The WHO classification of mature T-cell leukemias

Blood ◽  
2004 ◽  
Vol 104 (9) ◽  
pp. 2989-2990 ◽  
Author(s):  
Daniel Catovsky ◽  
Estella Matutes ◽  
Claire Dearden ◽  
Nnenna Osuji ◽  
Vasantha Brito-Babapulle
Keyword(s):  
T Cell ◽  
Who Classification

Monocytosis As Prognostic Factor in Aggressive, Non-Primary Cutaneous Peripheral T-Cell Lymphoma: A Study of 251 Cases

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1608-1608
Author(s):  
Brady E Beltran ◽  
Erick Cotacallapa ◽  
Jorge J Castillo
Keyword(s):  
Overall Survival ◽  
T Cell ◽  
Who Classification ◽  
Cell Lymphoma ◽  
Statistical Significance ◽  
Prognostic Index ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
Worse Prognosis

Abstract Abstract 1608 Background: Peripheral T-cell lymphoma (PTCL) is a heterogeneous family of entities with a worse prognosis, stage by stage, than their B-cell counterparts. We have previously reported that an absolute lymphocyte count (ALC) <1000/uL is associated with a worse prognosis in Peruvian patients with PTCL (Castillo et al. 2010). The goal of this study is to investigate the prognostic value of absolute monocyte count (AMC) in the survival of patients with PTCL. Methods: A total of 251 cases of aggressive, non-primary cutaneous PTCL diagnosed at our institution between January 1997 and January 2012 were reviewed, reevaluated according to their morphological, immunological and clinical characteristics, and reclassified according to the 2008 WHO classification of lymphoid neoplasms. Characteristics will be presented descriptively. Kaplan-Meier method was used to estimate overall survival (OS) curves, which were compared using the log-rank test. The multivariate analysis was performed using the Cox proportional-hazard regression test. Results: According to the new WHO classification of lymphoid neoplasms, 104 cases (41%) were classified as adult T-cell leukemia/lymphoma (ATLL), 103 cases (41%) as PTCL, unspecified (PTCLU), 27 cases (11%) as analplastic lymphoma kinase (ALK)-negative anaplastic large cell lymphoma (ALCL), 11 cases (4%) as extranodal NK/T-cell lymphoma (NKTCL), nasal type, 4 cases (2%) as angioimmunoblastic lymphoma (AIL), and 2 cases (1%) were diagnosed with ALK+ ALCL. The median age at diagnosis was 57 years (range 14–92 years); 47% of patients were >60 years. The male-to-female ratio was 1:1. ECOG performance status >1 was seen in 51%, LDH was elevated in 67%, advanced stage was seen in 73%, and >1 extranodal sites were seen in 22% of the patients. Bone marrow involvement was reported in 30% and B symptoms in 64% of patients. An International Prognostic Index (IPI) score 3–5 was seen in 55%, and a Prognostic Index for PTCLU (PIT) score of 2–4 in 63%. The median overall survival (OS) for the whole group was 10 months. The IPI score, the PIT score, ALC <1000/uL and AMC >800/uL (Figure) showed statistical significance in the univariate survival analysis (p<0.001, p<0.001, p=0.001 and p=0.001, respectively). In the multivariate analysis, PIT score and AMC >800/uL showed statistical significance (p=0.006, p=0.046, respectively). Conclusions: Monocytosis, defined as AMC >800/uL, and the PIT score were independent prognostic factors for OS in patients with aggressive, non-primary cutaneous PTCL. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Uncommon Variants of Mature T-Cell Lymphomas (MTCLs): Imaging and Histopathologic and Clinical Features with Updates from the Fourth Edition of the World Health Organization (WHO) Classification of Lymphoid Neoplasms

Cancers ◽  
2021 ◽  
Vol 13 (20) ◽  
pp. 5217
Author(s):  
Ahmed Ebada Salem ◽  
Yehia H. Zaki ◽  
Gamal El-Hussieny ◽  
Khaled I. ElNoueam ◽  
Akram M. Shaaban ◽  
...  
Keyword(s):  
T Cell ◽  
Molecular Biology ◽  
Who Classification ◽  
Rare Variants ◽  
World Health ◽  
Complex Nature ◽  
Imaging Features ◽  
Fourth Edition ◽  
T Cell Lymphomas

