B lymphoblastic lymphoma pathology outlines Authors David T O Yeung 1 2 3 , Michael P Osborn 2 3 4 , Deborah L White 1 2 5 Affiliations 1 Precision Medicine Theme, South Australian Health and Lymphoblastic lymphoma: Can demonstrate similar blast-like morphology Relies on immunophenotyping to differentiate Positive for T cell or B cell lineage markers; commonly positive for TdT. An introduction to lymph nodes and lymph node pathology that is not lymphoma are in the articles lymph node and lymph node pathology. Case report We describe clinical, histologic, immunohistochemical, and molecular features of Sample pathology report. Overall, these findings are consistent with intravascular Peripheral T cell lymphomas (PTCLs) are a heterogeneous and often clinically aggressive group of neoplasms derived from mature post-thymic T lymphocytes. Answers A, B and C are incorrect because these entities are typically negative for myeloperoxidase, although acute lymphoblastic leukemia can show isolated myeloperoxidase positivity. This article is an introduction to lymphoma. The majority of B-LBLs express lymphoblast-defining markers CD34 and/or terminal deoxynucleotidyl transferase (TdT), the latter a DNA transferase expressed within the nucleus of immature B- and T-cells and present in 80–90% of lymphoblastic Pro-B acute lymphoblastic leukemia (Leuk Lymphoma 2002;43:1855, Exp Hematol 2005;33:1500) CD27 positive hairy cell leukemia, Japanese variant ( Virchows Arch 2016;468:375 ) Chronic myeloid leukemia, CD34 positive blasts ( J Clin Invest 2012;122:624 ) 18 year old man with primary cutaneous anaplastic large cell lymphoma (J Cancer Res Ther 2015;11:656) 8 men, age 46 and older, with primary cutaneous anaplastic large cell lymphoma small cell variant (Am J Dermatopathol 2017;39:877) Skin nonmelanocytic tumor Lymphoma and related disorders (see also Lymphoma chapter) Leukemic infiltrates of skin and cutaneous mastocytosis Leukemia cutis Of note, there are some types of B-cell lymphoma that only rarely, if ever, occur as primary dis- ease in the skin, such as small lymphocytic lymphoma, lym- phoplasmacytic lymphoma, and mantle cell lymphoma. 1 and Lymphoma / leukemia (e. To view them sorted by case number, diagnosis or category, visit our main Case of the Week page. Treatment regimens, especially in T-LBL with large mediastinal tumours, are complex with B-cell acute lymphoblastic leukaemia: recent discoveries in molecular pathology, their prognostic significance, and a review of the current classification Br J Haematol. Chemistry, toxicology & UA; Coagulation; Hematology & immune disorders; Lab admin & management; Microbiology & 15 year old girl with rearrangements of MYC and BCL6 by fluorescent in situ hybridization (Am J Clin Pathol 2016;145:710) ; 48 year old man, 72 year old woman and 80 year old man with nodal diffuse large B cell lymphoma with morphologic features of primary mediastinal large B cell lymphoma presenting in submandibular or supraclavicular lymph nodes (Am J Also Burkitt lymphoma, lymphoblastic lymphoma, follicular lymphoma, other low grade B cell lymphoma, Hodgkin lymphoma, T cell lymphomas Microscopic (histologic) images AFIP images Despite originating from B cells, Hodgkin and Reed-Sternberg (HRS) cells in classic Hodgkin lymphoma exhibit a disrupted B cell program, resulting in the loss or downregulation of numerous B cell markers. Answer A is a common B CLL / SLL phenotype. 90% of B cell lymphomas, although B cell lymphomas are CD20 negative after rituximab and other B cell markers (CD19, CD79a, PAX5) should be used (Am J Clin Pathol 2006;126:534, Biomark Res 2017;5:5, Ann Hematol 2020;99:2141) 40% of pre-B acute lymphoblastic leukemia / lymphoblastic lymphoma (ALL / LBL) (Hematology Am Soc Hematol More CD163+ macrophages in T cell histiocyte rich large B cell lymphoma-like nodular lymphocyte predominant Hodgkin lymphoma and in T cell histiocyte rich large B cell lymphoma Small T cells are predominantly of cytotoxic phenotype in T cell histiocyte rich large B cell lymphoma (Haematologica 2010;95:440, Int J Clin Exp Pathol 2009;3:39) Rare cases of plasmablastic lymphoma (PBL) or B lymphoblastic leukemia / lymphoma have been reported (Eur J Haematol 2001;67:322) Sample pathology report. Diagnosis. Medeiros: Diagnostic Pathology - Lymph Nodes and Extranodal Lymphomas, 3rd Edition, 2023, WHO Classification of Tumours Editorial Board: B cell lymphoblastic lymphoma is a very rare variant, affecting patients with a median age of 39. In this study, the clinicopathologic features and prognosis of 33 THL patients were compared to those of 83 patients with MYC/BCL2 DHL and 13 patients with MYC/BCL6 DHL. 4 Molecular; 8 Precursor T-cell lymphoblastic lymphoma/leukemia May 21, 2024 · 13 year old girl with mediastinal ALK negative anaplastic large cell lymphoma (Oxf Med Case Reports 2019;2019:omz077) 21 year old man with a pulmonary abscess (Clin Nucl Med 2019;44:234) 53 year old man with AIDS with subcutaneous nodules on the scalp and axillae and diffuse lymphadenopathy (BMJ Case Rep 2019;12:e230641) 54 year old man misdiagnosed Dec 17, 2015 · Lymphoblastic lymphoma is a rare aggressive neoplasm of T-/B-precursors resembling acute lymphoblastic leukemia, with no or limited bone marrow involvement (<25%), that develops more frequently in children and young adults and is typically characterized by a grossly enlarged mediastinum, and whose diagnostic hallmark is the expression of a T-/B Jul 9, 2024 · CAR T cell therapy was originally developed for B cell acute lymphoblastic leukemia It is also now Food and Drug Administration (FDA) approved for B cell non-Hodgkin lymphoma (NHL), follicular lymphoma, mantle cell lymphoma and multiple myeloma; its application to solid organ tumors is being studied · T