytometry cell analysis displays the pres- potential for B cell malignancy remains unknown. We describe the case of a 39-year-old female who presented with stage IV- ence of the CD19 antigen and surface IgM with a normal B large cell lymphoma 19 years after an initial diagnosis of kappa/lambda ratio. In contrast to CLL, CD5 is absent. There PPBL; her disease was rapidly fatal despite intensive chemo- is an association between HLA-DR 7 and PPBL in more than therapy and blood stem cell transplantation. Because we had two-thirds of the patients but the reason for such an associ- recently identified multiple bcl-2/lg gene rearrangements in ation remains unclear.3,6 There has been one report of PPBL blood mononuclear cells of patients with PPBL, we sought evi- occurring in identical female twins.7 No other obvious genetic dence of this oncogene in this particular patient: bcl-2/lg gene rearrangements were found in blood mononuclear cells but not predisposition or familial inheritance has yet been described in lymphoma cells.