Understanding the pathogenesis and molecular biology of malignant lymphomas is challenging, given the complex nature and incongruity of these disorders. The classification of lymphoma is continually evolving to account for advances in clinical, pathological, molecular biology and cytogenetic aspects, which impact our understanding of these disorders. The latest fourth edition of the WHO classification of lymphoid malignancies was released in 2016 to account for these changes. Additionally, unlike B-cell lymphomas (BCL), T-cell lymphomas (TCL) are uncommon, and may be sporadically experienced in clinical practice. These disorders are rare, thus early diagnosis is challenging for both physicians and radiologists, owing to the overlap in clinical and imaging features with other, more common disorders. We aim to discuss some rare variants of T-cell lymphomas, including clinicopathologic and imaging features, as well as to give a glimpse of the updates contained within the new 2016 WHO classification.

Distribution and Pathology Characteristics of Non Hodgkin Lymphoma in Peru: A Study of 1014 Cases Using WHO Classification of Lymphoid Neoplasm.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4419-4419 ◽  
Author(s):  
Brady Beltran ◽  
Domingo Morales ◽  
Pilar Quiñones ◽  
R. Salas ◽  
Antonio A. Carrasco-Yalan
Keyword(s):  
T Cell ◽  
Hodgkin’S Lymphoma ◽  
Who Classification ◽  
Hodgkin's Lymphoma ◽  
Asian Countries ◽  
Non Hodgkin’S Lymphoma ◽  
T Cell Lymphomas ◽  
Non Hodgkin's Lymphoma ◽  
Indolent Lymphomas

Abstract BACKGROUND: The frequency of various subtypes of non-Hodgkin’s lymphoma (NHL) differs in various regions worldwide. OBJECTIVE: To investigate the clinical and pathological features of non-Hodgkin’s lymphoma (NHL) and to evaluate the applicability of the new WHO classification of lymphoid neoplasms. METHODS: According to the new WHO classification, a total of 1014 cases of non-Hodgkin’s lymphoma diagnosed during the period 2002–2006 were reviewed and reappraised with their morphological, immunological and clinical characteristics in one general hospital from Lima, Peru. All cases corresponded >18 years old. RESULTS: There were 535 males and 479 females, mean age was 62.1 years (range 18 – 97 years) and the median was 64 years. B-cell neoplasms accounted for 763 cases (75.2%) and T/NK-cell neoplasms for 189 (18.6%). Sixty two cases (6.1%) were not classified. It was seen compared to that in the other Asian countries. Indolent lymphomas accounted for 17%, and aggressive ones for 83%. Among indolent lymphomas follicular grade I and II were the most common subset while MALT was second with low frequency. Among the aggressive lymphomas diffuse large cell lymphoma (DLCL) was the most common subtype, and accounted for 58.8% of all B cell lymphomas. Mantle and Burkitt lymphoma were very low incidence. Among the T cell lymphomas, peripheral T cell lymphomas, mycosis fungoides, Adult T Lymphoma/Leukemia (ATLL), T/NK nasal type Lymphoma were the most common subtypes. Nodal NHL occurred in 52% and extranodal in 48% of the cases. The more common extranodal presentation was stomach (14.1%), skin (8.1%), small intestine (2.9%) and nose (2.3%) CONCLUSIONS: The high incidence of T cell lymphomas, extranodal presentation and reduced frequency of indolent lymphoma in the current study is comparable to that reported from Asian countries.

scholarly journals Primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphomas: reappraisal of a provisional entity in the 2016 WHO classification of cutaneous lymphomas

2017 ◽  
Vol 30 (5) ◽  
pp. 761-772 ◽  
Author(s):  
Joan Guitart ◽  
M Estela Martinez-Escala ◽  
Antonio Subtil ◽  
Madeleine Duvic ◽  
Melissa P Pulitzer ◽  
...  
Keyword(s):  
T Cell ◽  
Who Classification ◽  
Cytotoxic T Cell ◽  
T Cell Lymphomas ◽  
Cutaneous Lymphomas

scholarly journals The 2008 WHO classification of lymphomas: implications for clinical practice and translational research

Hematology ◽  
2009 ◽  
Vol 2009 (1) ◽  
pp. 523-531 ◽  
Author(s):  
Elaine S. Jaffe
Keyword(s):  
T Cell ◽  
Who Classification ◽  
Cell Lymphoma ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
Lymphoid Tissues ◽  
T Cell Lymphomas ◽  
Specific Factors ◽  
Definition Of