lymphoblastic leukemia (Am J Surg Pathol 1992;16:1075) T cell lymphomas (peripheral T cell lymphoma, NOS [57% positive], angioimmunoblastic T cell lymphoma [80%], ALK negative [17%] and ALK positive [0%] anaplastic large cell lymphoma) (Am J Hematol 2017;92:E1, Cytometry B Clin Cytom 2006;70:142) Sep 1, 2015 · Most are diffuse large B cell lymphoma, marginal zone B cell / MALT lymphoma or mixtures of these two Rarely follicular cell lymphoma . Clonal T cell EBV positive diffuse large B cell lymphoma (Pathology 2020;52:40): Activated B cell phenotype CD30+ is common EBER+ is definitional of the disease More common nodal presentation T cell large granular lymphocytic leukemia (Am J Clin Pathol 2015;144:607, Am J Surg Pathol 2017;41:82, Histopathology 2014;64:171): Elderly patients 48 year old woman with involvement of mandible by non-Hodgkin lymphoma (J Postgrad Med 1995;41:90) 51 year old woman with non-Hodgkin lymphoma presenting with numb chin syndrome (BMJ Case Rep 2011;2011:bcr0120113712) 53 year old man with diffuse large B cell lymphoma of mandible (Case Rep Oncol 2015;8:451) Lymphoblastic lymphoma (85%), variable for other lymphomas (Appl Immunohistochem Mol Morphol 2009;17:409) Melanoma (50%; Sample pathology report. 116 B cell lymphoblastic lymphoma presents without mediastinal mass but instead involves lymph nodes and extranodal sites. Recent Findings T-LBL displays a mediastinal mass with pleuro-pericardic Diffuse proliferation of intermediate to large sized B cells Fine compartmentalizing fibrosis common Neoplastic cells positive for CD20 and CD45; May show variable / heterogeneous CD30 expression Often CD23 positive B cell lymphoma unclassifiable, with features intermediate between diffuse large B cell lymphoma and CHL (gray zone lymphoma): 34 year old woman with no subjective symptoms diagnosed with T lymphoblastic lymphoma (LBL) with ZNF198-FGFR1 or ZMYM2-FGFR1; bone marrow (BM) aspirate and biopsy showed hypercellularity with hypereosinophilia (Hematology 2018;23:470) ; 48 year old Japanese man with peripheral leukocytosis, diagnosed with B-ALL with BCR-FGFR1 gene CD3+, CD4+, CD5+, CD7+, CD8-. primary cutaneous follicle center lymphoma (PCFCL). Indolent NK cell lymphoproliferative disorder of the gastrointestinal tract, previously thought to be reactive, is newly recognized as neoplastic. • HGBL, 3. g. The term lymphoblastic lymphoma (LBL) is used to describe predominantly lymph node–based disease; however, because lymphoblastic lymphoma and acute lymphoblastic leukemia (ALL) are known to represent the same disease entity, the World Health 30 January 2018 - Case of the Week #474 All cases are archived on our website. Monomorphic epitheliotropic intestinal T cell lymphoma (MEITL) (Ann Hematol 2019;98:2541): B-lymphoblastic leukemia/lymphoma, also known as B-acute lymphoblastic leukemia, is derived from B-cell progenitors. 6 cm (see synoptic report) Comment: Immunohistochemistry was performed and the tumor expresses OCT 3/4 and CD117. Common chromosomes include 21, X, 14, and 4. 2022 Apr;197(1):13-27. Differential diagnosis. Skin, left thigh, punch biopsy: Intravascular large B cell lymphoma (see comment) Comment: Biopsy shows large atypical CD20 positive B cells present within vascular spaces in the subcutaneous fat. They are usually aggressive and may present in lymph nodes, blood, bone marrow, or other organs. MRD can be assessed using methodologies including flow cytometry and molecular genetics, with the former being rapid, Conversely, 10% of non-Hodgkin lymphomas in US / Europe are AIDS related Most common LHIV: Burkitt lymphoma, diffuse large B cell lymphoma (DLBCL), primary effusion lymphoma (PEL), plasmablastic lymphoma (ScientificWorldJournal 2011;11:687) Burkitt Lymphoma in HIV patients is 1000× more common than in non HIV+ individuals Three Enzyme digestion and heat induced epitope retrieval techniques may enhance the performance of IHC staining in low grade B cell lymphoma As noted above under Uses by pathologists, kappa and lambda testing is useful to differentiate nonneplastic (not kappa or lambda restricted) from neoplastic disease (either kappa or lambda restricted) Precursor B-cell lymphoblastic leukaemia/lymphoma (B-LBL) is a highly aggressive malignancy arising from precursor B-cells. To subscribe or unsubscribe to Case of the Month or our other email lists, click here. Hong Kong / Chinese cases are only rarely EBV+ (Am J Clin Pathol 1999;112:263) Follicular lymphoma has two subgroups: t(14;18) or bcl2 overexpression, usually CD10+ and WHO grade 1-2 Early B cell precursor is TdT+, CD34+, HLA-DR+, then undergoes heavy (H) chain rearrangement and adds CD19, then adds CD10, then adds IgM heavy chain; then adds light (L) chain rearrangement and adds cytoplasmic IgM with heavy and light chains, then B cells express IgM and IgD with the same binding site, then adds CD20 (now called pre B cell); then adds Distinction of DLBCL, NOS from other lymphomas is crucial because certain cases of these neoplasms, especially B-lymphoblastic lymphoma (B-LBL), Burkitt lymphoma (BL), and DLBCL, NOS, respond favorably to specific treatment regimens with long-term survival. Chapters By Subspecialty . The cells are negative with CD30 and AFP. 17879. T-lymphoblastic lymphoma (T-LBL) comprises 70–80% of cases, while precursor B-lymphoblastic lymphoma (pB-LBL) makes up the remaining 20–25% of cases. Answer A is incorrect because although it can differentiate MGUS from myeloma, MGUS plasma cells are CD56- and 73 year old woman with coexisting mantle cell lymphoma (Leuk Lymphoma 2005;46:1813) 82 year old man with acute monoblastic leukemia following granular lymphocyte proliferative disorder (Rinsho Ketsueki 2011;52:1870) Marginal zone lymphoma, MALT type is an extranodal, low grade B cell lymphoma composed by a heterogeneous population of centrocyte-like, monocytoid, scattered immunoblasts and centroblast-like cells The diagnosis of B-cell non-Hodgkin lymphomas has changed significantly over the past few decades as new immunophenotypic markers, molecular subtype classification schemes, and novel biomarkers have emerged. 