scholarly journals 51 Small cell/lymphohistiocytic morphology is associated with CD8 positivity, retained T cell markers, a trend of decreased PD-L1 expression, but not outcome in adults with ALK+ ALCL

2020 ◽  
Vol 8 (Suppl 3) ◽  
pp. A54-A54
Author(s):  
Mahsa Khanlari ◽  
Shaoying Li ◽  
Roberto N Miranda ◽  
Swaminathan Iyer ◽  
Cameron Yin ◽  
...  
Keyword(s):  
T Cell ◽  
Anaplastic Large Cell Lymphoma ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Small Cell ◽  
Adult Patients ◽  
World Health ◽  
Proliferation Index ◽  
Cell Markers ◽  
Large Cell Lymphoma

BackgroundSeveral morphologic patterns of ALK+ anaplastic large cell lymphoma (ALCL) are recognized: common, small cell, lymphohistiocytic, Hodgkin-like, and composite patterns.1 Small cell (SC) and lymphohistiocytic (LH) patterns are thought to be closely associated with poorer outcome in children with ALK+ ALCL.2 However, the clinicopathologic and prognostic features of SC/LH patterns of ALK+ ALCL are not yet reported in adults. Recently, we found PD-L1 expression in a large subset of ALK+ ALCL cases, however, PD-L1 expression in SC/LH versus non-SC/LH ALCL has not been reported.MethodsAmong 102 adult patients with ALK+ ALCL seen at our institution from January 1, 2007 through August 30, 2018, 18 (18%) cases had a SC and/or LH pattern. The clinical, pathologic, and outcome data were compared between SC/LH and non-SC/LH ALK+ ALCL cases using Fisher’s exact test. Overall survival (OS) was analyzed using the Kaplan-Meier method and compared using the log-rank test.ResultsThere were no significant differences in clinical features including age, gender, nodal versus extranodal involvement, B symptoms, stage, leukocytosis/lymphocytosis, and serum LDH levels between patients with SC/LH versus non-SC/LH ALK+ ALCL. Compared to non-SC/LH cases, SC/LH ALCL was more often positive for CD2 (92% vs. 36%, p = 0.0007), CD3 (81% vs. 15%, p = 0.0001), CD7 (80% vs. 37%, p = 0.03), and CD8 (54% vs. 7%, p = 0.0006). SC/LH ALCL showed a trend of decreased PD-L1 expression than non-SC/LH cases (24% vs. 46%, p = 0.11). There were no significant differences in the expression of CD4, CD5, CD25, CD43, CD45, CD56, TCR A/B, TCR G/D, cytotoxic markers, EMA, Ki-67 proliferation index. The induction chemotherapy and response rate in patients with SC/LH ALK+ ALCL were similar to patients with non-SC/LH ALK+ ALCL. After a median follow-up of 30.5 months (range, 0.3–224 months), there was no significant difference in OS between patients with SC/LH versus non-SC/LH ALK+ ALCL (p = 0.88).ConclusionsIn adults with ALK+ALCL, the SC/LH morphologic pattern is associated with a CD8+ T cell immunophenotype and retention of expression of T cell markers (CD2, CD3, and CD7). The trend of decreased PD-L1 expression in SC/LH ALCL suggests that these patients may not be ideal candidates for PD-L1 immunotherapy. The SC/LH patterns of ALK+ ALCL have no impact on the prognosis of adult patients which is in contrast to the reported association of the SC/LH patterns with poorer outcome in children with ALK+ ALCL.Ethics ApprovalThe study was approved by the Institutional Review Board at MD Anderson Cancer Center, Approval number: PA16-0897ReferencesSwerdlow SH, Campo E, The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016;127:2375–2390.Brugières L, Deley MC, CD30 (+) anaplastic large-cell lymphoma in children: Analysis of 82 patients enrolled in two consecutive studies of the French Society of Pediatric Oncology. Blood 1998;92:3591–3598.

scholarly journals How I treat breast implant–associated anaplastic large cell lymphoma

Blood ◽  
2018 ◽  
Vol 132 (18) ◽  
pp. 1889-1898 ◽  
Author(s):  
Neha Mehta-Shah ◽  
Mark W. Clemens ◽  
Steven M. Horwitz
Keyword(s):  
T Cell ◽  
Anaplastic Large Cell Lymphoma ◽  
Cell Lymphoma ◽  
Breast Implant ◽  
Large Cell ◽  
World Health ◽  
Team Approach ◽  
Textured Surface ◽  
Complete Surgical Excision ◽  
Large Cell Lymphoma

Abstract Breast implant–associated anaplastic large cell lymphoma (BIA-ALCL) is a recently described form of T-cell non-Hodgkin lymphoma now formally recognized by the World Health Organization classification of lymphoid neoplasms. The disease most often presents with a delayed seroma around the breast implant, almost exclusively with a textured surface, and manifests with breast pain, swelling or asymmetry, capsular contracture, but can also present with a breast mass, and lymph node involvement. The prognosis of BIA-ALCL is favorable compared with many other subtypes of systemic T-cell lymphoma; however, unlike other non-Hodgkin lymphomas, complete surgical excision for localized disease is an important part of the management of these patients. In this paper, we share our recommendations for a multidisciplinary team approach to the diagnosis, workup, and treatment of BIA-ALCL in line with consensus guidelines by the National Comprehensive Cancer Network.

