Blood.
Of the patients with advanced-stage disease, only one had stage IV disease. Newest Articles This site needs JavaScript to work properly. The Lymphoid Cancer Database of the British Columbia Cancer Agency was searched to identify all patients diagnosed with NLPHL between 1965 and 2006. The importance of pathologic rereview of older cases previously classified as NLPHL before the routine availability of immunohistochemistry cannot be overemphasized. It has long been recognized that large-cell lymphoma can occur either at diagnosis or as a subsequent relapse in patients with NLPHL.10,11,16 The incidence has been reported to range up to 10%; however, in many cases, the diagnosis of NLPHL was based on obsolete classification systems, and follow-up has been quite limited. 5 P50 CA097274/CA/NCI NIH HHS/United States. Leuk Lymphoma. | Epub 2019 Feb 15. Between 1970 and 2011, 222 consecutive adult patients with new untreated NLPHL were identified. P= .03) were significantly associated with increased risk of transformation. In a multivariate analysis, use of any prior chemotherapy ( ITALIC! | In summary, this large study with long-term follow-up confirms that patients with NLPHL have a substantial risk of future transformation to aggressive lymphoma, which can occur decades after the primary diagnosis of NLPHL with no apparent plateau in risk. Median age at diagnosis was 40 years, and 146 (66%) were males. With a median follow-up time of living patients of 6.5 years (range, 2.5 to 32 years), 13 patients (14%) experienced transformation to aggressive lymphoma have (Table 2). Provision of study materials or patients: Joseph M. Connors, Randy D. Gascoyne, Brian Skinnider, Collection and assembly of data: Mubarak Al-Mansour, Randy D. Gascoyne, Brian Skinnider, Kerry J.
P= .04) and splenic involvement ( ITALIC! In the initial screen, 150 patients with NLPHL were identified. NIH A number of reports have shown a propensity of nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) to transform into diffuse large B-cell lymphoma (DLBCL). Baseline Clinical Characteristics of Patients at the Time of Transformation to Aggressive Lymphoma and Treatment Received. The 5-year overall survival (OS) in those with transformed disease was 76.4%, and transformation did not adversely affect OS when compared with patients who did not experience transformation. Hematology Am Soc Hematol Educ Program. P= .04) and splenic involvement ( ITALIC! 2004 Aug;45(8):1551-7. doi: 10.1080/1042819031000149421. Blood.
Two patients underwent surgery alone for stage IA disease because the diagnosis of NLPHL was established retrospectively on pathologic rereview (Table 1). At diagnosis of NLPHL, the median age was 37 years (range, 15 to 77 years), and the majority of patients were male (73%) with stage I or II disease (67%) and a good performance status (0 or 1; 91%; Table 1). Long-term data on the incidence and outcomes of transformed NLPHL are lacking. The majority of the patients present with early-stage disease, and treatment with local radiation provides excellent disease control and overall survival (OS). USA.gov. DOI: 10.1200/JCO.2009.24.9516 Journal of Clinical Oncology - Int J Mol Sci. With a median follow-up time for living patients of 6.5 years (range, 2.5 to 33 years), 13 patients (14%) experienced transformation to aggressive lymphoma (median time to transformation, 8.1 years; range, 0.35 to 20.3 years). [Update on nodular lymphocyte predominant Hodgkin's lymphoma and related lesions]. In one of these patients, a repeat biopsy demonstrated NLPHL with an area of atypical sclerosis suggestive, but not definitive, of an evolving TCRBCL. Clinically, the latter patient had an LDH ≥ 4× ULN and rapid clinical deterioration. Seventeen patients (7.6%) developed a transformation to DLBCL. CancerLinQ 2017 Feb;38(1):3-10. doi: 10.1007/s00292-016-0257-6. Between 1970 and 2011, 222 consecutive adult patients with new untreated NLPHL were identified. Insights Imaging. Fig 2. 2019 Feb 22;10(1):25. doi: 10.1186/s13244-019-0705-y. We evaluated the frequency of transformation in all patients diagnosed with NLPHL at the British Columbia Cancer Agency with long-term follow-up. Most patients received an ABVD-like regimen (ABVD, n = 19; or MOPP/ABV, n = 4), and three patients received MOPP followed by radiotherapy. Conquer Cancer Foundation The 5-year overall survival (OS) in those with transformed disease was 76.4%, and transformation did not adversely affect OS when compared with patients who did not experience transformation. 2017 Dec 8;2017(1):324-328. doi: 10.1182/asheducation-2017.1.324. Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a rare entity distinct from classical Hodgkin lymphoma (cHL), both in terms of histopathological features and clinical presentation. 2010 Feb 1;116(3):631-9. doi: 10.1002/cncr.24819. After pathologic review, 95 patients with NLPHL were confirmed. Ten patients had a pathologic diagnosis of DLBCL (DLBCL, n = 8; T-cell–rich B-cell lymphoma [TCRBCL], n = 2), and in one patient, a fine-needle aspiration was performed that demonstrated an aggressive B-cell lymphoma, but further subclassification was not possible as a result of limited pathologic material. On pathologic reassessment, 55 patients were excluded for the following reasons: classical Hodgkin's lymphoma (total, n = 20; mixed cellularity, n = 7, lymphocyte rich, n = 9; lymphocyte depleted, n = 1; nodular sclerosis, n = 1; not classifiable, n = 2); composite or gray-zone lymphoma (n = 4) or discordant lymphoma (n = 1); NHL (DLBCL, n = 4; not classifiable, n = 1); and paraffin blocks and/or slides not available for review (n = 25). HHS Please enable it to take advantage of the complete set of features! 