2011 Resemble lymphoblastic lymphoma Pleomorphic: large cells with irregular nuclear border, cerebriform nuclei, multinucleation, lack of monomorphism Prominent nucleoli and abundant pale cytoplasm Resemble CD10- B lymphoblastic leukemia / lymphoma is associated with t(v; 11q23. The immunoprofile supports the morphologic interpretation of seminoma. Both enteropathy associated T cell lymphoma (EATL) and monomorphic epitheliotropic intestinal T cell lymphoma (MEITL) show infiltrative and destructive lymphoid infiltrates ; In contrast, indolent T-LPD shows a nondestructive infiltrate In EATL, the adjacent noninvolved intestinal mucosa shows morphological features of High CD99 expression in B cell acute lymphoblastic leukemia and large B cell lymphoma is associated with poor survival in (Br J Haematol 2019;184:418, Acta Haematol 2011;125:167) High CD99 expression in acute myeloid leukemia and multiple myeloma is associated with improved survival (Haematologica 2020;105:999, Korean J Pathol 2014;48:209) Leukemia acute - B lymphoblastic leukemia / lymphoma with t(5;14)(q31;q32); IL3-IGH a “B-lymphoblastic leukemia/lymphoma” is coded in the International Classification of Diseases (ICD)-11 as B lymphoblastic leukemia or lymphoma, not elsewhere classified ; b New entities in 2022, or upgraded to definite from provisional entities in the revised 4th WHO edition in 2017; c Provisional entity in the revised 4th WHO edition in 2017 not recognized by WHO-HAEM5, as Lymphoblastic leukemia / lymphoma (B or T cell), approximately 33 - 100% with various antibody types used; association with certain genetic subtypes has been identified in the third study listed (Haematologica 2009;94:1563, Haematologica 2009;94:1555, Sci Rep 2020;10:2043) Of note, 0% (0/7) cases of T ALL were positive in one study (Sci Rep 2020;10:2043) Burkitt lymphoma: 20 - TNFα inhibitors in Crohn's disease: fourfold increased risk of lymphomas (B cell lymphomas and hepatosplenic T cell lymphoma) (Am J Gastroenterol 2013;108:99) Risk factors for hepatosplenic T cell lymphoma Hematopoietic: follicular lymphoma (germinal centers stain); also acute lymphoblastic leukemia, angiotropic / intravascular lymphoma, Burkitt lymphoma (occasionally, Indian J Pathol Microbiol 2011;54:290), diffuse large B cell lymphoma (variable), extranodal NK/T-cell lymphoma, nasal-type: 39% (Am J Clin Pathol 2008;130:343), Hodgkin lymphoma Background T-cell lymphoblastic lymphoma comprises approximately 85-90% of all lymphoblastic lymphomas. Here we focused on the Rare B cell neoplasm characterized by production of monoclonal heavy chain unable to bind light chain (truncated or mutated) Alpha heavy chain disease is the most common; it is also known as immunoproliferative small intestinal disease (IPSID), a variant of extranodal marginal zone lymphoma of mucosa associated lymphoid tissue (MALT lymphoma) 13 year old girl with mediastinal ALK negative anaplastic large cell lymphoma (Oxf Med Case Reports 2019;2019:omz077) 21 year old man with a pulmonary abscess (Clin Nucl Med 2019;44:234) 53 year old man with AIDS with subcutaneous nodules on the scalp and axillae and diffuse lymphadenopathy (BMJ Case Rep 2019;12:e230641) 54 year old man misdiagnosed Lymphoblastic lymphoma is a neoplasm of lymphoblasts, more commonly of T-cell origin than of B-cell origin, that resembles acute lymphoblastic leukemia (ALL), as Reprint requests to: Bouthaina Shbib Dabaja, MD, Department of Radiation Oncology, University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston 77030. Prognostic factors. Answer B is a common Sézary syndrome phenotype. 4% IS BCR::ABL1 p210 result interpretation: The BCR::ABL1 p210 fusion transcript (e13a2 or e14a2) is detected. Answer C is a common T lymphoblastic leukemia / lymphoma phenotype. From a histopathological point of view, blasts may be encountered in tissue biopsy and/or bone marrow (BM). Answer E is a common T cell large Leukemia acute - B lymphoblastic leukemia / lymphoma with hyperdiploidy. "High hyperdiploidy" means > 50 chromosomes and 66 chromosomes without translocations or other structural alternations Generally arises by simultaneous gain of all additional chromosomes in a single abnormal mitosis (Genes Chromosomes Cancer 2005;44:113) Leukemia acute - B lymphoblastic leukemia / lymphoma with t(1;19)(q23;p13. BCR::ABL1 p210 result: 22. Lija In the latest 2016 World Health Organization classification of hematological malignancies, T-cell lymphoblastic lymphoma (T-LBL) and lymphoblastic leukemia (T-ALL) are grouped together into one AML M4 and M5 (50%), follicular lymphoma (some), Langerhans cell histiocytosis, lymphoplasmacytic lymphoma (81%) (Am J Clin Pathol 2005;124:414) Psoriatic skin lesions (Proc Natl Acad Sci USA 2005;102:19057) Sinus histiocytosis with massive lymphadenopathy (Hum Pathol 1992;23:647) Small lymphocytic lymphoma (Am J Clin Pathol 1998;110:582) Sample pathology report. 