Survival Analysis of Anaplastic Large Cell Lymphoma, Systemic and Cutaneous-Types: Report from the International T-Cell Lymphoma Project.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 293-293
Author(s):  
Kerry J. Savage ◽  
Julie M. Vose ◽  
Nancy Lee Harris ◽  
Keyword(s):  
T Cell ◽  
Anaplastic Large Cell Lymphoma ◽  
Cell Lymphoma ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
World Health ◽  
Anaplastic Lymphoma ◽  
Limited Stage ◽  
Significant Difference ◽  
Large Cell Lymphoma

Abstract The WHO (World Health) Organization Classification recognizes two distinct subtypes of anaplastic large cell lymphoma: Primary systemic and Primary cutaneous types, which have differences in immunophenotype, genetics, and clinical behavior. It is now known that approximately 60% of systemic ALCLs express the anaplastic lymphoma kinase (ALK) protein (ALK-pos) and have a significantly superior survival to ALK-neg cases. Since ALK-neg ALCL appear to have a prognosis similar to peripheral T-cell lymphoma unspecified (PTCL-U), it has been suggested that they should be classified as PTCL-U. Herein, we report the clinical features of newly diagnosed systemic and cutaneous ALCL from the International T-cell Lymphoma Study Group. Materials and Methods: 186 cases of ALCL were identified by the WHO disease definitions: systemic ALCL 163 (88%) (ALK-pos 91 (56%), 72(44%) ALK-neg), and 23 (17%) cutaneous ALCL (cut ALCL). The median age of ALK-pos, ALK-neg and cut ALCL was 32, 57.5 and 54, respectively. There was a male predominance for all subtypes. Most cases of systemic ALCL presented with stage III or IV disease (64% ALK-pos, 58% ALK-neg) and in contrast, 87% of cut ALCL had localized disease. The majority of patients with systemic ALCL were treated with CHOP-type chemotherapy. Most patients with cut ALCL (91%) received additional therapy: 13 (62%) CHOP-type chemotherapy, 11(52%) chemoradiation, 4 (19%) radiation alone. Results: The 5y failure free survival (FFS) and overall survival (OS) was superior for ALK-pos ALCL (70.5% and 58%) compared to ALK-neg ALCL (49% and 36%) (p=.022 and p=.014 for FFS and OS, respectively). Comparison of ALK-pos (n=16) and ALK-neg ALCL (n=23) patients with limited stage disease (defined as stage I or II, no B symptoms and non-bulky) failed to demonstrate a significant difference in FFS (p=.54) or OS (p=.21). Both ALK-pos and ALK-neg ALCL had a superior FFS (ALK-pos p< .001; ALK-neg p=.012) and OS (ALK-pos p<.001; ALK-neg p=.032) than PTCL-U. In contrast to PTCL-U, an apparent plateau was observed on the FFS curve for ALK-neg ALCL. For cut ALCL, the 5y FFS and OS was 90% and 57%, superior to systemic ALCL. The administration of chemotherapy did not appear to impact outcome in patients with cut ALCL (p=.64). Among the prognostic factors analyzed, the international prognostic index (IPI) was the most effective for defining risk categories in ALK-neg ALCL. For ALK-pos ALCL both the IPI and anemia (Hb < 11.0 g/L) were effective in risk-group stratification in multivariate analysis. Conclusions: Similar to prior reports, ALK-pos ALCL has a superior outcome to ALK-neg ALCL. For limited stage patients, this survival difference is not apparent, suggesting that a small subgroup of patients with ALK-neg ALCL may have a more favorable prognosis, similar to ALK-pos ALCL. The IPI is effective in both ALK-neg and ALK-pos ALCL at risk stratification. Finally, contrary to prior reports, ALK-neg ALCL patients appear to have a superior outcome to PTCL-U and an apparent plateau in the FFS curve. These results suggest that ALK-neg ALCL should still be distinguished from both ALK-pos ALCL and PTCL-U.

PAX-5 Positivity in Anaplastic Lymphoma Kinase–Negative Anaplastic Large Cell Lymphoma: A Case Report and Review of Literature

2016 ◽  
Vol 25 (4) ◽  
pp. 333-338 ◽  
Author(s):  
Indu Arun ◽  
Paromita Roy ◽  
Neeraj Arora ◽  
Saurabh Jayant Bhave ◽  
Reena Nair ◽  
...  
Keyword(s):  
T Cell ◽  
B Cell ◽  
Anaplastic Large Cell Lymphoma ◽  
Anaplastic Lymphoma Kinase ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Large Cell ◽  
World Health ◽  
Anaplastic Lymphoma ◽  
Large Cell Lymphoma