Abstract The 4th edition of the WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues published in 2008 builds upon the success of the 2001 3rd edition; new entities are defined, and solutions for problematic categories are sought. Recent studies have drawn attention to the biological overlap between classical Hodgkin lymphoma (CHL) and diffuse large B-cell lymphomas (DLBCL). Similarly, there is a greater appreciation of the borderlands between Burkitt lymphoma and DLBCL. Strategies for the management of these borderline lesions are proposed. Additionally, age-specific and site-specific factors play an important role in the definition of several new entities, which also have biological underpinnings. Among the peripheral T-cell lymphomas (PTCL), more precise definitions were introduced for several entities, including anaplastic large cell lymphoma, angioimmunoblastic T-cell lymphoma, enteropathy-associated T-cell lymphoma, and subcutaneous panniculitis-like T-cell lymphoma. Several new variants of primary cutaneous T-cell lymphomas are proposed. Finally, the subclassification and categorization of the most common lymphoma subtypes, follicular lymphoma (FL) and DLBCL, were altered to enhance diagnostic accuracy and aid in clinical management. The 2008 WHO classification also draws attention to early events in lymphomagenesis. These lesions help delineate the earliest steps in neoplastic transformation and generally mandate a conservative therapeutic approach. The 2001 classification was rapidly adopted for clinical trials and successfully served as a common language for scientists comparing genetic and functional data. The modifications made in the 2008 classification are the result of this successful partnership among pathologists, clinicians, and biologists, but are only a stepping stone to the future.

Primary cutaneous lymphomas: applicability of current classification schemes (European Organization for Research and Treatment of Cancer, World Health Organization) based on clinicopathologic features observed in a large group of patients

Blood ◽  
2002 ◽  
Vol 99 (3) ◽  
pp. 800-805 ◽  
Author(s):  
Regina Fink-Puches ◽  
Paulus Zenahlik ◽  
Barbara Bäck ◽  
Josef Smolle ◽  
Helmut Kerl ◽  
...  
Keyword(s):  
T Cell ◽  
B Cell ◽  
Who Classification ◽  
Cell Lymphoma ◽  
World Health ◽  
European Organization ◽  
Diagnostic Categories ◽  
Cutaneous Lymphomas ◽  
Health Organization

Abstract Classification of primary cutaneous lymphomas (PCLs) is the subject of ongoing controversy. Based on a series of 556 patients, the applicability of the European Organization for Research and Treatment of Cancer (EORTC) classification for PCLs was assessed and compared to the proposed World Health Organization (WHO) classification of hematologic malignancies. The large majority of patients could be properly classified according to the scheme proposed by the EORTC. Comparison of estimated 5-year survival for specific diagnostic categories of PCLs demonstrated nearly complete concordance of the present results with those of the EORTC study for most of the indolent cutaneous T-cell lymphomas and cutaneous B-cell lymphomas, whereas differences were found for mycosis fungoides-associated follicular mucinosis and Sezary syndrome. A few patients with newly described entities (CD8+ epidermotropic cytotoxic T-cell lymphoma, primary cutaneous natural killer/T-cell lymphoma) could not be classified according to the EORTC scheme. Comparison of the EORTC with the WHO classification showed that the EORTC scheme allows a more precise categorization of the patients, especially for cutaneous B-cell lymphoma. In conclusion, the study confirmed that the EORTC classification allows a better management of patients with PCL. Small amendments to that classification should be carried out to account for recently described entities and to unify some of the diagnostic categories.

scholarly journals The 2008 WHO classification of lymphomas: implications for clinical practice and translational research

Hematology ◽  
2009 ◽  
Vol 2009 (1) ◽  
pp. 523-531 ◽  
Author(s):  
Elaine S. Jaffe
Keyword(s):  
T Cell ◽  
Who Classification ◽  
Cell Lymphoma ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
Lymphoid Tissues ◽  
T Cell Lymphomas ◽  
Specific Factors ◽  
Definition Of