5 years previously on maintenance chemotherapy, with right lower lobe consolidation (Fetal Pediatr Pathol 2018;37:7) 22 year old man with lymphomatoid granulomatosis of the CNS (Neuropathology 2019;39:479) 56 year old renal allograft recipient; 11 years after B lymphoblastic lymphoma: CD19+, TCF4 / CD123- dual color T lymphoblastic lymphoma: CD3+, TCF4 / CD123- dual color Neuroendocrine tumor (including Merkel cell carcinoma): Chromogranin+, synaptophysin+, 30 and 45 year old women with NK lymphoma (Arch Pathol Lab Med 2000;124:1510) 32 year old woman with sarcomatoid variant of B cell lymphoma (Int J Gynecol Pathol 2003;22:289) 38 year old woman with precursor T cell acute lymphoblastic leukemia/lymphoma (Ann Diagn Pathol 2002;6:125) 42 year old woman with Burkitt lymphoma CD10- B lymphoblastic leukemia / lymphoma is associated with t(v; 11q23. Although cytogenetic workup does not show any abnormalities in the submitted bone marrow specimen, flow cytometry reveals an expanded A. Jamie Shutter, University of South Florida, Tampa, Florida (USA), for contributing this case. Multiple myeloma with Acute lymphoblastic leukemia/lymphoma (ALL) is a neoplasm of precursor B or T lymphoid cells, representing the most common leukemia and cancer in the pediatric age group. Y & XH0KD4 - other B lymphoblastic leukemia / lymphoma with recurrent genetic abnormalities & B lymphoblastic leukemia / lymphoma with iAMP21 Epidemiology Seen in older children with a median age of 9 years ( Cancer Genet 2020:243:52 ) Lymphoblastic lymphoma (LBL) is a neoplasm of immature B cells committed to the B-(B-LBL) or T-cell lineage (T-LBL) that accounts for approximately 2% of all lymphomas. Clinical significance: This assay is intended for use as an aid in the diagnosis and management of BCR::ABL1 p210 positive chronic myeloid leukemia (CML), B lymphoblastic leukemia / Leukemia acute - B lymphoblastic leukemia / lymphoma with hypodiploidy. Risk-adapted intensive chemotherapy is effective in treating most children with B-acute lymp Advances in the molecular pathobiology of B-lymphoblastic leukemia Hum B cell lymphomas (Appl Immunohistochem Mol Morphol 2010;18:561) B cell lymphoma status post rituximab therapy (Histopathology 2008;53:278, Am J Clin Pathol 2006;126:534) B lymphoblastic leukemia / lymphoma (Histopathology 2008;53:278, Clin Med Res 2010;8:84) Classic Hodgkin lymphoma (Mod Pathol 2004;17:1531) Objectives: This session of the 2013 Society of Hematopathology/European Association for Haematopathology Workshop was dedicated to B-acute lymphoblastic leukemia (B-ALL)/lymphoblastic lymphoma (LBL) with recurrent translocations and not otherwise specified. Which of the following combinations of IHC markers is diagnostic of ALK positive T cell lymphomas with a T follicular helper phenotype (angioimmunoblastic T cell lymphoma, nodal peripheral T cell lymphoma with T follicular helper phenotype and follicular T cell lymphoma): Expression of at least 2 or 3 of the following markers precludes the classification of PTCL, NOS: CD10, BCL6, PD1, CXCL13, CXCR5, CD278 and SAP Typically, an aggressive lymphoma that may involve several anatomic sites and may be resistant to chemotherapy Often a diffuse growth pattern (Virchows Arch 2011;459:557) Neoplastic cells compose < 10% of total cells and are often scattered and not in aggregates (Virchows Arch 2011;459:557) Reactive cells compose > 90% of total cells with numerous B-lymphoblastic leukemia/lymphoma with hyperdiploidy: Hyperdiploidy in B-ALL/LBL is characterized by more than 50 and fewer than 66 chromosomes without other structural abnormalities. Although histological features are usually sufficient to distinguish lymphoblastic from mature B- or T-cell neoplasms, of greater i Lymphoblastic lymphoma Crit Rev Oncol Hematol. T1w MRI, DLBCL, fat suppressed. It often arises as a mediastinal mass, and with bone marrow involvement. Liver, left lobe mass, Trucut biopsy: Features are consistent with epithelioid hemangioendothelioma (see comment) Comment: Histological examination shows cores of a neoplasm composed of Differential considerations include de novo diffuse large B-cell lymphoma, leg type vs. 31 Virtually all follicular lymphomas express monoclonal surface immunoglobulin (Ig), HLA Recent discovery of new biomarkers has led to improvements in diagnosis and prognosis in B-cell lymphomas. Persistent (more than 6 months) increase in the number of peripheral blood large granular lymphocytes, usually 2-20 x 10⁹ Distinct T cell population with coexpression of one or more NK cell associated antigens (CD16, Double hit lymphoma B lymphoblastic leukemia / lymphoma (TdT positive) Follicular lymphoma can relapse as classic Hodgkin lymphoma; Reference: Medeiros: Diagnostic Pathology - Lymph Nodes and Extranodal Lymphomas, 2nd Edition, 2017. Further molecular testing and clinical workup is recommended. Answer D is incorrect 14 year old boy with trisomy 21 and history of B lymphoblastic leukemia / lymphoma diagnosed 1. Prognosis is Pseudo B cell lymphomas: mantle cells, germinal center B cells (Mod Pathol 2006;19:1270) B cell non-Hodgkin lymphomas (Mod Pathol 2006;19:1270) B lymphoblastic lymphoma (acute lymphoblastic leukemia) Lymphocyte predominant cells in nodular lymphocyte predominant Hodgkin lymphoma (Mod Pathol 2006;19:1010, Rom J Morphol Embryol 2011;52:69) 50 year old African American woman with clonally related histiocytic sarcoma and B lymphoblastic leukemia / lymphoma (Am J Clin Pathol 2019;152:486) 63 year old white woman with histiocytic sarcoma associated with splenic marginal zone lymphoma (J Med Case Rep 2017;11:92) 72 year old man with histiocytic sarcoma secondary to chronic myeloid leukemia 1 Table of B-cell lymphoma; 2 Follicular lymphoma; 3 Mantle cell lymphoma; 4 Marginal zone lymphoma; 5 Hairy cell leukemia; 6 B cell small lymphocytic lymphoma/chronic lymphocytic leukemia; 7 Precursor B-cell lymphoblastic lymphoma/leukemia. 4 Molecular; 8 Precursor T-cell lymphoblastic lymphoma/leukemia Jan 1, 2013 · B-lymphoblastic lymphoma/leukemia, not otherwise specified (NOS), is the most common type of B-LBL. 1 Introduction 3. 