2020 Jan;52(1):142-153. doi: 10.1016/j.pathol.2019.10.003. Pathology. Clipboard, Search History, and several other advanced features are temporarily unavailable. Chimeric Antigen Receptor (CAR) T Cell Therapy in Acute Myeloid Leukemia (AML). Future studies should separate concurrent and sequential patients to further understand any prognostic differences. A number of reports have shown a propensity of nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) to transform into diffuse large B-cell lymphoma (DLBCL). 2020 Jan;52(1):142-153. doi: 10.1016/j.pathol.2019.10.003. Pathologe. †Two patients had etoposide substitution for the anthracycline. A number of reports have shown a propensity of nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) to transform into diffuse large B-cell lymphoma (DLBCL). Nodular lymphocyte-predominant Hodgkin lymphoma: a unique disease deserving unique management. Al-Mansour M, Connors JM, Gascoyne RD, Skinnider B, Savage KJ. A comprehensive analysis of the actively maintained Mayo Clinic Lymphoma Database was performed. COVID-19 is an emerging, rapidly evolving situation. In univariate analysis, splenic involvement at diagnosis of NLPHL (P = .006) was a strong risk factor for future transformation, and there was a trend toward an increased risk with advanced-stage disease (P = .057; Table 4). See this image and copyright information in PMC. NLPHL was identified as a distinct subtype of HL in the Revised European-American Lymphoma (REAL) classification and in the WHO classification of lymphomas1 based on unique histopathologic and clinical characteristics that distinguish it from classical HL. Permissions, Authors *Nineteen patients were missing LDH (n = 19 nontransformed).
Patients with NLPHL are usually male, with a median age of 30 to 40 years, and usually present with slowly progressive, localized peripheral lymphadenopathy.2 Bulky disease, “B” symptoms, mediastinal or abdominal involvement, and extranodal disease are uncommon.3 NLPHL is characterized by a nodular or a nodular and diffuse proliferation of large neoplastic lymphocyte-predominant (LP) cells (so-called popcorn cells), which were previously referred to as lymphocytic and histiocytic or Reed-Sternberg cell variants.4 In contrast to classical Hodgkin's Reed-Sternberg cells, LP cells express the B-cell markers CD19, CD20, and CD79, as well as immunoglobulins, J chain, and epithelial membrane antigen, but they do not express CD15 and CD30.3 This B-cell phenotype and demonstration of clonally arranged immunoglobulin genes with ongoing mutations suggest a close relationship with B-cell non-Hodgkin's lymphoma (NHL).5. Diffuse large B-cell lymphoma arising in nodular lymphocyte predominant Hodgkin lymphoma: a report of 21 cases from the Nebraska Lymphoma Study Group.
Consistent with this, there were no relapses seen after 2 years, highlighting a different natural history than de novo DLBCL or follicular lymphoma that subsequently transformed. Long-term data on the incidence and outcomes of transformed NLPHL are lacking. Patients with NLPHL had the following characteristics at diagnosis: median age of 37 years, 73% male, and 68% stage I or II disease.
2010 Feb 1;116(3):631-9. doi: 10.1002/cncr.24819. Hematology Am Soc Hematol Educ Program. Long-term data on the incidence and outcomes of transformed NLPHL are lacking. National Center for Biotechnology Information, Unable to load your collection due to an error, Unable to load your delegates due to an error.
A revised European-American classification of lymphoid neoplasms: A proposal from the International Lymphoma Study Group, Clinical presentation, course, and prognostic factors in lymphocyte-predominant Hodgkin's disease and lymphocyte-rich classical Hodgkin's disease: Report from the European Task Force on Lymphoma Project on Lymphocyte-Predominant Hodgkin's Disease, Lymphocyte predominant Hodgkin's disease: Clinical presentation and results of treatment, Origin of nodular lymphocyte-predominant Hodgkin's disease from a clonal expansion of highly mutated germinal-center B cells, Clinical features of nodular paragranuloma (Hodgkin's disease, lymphocyte predominance type, nodular), Non-Hodgkin's lymphoma arising in patients treated for Hodgkin's disease in the BNLI: A 20-year experience—British National Lymphoma Investigation, Further phenotypic evidence that nodular, lymphocyte-predominant Hodgkin's disease is a large B-cell lymphoma in evolution, Nodular paragranuloma can transform into high-grade malignant lymphoma of B type, Hodgkin's disease, lymphocytic predominance nodular. Pathologe. Get the latest research from NIH: https://www.nih.gov/coronavirus. A small proportion of patients had B symptoms (8%) or an elevated LDH (7%), and 12 patients (12%) had splenic involvement diagnosed by either staging splenectomy (n = 6) or imaging (n = 6). Blood. There were no relapses after 2 years, including NLPHL (Fig 2). published online before print
The risk of transformation in patients with NLPHL to DLBCL is substantial and underappreciated. There was one toxic death as a result of febrile neutropenia during therapy for NLPHL and no deaths as a result of refractory NLPHL. Nodular lymphocyte-predominant Hodgkin's Lymphoma (NLPHL) accounts for approximately 5% of all Hodgkin's lymphoma (HL). The final cohort of 95 patients with NLPHL had the typical clinical features encountered in this disease, namely male predominance, early stage, and few adverse prognostic factors.26–28 Consistent with prior reports, overall, the prognosis of our NLPHL patients was favorable, with a 10-year OS rate of 91%.
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