The 4th edition of the WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues published in 2008 builds upon the success of the 2001 3rd edition; new entities are defined, and solutions for problematic categories are sought. Recent studies have drawn attention to the biological overlap between classical Hodgkin lymphoma (CHL) and diffuse large B-cell lymphomas (DLBCL). Similarly, there is a greater appreciation of the borderlands between Burkitt lymphoma and DLBCL. Strategies for the management of these borderline lesions are proposed. Additionally, age-specific and site-specific factors play an important role in the definition of several new entities, which also have biological underpinnings. Among the peripheral T-cell lymphomas (PTCL), more precise definitions were introduced for several entities, including anaplastic large cell lymphoma, angioimmunoblastic T-cell lymphoma, enteropathy-associated T-cell lymphoma, and subcutaneous panniculitis-like T-cell lymphoma. Several new variants of primary cutaneous T-cell lymphomas are proposed. Finally, the subclassification and categorization of the most common lymphoma subtypes, follicular lymphoma (FL) and DLBCL, were altered to enhance diagnostic accuracy and aid in clinical management. The 2008 WHO classification also draws attention to early events in lymphomagenesis. These lesions help delineate the earliest steps in neoplastic transformation and generally mandate a conservative therapeutic approach. The 2001 classification was rapidly adopted for clinical trials and successfully served as a common language for scientists comparing genetic and functional data. The modifications made in the 2008 classification are the result of this successful partnership among pathologists, clinicians, and biologists, but are only a stepping stone to the future.

Prognostic Factors in Aggressive, Non-Primary Cutaneous Peripheral T-Cell Lymphoma in Peru: A Study of 227 Cases.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 5021-5021
Author(s):  
Brady Beltran ◽  
Domingo Morales ◽  
Pilar Quinones ◽  
Carlos Desposorio ◽  
Lubomir Sokol ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
T Cell ◽  
Clinical Characteristics ◽  
Who Classification ◽  
Median Overall Survival ◽  
Cell Lymphoma ◽  
Prognostic Index ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma

Abstract Abstract 5021 Background Peripheral T-cell lymphoma (PTCL) is a heterogeneous family of entities with a worse prognosis, stage by stage, than their B-cell counterparts. We have previously reported that Peruvian population has a higher incidence of PTCL, akin to the Asian population. The goal of this study is to therefore investigate the clinical characteristics and prognostic factors in patients with PTCL in a reference center in Peru. Methods A total of 227 cases of aggressive, non-primary cutaneous PTCL diagnosed between January 1997 and December 2008 were reviewed, reappraised according to their morphological, immunological and clinical characteristics, and reclassified according to the new WHO classification of lymphoid neoplasms. Kaplan-Meier method was used to estimate survival curves, which were compared using the log-rank test. The multivariate analysis was performed using the Cox proportional-hazard regression test. Results The mean age at diagnosis was 57 years (range 14 – 92 years) with a male-to-female ratio of 1:1. According to the new WHO classification of lymphoid neoplasms, 97 cases (43%) were classified as adult T-cell leukemia/lymphoma (ATLL), 84 cases (37%) as PTCL, unspecified (PTCLU), 28 cases (12%) as anaplastic large cell lymphoma (ALCL), 10 cases (4%) as extranodal NK/T lymphoma, nasal type, 4 cases (2%) as angioimmunoblastic lymphoma and 3 cases (1%) were diagnosed with more rare PTCL subtypes. Fifteen percent were stage I, 12% stage II, 16% stage III and 56% stage IV. Distribution based on the International Prognostic Index (IPI) score was: low 24%, low-intermediate 24.5%, intermediate-high 26% and high 25.5%. B symptoms were present in 64% of patients. The median overall survival (OS) for the whole group was 8.4 months, the 2-year OS was 34% and the 5-year OS was 27%. ATLL patients showed a median overall survival of 6.1 months, PTCL of 11.4 months, ALCL of 12.4 months and extranodal NK/T cell lymphoma of 7.8 months. The IPI score, the Prognostic Index for PTCLU (PIT) score and the presence of B symptoms showed statistical significance in both the univariate and the multivariate survival analysis (p=0.001, p=0.005 and p=0.005, respectively). Conclusions The distribution of PTCL subtypes in Peru is comparable to the series of patients reported in Asian countries. ATLL and PTCL-U are the most frequent aggressive, non-primary cutaneous PTCL subtypes found. PTCL tends to present with advanced stages and has a poor prognosis, with a 5-year OS of less than 30%. In the multivariate analysis, the IPI score, the PIT score and presence of B symptoms were independent prognostic factors for survival in PTCL. Disclosures No relevant conflicts of interest to declare.

scholarly journals Clinical Characteristics and Outcomes of an Analysis of a Single Institution Experience of the 2017 World Health Organization (WHO) Classification of Post-Transplant Lymphoproliferative Disorders (PTLD)