3); KMT2A (MLL) rearrangement, infantile (< 1 year of age) presentation and poor prognosis (Leukemia 2002;16:1233) CD10 in diffuse large B cell lymphoma is a marker for germinal center phenotype (also HGAL, BCL6, CD38, LMO2), generally considered a favorable prognostic Precursor B cells (hematogones) (Hum Pathol 2017;65:175) Various subsets of normal B cells, from antigen committed B cells to germinal center B cells Surface CD3− CD4− CD8− thymocytes (Nat Rev Cancer 2005;5:640) Aggressive B cell lymphoma has more favorable prognosis with aggressive therapy lymphoblastic lymphoma. In the updated World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues, Burkitt lymphoma (BL) is defined as a mature highly aggressive B-cell neoplasm that often presents in extranodal sites or as an acute leukemia [1,2,3]. ALL also Aggressive mature B cell lymphoma Category of high grade B cell lymphoma newly defined in the revised 2016 WHO (Blood 2016;127:2375, Swerdlow: WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 2017) All B cell lymphomas harboring a MYC rearrangement (chromosome 8q24. Epub 2021 Nov 7. To our knowledge, we Sample pathology report. Recent findings: T-LBL displays a mediastinal mass with pleuro-pericardic effusions as key distinctive features B-lymphoblastic lymphoma/leukemia, not otherwise specified (NOS), is the most common type of B-LBL. Burkitt lymphoma: Usually has a prominent starry sky appearance, though the starry sky appearance is not entirely specific B-lymphoblastic lymphoma (B-LBL) is a rare and aggressive type of non-Hodgkin lymphoma. These neoplasms are rare and usually diagnostically challenging. 1. Monomorphous infiltration of lymphocytes, distortion of alveolar architecture, Dutcher bodies, pleural infiltration are more common in lymphoma Immunohistochemistry is often helpful Nonspecific interstitial pneumonia 6 year old girl with B cell acute lymphoblastic leukemia (B ALL) and CD56 and CD57 coexpression (Pediatr Hematol Oncol (NK) cells and NK lymphomas. To view them sorted by case number, diagnosis or category, visit our main Case of the Month page. Mammogram and ultrasound, DLBCL, 56 yo. 3); E2A-PBX1 (TCF3-PBX1) Classification of cutaneous large B cell lymphomas. Embryonal carcinoma, solid PAS (Periodic acid-Schiff) is a special stain, not an immunostain, that stains basement membrane (normal and in tumors), glycogen, some mucins and mucopolysaccharides Assessment for minimal/measurable residual disease (MRD) is a powerful prognostic factor in B lymphoblastic leukemia/lymphoma (B-LL/L) that is quickly becoming standard of care in assessing patients with B-LL/L posttherapy. The World Health Organization (WHO) 2017 classification system categorizes these disease B-lymphoblastic leukemia/lymphoma (B-ALL/LBL) is a neoplastic process of hematopoietic precursors that are committed to B lineage, also known as B lymphoblasts. Methods: In this review, we summarize the cases discussed during the workshop, review the B cells, histiocytes and histiocytic lesions, osteoblasts Most B cell lymphomas, but aberrant staining occurs Aberrant loss / dim positivity of CD3 in some cases of: Mycosis fungoides (Am J Clin Pathol 2000;114:467, Cytometry B Clin Cytom 2021;100:132, Blood 2005;105:3768) Anaplastic large cell lymphoma (Blood 2005;105:3768) T- and natural killer (NK)-cell lymphomas are neoplasms derived from immature T cells (lymphoblastic lymphomas), or more commonly, from mature T and NK cells (peripheral T-cell lymphomas, PTCLs). MRI, MALT lymphoma, 56 yo. Although TdT expression is documented in mature B-cell lymphomas, these cases need to be differentiated from B-lymphoblastic leukemia/lymphoma (B Nodal T cell lymphomas with T follicular helper (TFH) phenotype An umbrella category created to highlight the spectrum of nodal lymphomas with a TFH phenotype, including angioimmunoblastic T cell lymphoma, follicular T cell lymphoma and other nodal PTCL with a TFH phenotype Overlapping recurrent molecular / cytogenetic abnormalities Purpose of review: Lymphoblastic lymphoma (LBL) is a rare, highly aggressive non-Hodgkin lymphoma variant virtually indistinguishable from acute lymphoblastic leukemia (ALL). This case was contributed by Dr. Primary cutaneous follicle center lymphoma are essentially follicular lymphomas arising from/associated with skin hair Sample pathology report. Aka Discoidin Domain Receptor 1 (DDR1) ; Has homologous region to the Dicytostelium discoideum protein discoidin I in extracellular domain Magdalena Czader, Catalina Amador, James R Cook, Devang Thakkar, Clay Parker, Sandeep S Dave, Ahmet Dogan, Amy S Duffield, Reza Nejati, German Ott, Wenbin Xiao, Mariusz Wasik, John R Goodlad, Progression and transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma and B-cell prolymphocytic leukemia: Report from the TdT can be variable intensity and correlates with immature immunophenotype. Images hosted on other servers: Mammogram and ultrasound, DLBCL, 47 yo . Lymph node, right level II, excision: Diffuse large B cell lymphoma, nongerminal center subtype (see comment) Comment: In light of the patient's clinical history of chronic lymphocytic leukemia B-lymphoblastic leukemia/lymphoma is a neoplastic process of hematopoietic precursors committed to B lineage, which has recently undergone further subclassification based on underlying genetic aberrations. Symptoms: fatigue, fever or night sweats, weight loss or recurrent infections Most patients with follicular Over 75 genetic arrangements; most translocations at 11q23 involve MLL (mixed lineage leukemia) gene t(4;11)(q21;q23) - MLL-AF4: occurs in 60% of infants, 2% of other children and 3 - 6% of adults Large B cell lymphoma that is confined to and presumably arises in testis Recently classified under large B cell lymphomas (LBCL) of immune privileged sites in the most recent revision of the WHO, 5th edition; this is a new umbrella term that acknowledges common biological features of aggressive B cell lymphomas (Leukemia 2022 Jun 22 [Epub ahead of print]) Intestinal T cell lymphoma: . The most common type of DLBCL, designated as not otherwise specified, Lymphoblastic lymphoma (LBL) is a neoplasm of immature B cells committed to the B-(B-LBL) or T-cell lineage (T-LBL) that accounts for approximately 2% of all lymphomas. Lymphoblastic Lymphoma B-precursor lymphoblastic lymphoma (LBL) is very uncom- mon, probably comprising < 2% of PCBCL. The two entities are biologically very close but not identical; however in LBL, the bone marrow (BM) is not or only Diffuse large B cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma worldwide, representing approximately 30–40% of all cases in different geographic regions. These include pan-B cell antigens, such as CD19, CD20, CD22 and CD79a, as well as B cell transcription factors, such as OCT2, BOB. Lymphoblasts show the following ICD-11: 2A70. We review the advancements in diagnostics, staging, treatment, and response assessment. HGAL marks germinal center B cells and can aid in the identification of tumors that arise from B lymphocytes such as primary cutaneous large B cell lymphoma. Right testis, mass, radical orchiectomy: Seminoma, 3. This entity is common and accounts for to up to 25% of childhood B-ALL. These cells coexpress CD5, BCL6 and MUM1 and are negative for CD10 and CD3. B-LBL is closely related to B-cell acute lymphoblastic leukemia (B-ALL), with the main difference being where the disease primarily manifests. Lymphoid: B lymphoblastic leukemia / lymphoma (example: B ALL with KMT2A rearrangement) Immature lymphoid neoplasm Intermediate sized lymphoblasts Round to irregular nuclei Dispersed chromatin Distinct nucleoli Scant amount of basophilic cytoplasm 11 year old boy with chronic myeloid leukemia possessing the unique combination of T lymphoblastic transformation and a subclone harboring inv(3)(q21q26. B-acute lymphoblastic leukemia occurs predominantly in children, but can occur at any age. The most common phenotype of T PLL is that of mature CD4+ T cells expressing pan T cell antigens, including CD7. A first consideration in approaching large B-cell lymphomas is distinction of BL from DLBCL, NOS. 2017 May:113:304-317. 1 However, KMT2A amplification is rarer and generally reported to occur in only 1% of cases of acute myeloid leukaemia (AML). CD Markers - CD160-169. Regardless of stage, patients require aggressive chemotherapy regimens as suitable for B Sep 28, 2021 · Relatively rare, indolent low grade B cell lymphoma derived from postgerminal center B cell Patients present with localized or generalized peripheral lymphadenopathy Perifollicular and interfollicular lymphoid infiltrate that surrounds variably preserved germinal centers; diffuse pattern of infiltration can also be seen Purpose of review: Lymphoblastic lymphoma (LBL) is a rare, highly aggressive non-Hodgkin lymphoma variant virtually indistinguishable from acute lymphoblastic leukemia (ALL). 1111/bjh. B lymphoblastic lymphoma, not otherwise specified (see microscopic description and FISH) Microscopic description: Biopsy of the tongue shows benign squamous mucosa with an extensive and diffuse subepithelial infiltrate of medium sized to large neoplastic cells, some Current WHO B cell lymphoma classification features the following major updates (Int J Lab Hematol 2017;39:14): Significantly more detailed sorting of B lymphoblastic leukemia Care should be taken before diagnosing a transformed follicular lymphoma as high grade B cell lymphoma, especially if any retained follicular architecture exists Acute lymphoblastic leukemia (ALL)/lymphoblastic lymphoma (LBL) is a clonal hematopoietic stem cell disorder of B or T cell origin. Thanks to Gabriel Lerner, MS4 at Boston University School of Medicine, Boston, MA (USA) and Drs. A general introduction to haematopathology is in the haematopathology article. Lin et al reported Lymphoma, especially mucosa (bronchus) associated lymphoid tissue lymphoma. All LBCL withMYC and BCL2 and/ or BCL6 rearrangements, either belonging to the previous BCLU or DLBCL, will be included in a single category designated as HGBL with MYC and BCL2 and/or BCL6 translocations. , T lymphoblastic lymphoma / leukemia, small B cell lymphomas, T cell lymphomas, classic Hodgkin lymphoma): No diffuse meshwork of keratin positive neoplastic cells Effaced architecture in lymphoma ALK positive large B cell lymphoma (see comment) Medeiros: Diagnostic Pathology - Lymph Nodes and Extranodal Lymphomas, 2nd Edition, 2017, Medeiros: AFIP Atlas of Tumor Pathology - Tumors of the Lymph Node and Spleen, Series 4, 2017. Cases that fulfill the criteria for a follicular lymphoma or B lympho-blastic lymphoma are excluded. Our understanding of the pathogenesis of PTCL is increasing and this improved knowledge is leading us to better molecular characterization, more objective Lymphoblastic leukemias/lymphomas are neoplasms of precursor T cells and B cells or lymphoblasts. . 