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 456-456
Author(s):  
Thomas M. Habermann ◽  
Angelo Fama ◽  
Kay M. Ristow ◽  
Matthew J. Maurer ◽  
William R. Macon ◽  
...  
Keyword(s):  
Overall Survival ◽  
T Cell ◽  
B Cell ◽  
Mayo Clinic ◽  
Research Funding ◽  
Who Classification ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Non Destructive

Abstract Background: PTLD is the most common malignancy, other than non-melanoma skin cancer, complicating solid organ transplantation (SOT) and has been one of the most commonly observed fatal consequences in SOT. The clinical presentations, management strategies, histologies, causes of death and outcomes are diverse (Dierickx D, Habermann TM. N Engl J Med 2018;378:549-62). The 2017 WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues has redefined the categories as non-destructive PTLDs (plasmacytic hyperplasia, infectious mononucleosis, and florid follicular hyperplasia), polymorphic PTLD, monomorphic PTLD: B-cell neoplasms (diffuse large B-cell lymphoma, Burkitt lymphoma, high-grade B-cell lymphoma, plasmablastic lymphoma, plasma cell myeloma, plasmacytoma, and other) and T-cell neoplasms (peripheral T-cell lymphoma NOS, hepatosplenic T-cell lymphoma, other), and classic Hodgkin lymphoma (CHL) PTLD. We report the outcomes and long-term follow-up of patients from a single institution based on these categories whose pathology was retrospectively reviewed and reclassified based on the WHO 2017 classification. Methods: Patients with SOT who were diagnosed with PTLD at Mayo Clinic (Rochester, MN) were identified through the Mayo Clinic Lymphoma Data base and the University of Iowa/Mayo Clinic SPORE Molecular Epidemiology Resource (MER). The histology was re-reviewed in 80% of the cases (RLK) and classified according to the WHO Classification of Tumours of Haematopoietic an Lymphoid Tissues 2017. Cases were considered "PTLD, unclassified" if the histology could not be confidently classified based on pathology material available for review. Indolent small B-cell lymphomas were not included among the PTLDs except for EBV-positive marginal zone lymphoma. Cox proportional hazards models were used to assess the association of clinical factors in overall survival (OS). Results: 233 patients diagnosed with PTLD between 1987 and 2017 were identified. The median age at the time of diagnosis of PTLD was 54 years (range 16 to 84) with 85 patients (36%) over the age of 60. 156 (67%) were male. The transplanted organs were kidney (41%), kidney/pancreas (5%), liver (29%), heart (9%), lung (7%), and other (8%). PTLD occurred late (more than one year after transplantation) in 66%. There were 69 stage I, 19 stage II, 8 stage III, and 128 stage IV patients. 84% presented with extranodal disease. 21% had involvement of the engrafted organ. 64% of the patients developed a PTLD that was EBV positive by in situ hybridization. Initial approaches to management included reduction of immunosuppression (N=55), chemotherapy/immunochemotherapy (N=71), reduction in immunosuppression with rituximab (N=58), single agent rituximab (N=14), and radiation therapy (N=5). At a median follow-up of 87 months (range 9-289), 139 (60%) patients had died. All six CHL-PTLD patients are alive, two of whom had an event. The median overall survival (OS) was 85 months (95% CI: 39-144) in 177 monomorphic B-cell lymphoma PTLD, 95.5 months (95% CI: 40-not reached) in 24 polymorphic PTLD, and 60 months in 8 non-destructive PTLD cases. In contrast, the median overall survival was 12 months (95% CI: 4-200) in 8 monomorphic T cell and 3 months (95% CI: 1-unreached) in 9 unclassified PTLD cases. Conclusion: PTLD is a heterogeneous group of immunodeficiency-associated lymphoproliferative disorders. The overall survival in non-destructive, polymorphic, and monomorphic PTLD were similar. Monomorphic T/NK cell types had inferior outcomes. Figure. Figure. Disclosures Maurer: Morphosys: Research Funding; Nanostring: Research Funding; Celgene: Research Funding. Ansell:Celldex: Research Funding; Merck & Co: Research Funding; Takeda: Research Funding; LAM Therapeutics: Research Funding; Seattle Genetics: Research Funding; Trillium: Research Funding; Bristol-Myers Squibb: Research Funding; Affimed: Research Funding; Regeneron: Research Funding; Pfizer: Research Funding. Cerhan:Celgene: Research Funding; Nanostring: Research Funding; Jannsen: Other: Scientific Advisory Board.

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