3) or in BCL6 T lymphoblastic leukemia / lymphoma (T-LBL) Large mediastinal mass in adolescent or young adult TdT+, CD1a+ T cell large granular lymphocytic leukemia (LGL) Peripheral blood lymphocytes have increased pale cytoplasm and prominent azurophilic granules Mild nuclear indenting but no polylobated nuclei as in ATLL CD8+, CD16+, CD57+ Additional 2 new classification systems for B cell lymphoid neoplasms have emerged in 2022: WHO 5th edition (WHO HAEM5) (Leukemia 2022;36:1720) International Consensus Classification (ICC) (Blood 2022;140:1229) Evolution from the 2016 WHO revised 4th edition (WHO HAEM4R) classification system to the new version reflects advancements in genomic profiling and B lymphoblastic leukemia / lymphoma can be negative; if positive, it is usually dimly expressed and the strong expression has been described as associated with a worse prognosis in B cell progenitor acute lymphoblastic leukemia (Blood Cells Mol Dis 2021; 89:102562) B and T cell lymphomas (most), including the lymphocyte predominant (LP) cells of nodular lymphocyte Non-Hodgkin lymphomas arise from monoclonal expansion of malignant B or T cells B cell lymphomas. Event-free and overall survival (EFS and OS) for paediatric LBL patients both exceed 80% with current therapies. Patients are most commonly young children, younger than 6 years old, who present with extranodal disease particularly involving skin, bone, and soft tissue [2, 3, 7, 8]. The prognosis for these patients may be better than for adults with T-cell lymphoblastic lymphoma. Mammogram, CLL / SLL. KMT2A amplification is rarely reported in B-lymphoblastic leukaemia (B-ALL) with only a handful of cases2–6 in the literature. doi: 10. This website is intended for pathologists and laboratory personnel but not for patients. 2) and a rearrangement in BCL2 (18q21. In tissue sections, LBL Lymphoblastic lymphoma Crit Rev Oncol Hematol. Autopsy & forensics; Bone, joints & soft tissue . 3 IHC; 7. It is similar to acute lymphoblastic leukemia (ALL) in terms of the cells it involves and its aggressive nature, but while ALL primarily affects the bone marrow and blood, lymphoblastic lymphoma primarily involves 20 September 2007 - Case #96 All cases are archived on our website. To subscribe or unsubscribe to Case of the Week or our other email lists, click here. Patients most often present with a rapidly growing tumour mass in single or multiple, nodal or extranodal sites. Answers B and D are incorrect because these cells are typically negative for CD56. May coexist or progress to EBV associated B cell lymphomas (e. 7. secondary involvement of DLBCL from a different site (less likely, given no prior history of lymphoma) vs. Tel: 713-563-2406; E-mail: Enteropathy associated T cell lymphoma (EATL) (Leukemia 2013;27:1688, Pathology 2020;52:128): Morphology and immunophenotype do not reliably distinguish between ITCL, NOS and EATL EATL is associated with celiac disease. It originates from immature B cells, a type of lymphocyte (a white blood cell) that normally helps the body fight infections. Bone & joints; Soft tissue ; Breast; Clinical pathology . Massively parallel sequencing technologies have identified Which of the following is true regarding B cell or plasma cell neoplasms as defined by the International Consensus Classification (ICC) system? C. Answer B is incorrect because HGAL is less specific for follicular lymphomas involving the bone marrow and has shown downregulation in Intestinal lymphoma derived from intraepithelial T cells that occurs in patients with celiac disease (Blood 2011;118:148) Comprises less than 1% of all non-Hodgkin lymphomas, 10% of all peripheral T cell lymphomas and 10 - 25% of all primary intestinal lymphomas (Blood 2011;118:148, Gut 2003;52:347, Curr Hematol Malig Rep 2016;11:504) Unfavorable prognostic features: Clinical: ↑ age, ↑ LDH, Eastern Cooperative Oncology Group (ECOG) ≥ 2, Ann Arbor stage III / IV, extranodal disease, bulky disease (J Clin Oncol 2010;28:2373, Blood 2014;123:837) Cell of origin status (Blood 2004;103:275): Activated B cell subtype (~40%) and germinal center B cell subtype (~60%) associated with 5 year A bone marrow aspirate sample obtained from a 2 year old girl with a history of B lymphoblastic leukemia (B ALL) is submitted for flow cytometric and cytogenetic analysis for minimal residual disease (MRD) assessment. B cell acute lymphoblastic lymphoma (ALL) Chronic lymphocytic lymphoma / small lymphocytic leukemia (CLL / SLL) Mantle cell lymphoma Follicular lymphoma Marginal zone B cell lymphoma Extranodal MALT type Hairy cell leukemia Circulating tumor cells are associated with poor prognosis (Pathology 2020;52:100) 30% relapse, often remain sensitive to chemotherapy (J However, most microvillous lymphomas are unusual variants of diffuse large B cell lymphoma (Am J Surg Pathol 1990;14:1047) Not used in routine diagnosis Molecular / cytogenetics description. CAR T cell therapy was originally developed for B cell acute lymphoblastic leukemia It is also now Food and Drug Administration (FDA) approved for B cell non-Hodgkin lymphoma (NHL), follicular lymphoma, mantle cell lymphoma and multiple myeloma; its application to solid organ tumors is being studied In AML without maturation (FAB AML M1), the myeloblasts are positive for myeloperoxidase. 2 Microscopic; 7. Sample pathology report. doi: Lymphoma is almost a specialty for itself. Accounts for 5-10% of acute leukemias, usually M4 or M5 Aggressive clinical features and poor outcome Other small B cell lymphoma with low level peripheral blood involvement (particularly monoclonal B cell lymphocytosis with non-CLL phenotype or atypical CLL phenotype): Some cases of monoclonal B cell lymphocytosis with non-CLL phenotype have 7q aberrations and develop splenomegaly, suggesting a connection with splenic marginal zone Purpose of Review Lymphoblastic lymphoma (LBL) is a rare, highly aggressive non-Hodgkin lymphoma variant virtually indistinguishable from acute lymphoblastic leukemia (ALL). Comment Here Reference: Myeloperoxidase B lymphoblastic lymphoma. Board review style question #1. It showed that Early studies suggested that follicular lymphoma and normal follicular-center B cells were related by their expression of cell surface antigens. Regardless of stage, patients require aggressive chemotherapy regimens as suitable for B Large B Cell Lymphoma • High grade B cell lymphoma (HGBL) with rearrangements of MYC and BCL2 or BCL6: new category for “double hit” or “triple hit” lymphomas; excludes cases that fulfill criteria for follicular or lymphoblastic lymphoma. KMT2A gene rearrangements are known to occur in both myeloid and lymphoid leukaemia. Cervical lymph node, excision biopsy: Mantle cell lymphoma (see comment) Comment: The tumor expresses CD5 and cyclin D1 in addition to other B cell markers, with a low proliferative index and negative CD23, consistent with a diagnosis of mantle cell lymphoma. PTCLs are rare but show marked biological and clinical diversity. It is composed of monomorphic medium-sized B-cells with Low grade B cell lymphomas: Monotonous proliferation of small lymphoid cells with typical diagnostic features of each entity Lacks history of immune deficiency / dysregulation EBV negative Additional references. Lymphoblastic lymphoma (LBL) is a highly aggressive neoplasm of lymphoblasts of either B- (B-LBL) or T-cell origin (T-LBL), grouped together with acute lymphoblastic leukemia (ALL) in the 2008 WHO classification of hematopoietic malignancies 1. Answer B is incorrect because indolent T lymphoblastic proliferation is not considered neoplastic under WHO 5th edition (2022) but a tumor-like lesion with T cell predominance. , central nervous system, testicle) 6q deletion in 40 - 60% of lymphoplasmacytic lymphoma cases Most common recurrent cytogenetic abnormality Tumor suppressor genes B lymphocyte induced maturation Immunohistochemical studies showed that lymphoma cells were positive for MUM1, CD138, CD38, CD30 and C-MYC (~10%) and negative for CD20 (therefore, choices A and E are eliminated), PAX5, CD3 (choices C and D are eliminated), ALK (ALK positive large B cell lymphoma would be ruled out) and HHV8 (primary effusion lymphoma is ruled out). 1 General; 7. 3); KMT2A (MLL) rearrangement, infantile (< 1 year of age) presentation and poor prognosis (Leukemia 2002;16:1233) CD10 in diffuse large B cell lymphoma is a marker for germinal center phenotype (also HGAL, BCL6, CD38, LMO2), generally considered a favorable prognostic Present in > 50% of primary cutaneous diffuse large B cell lymphoma, leg type In many diffuse large B cell lymphoma at immune privileged sites (e. Two new classifications, the fifth edition of the World Health Organization classification of Haematolymphoid Tumors and the International Classification Primary central nervous system (CNS) lymphoma occurs first in the brain, eye, spinal cord or leptomeninges, without systemic involvement and not arising in the setting of immunodeficiency Majority are high grade diffuse large B cell lymphomas, with only rare cases representing T cell lymphomas, low grade B cell lymphomas or Burkitt lymphoma Answer B is incorrect because CD5 expression does not appear to impact the prognosis of marginal zone lymphoma but may increase the risk of histologic transformation to diffuse large B cell lymphoma. It can be subclassified a number of ways. Recent findings: T-LBL displays a mediastinal mass with pleuro-pericardic effusions as key distinctive features · B cells: B cells express surface immunoglobulin (Ig), composed of 2 heavy (H) and 2 light (L) chains (either kappa or lambda) B cell antigen receptor loci may have 4 types of modification: (a) recombination of variable, diversity and joining regions (VDJ); (b) somatic hypermutation of V segments; (c) immunoglobulin heavy chain gene class switching; and (d) Feb 6, 2022 · 1 Table of B-cell lymphoma; 2 Follicular lymphoma; 3 Mantle cell lymphoma; 4 Marginal zone lymphoma; 5 Hairy cell leukemia; 6 B cell small lymphocytic lymphoma/chronic lymphocytic leukemia; 7 Precursor B-cell lymphoblastic lymphoma/leukemia. Meanwhile, there has been an increasing emphasis on individualizing treatment approaches in Pathology of B-cell lymphomas: diagnosis and Cutaneous peripheral T cell lymphomas listed separately Cutaneous peripheral T cell lymphoma, NOS is applied to cases not falling within a specific category Section on primary immunodeficiency associated lymphoproliferative disorders added Reinclusion of reactive lymphoid hyperplasia (last covered in 3rd edition) Soft tissue tumors Lymphoblastic lymphoma is a rare type of non-Hodgkin lymphoma that primarily affects lymphocytes, a type of white blood cell crucial for the immune system. Bone marrow, right posterior iliac crest, aspirate, clot and core biopsy: T lymphoblastic leukemia / lymphoma and myeloid / lymphoid neoplasm with TRIP11::FLT3 rearrangement and eosinophilia (see comment) Markedly hypercellular bone marrow (~100%) with trilineage hematopoiesis, marked myeloid hyperplasia, increased Could be associated with other hematologic malignancies such as B cell lymphoma, myelodysplastic syndrome and aplastic anemia Diagnosis. 2) at diagnosis (Biomark Res 2016;4:14) 39 year old asymptomatic man with hematologically typical chronic phase chronic myeloid leukemia showed an unusual and complex translocation involving Sample pathology report. Bone marrow, aspirate, clot, core biopsy and peripheral blood: Therapy related B lymphoblastic leukemia / lymphoma (see comment) Comment: The bone marrow shows increased blasts (***% by manual differential; *** by immunohistochemistry for CD34 / TdT) with atypical morphology and an aberrant B lymphoblast immunophenotype by Leukemia acute - B lymphoblastic leukemia / lymphoma with t(12;21)(p13;q22); TEL-AML1 (ETV6-RUNX1) Menu. Presentation at other sites without nodal or mediastinal localization is uncommon. EBV+ diffuse large B cell lymphoma) and classic Hodgkin lymphoma (Am J Surg Pathol 2007;31:1077, Br J Haematol 2007;138:733, Am J Clin Pathol 2002;117:368) Usually shows a type 2 or 3 of latency B lymphoblastic leukemia / lymphoma with t(v;11q23); MLL rearranged; Acute myeloid leukemia with t(9;11)(p22;q23); MLLT3-MLL; Mixed phenotype acute leukemia (MPAL) Precursor B cell lymphoblastic leukemia / lymphoblastic lymphoma. 1 Definition.
stw fokx zrtpy fgjw gchtx mjzzy flrkv juiste jpqsc lwalu