Anaplastic lymphoma kinase (ALK)–negative anaplastic large cell lymphoma (ALCL) is a subtype of T-cell lymphomas that may mimic several other malignancies morphologically. With the help of immunohistochemistry, most cases of ALCL can be diagnosed on the basis of expression of T-cell lineage associated antigens. However, aberrations in the expression of immunohistochemical markers pose diagnostic challenges. The morphological and immunophenotypic features of ALCL show considerable overlap with classical Hodgkin lymphoma (CHL), which is a B-cell lymphoma. The 2008 World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues suggests that staining for the B-cell transcription factor, paired box 5 (PAX-5), is helpful in differentiating between them, as it is weakly positive in most CHL and should be negative in ALCL. We report a rare case of ALK-negative ALCL, which was positive for PAX-5 and CD15, mimicking CHL by immunohistochemistry, resulting in a diagnostic dilemma.

scholarly journals The Pathological Spectrum of Systemic Anaplastic Large Cell Lymphoma (ALCL)

Cancers ◽  
2018 ◽  
Vol 10 (4) ◽  
pp. 107 ◽  
Author(s):  
Ivonne Montes-Mojarro ◽  
Julia Steinhilber ◽  
Irina Bonzheim ◽  
Leticia Quintanilla-Martinez ◽  
Falko Fend
Keyword(s):  
T Cell ◽  
Anaplastic Large Cell Lymphoma ◽  
Cell Lymphoma ◽  
Breast Implant ◽  
Large Cell ◽  
World Health ◽  
Anaplastic Lymphoma ◽  
Molecular Features ◽  
Starting Point ◽  
Large Cell Lymphoma

Anaplastic large cell lymphoma (ALCL) represents a group of malignant T-cell lymphoproliferations that share morphological and immunophenotypical features, namely strong CD30 expression and variable loss of T-cell markers, but differ in clinical presentation and prognosis. The recognition of anaplastic lymphoma kinase (ALK) fusion proteins as a result of chromosomal translocations or inversions was the starting point for the distinction of different subgroups of ALCL. According to their distinct clinical settings and molecular findings, the 2016 revised World Health Organization (WHO) classification recognizes four different entities: systemic ALK-positive ALCL (ALK+ ALCL), systemic ALK-negative ALCL (ALK− ALCL), primary cutaneous ALCL (pC-ALCL), and breast implant-associated ALCL (BI-ALCL), the latter included as a provisional entity. ALK is rearranged in approximately 80% of systemic ALCL cases with one of its partner genes, most commonly NPM1, and is associated with favorable prognosis, whereas systemic ALK− ALCL shows heterogeneous clinical, phenotypical, and genetic features, underlining the different oncogenesis between these two entities. Recognition of the pathological spectrum of ALCL is crucial to understand its pathogenesis and its boundaries with other entities. In this review, we will focus on the morphological, immunophenotypical, and molecular features of systemic ALK+ and ALK− ALCL. In addition, BI-ALCL will be discussed.

scholarly journals Pitfalls in the Diagnosis of Anaplastic Large Cell Lymphoma with a Small Cell Pattern

2013 ◽  
Vol 2013 ◽  
pp. 1-6
Author(s):  
Rowan L. Grigoropoulos ◽  
Penny Wright ◽  
Mars B. van t'Veer ◽  
Mike A. Scott ◽  
George A. Follows
Keyword(s):  
T Cell ◽  
Anaplastic Large Cell Lymphoma ◽  
Cell Lymphoma ◽  
Rare Condition ◽  
Antigen Expression ◽  
Large Cell ◽  
Small Cell ◽  
Anaplastic Lymphoma ◽  
Cell Pattern ◽  
Large Cell Lymphoma

Anaplastic large cell lymphoma with a small cell pattern is a rare T-cell lymphoma. This condition is more frequently seen in younger patients and should be considered when patients present with leucocytosis and constitutional symptoms. In this report, we describe our diagnostic work-up for one such case using blood, lymph node, and bone marrow aspirate samples, highlighting the variability of antigen expression seen in different sample types and methodologies. This case shows the importance of having a high index of suspicion and assessing CD30 and anaplastic lymphoma kinase expression in all suspected T-cell neoplasms even though this rare condition is not necessarily expected.

scholarly journals Update on the Classification and Diagnostic Approaches of Mature T-Cell Lymphomas

2021 ◽  
Author(s):  
Xiaohui Zhang ◽  
Jiehao Zhou ◽  
Xin Han ◽  
Endi Wang ◽  
Linsheng Zhang
Keyword(s):  
T Cell ◽  
World Health Organization ◽  
Anaplastic Large Cell Lymphoma ◽  
Cell Lymphoma ◽  
Molecular Genetic ◽  
Large Cell ◽  
World Health ◽  
T Cell Lymphomas ◽  
Large Cell Lymphoma ◽  
Health Organization

Context.— In the 2017 revised World Health Organization classification of tumors of hematopoietic and lymphoid tissues, some mature T-cell lymphomas are reclassified and a few new provisional entities are established based on new data from clinical and laboratory studies. T follicular helper cell lymphoma is identified by T follicular helper cell markers. Anaplastic large cell lymphoma, ALK negative, is a better-defined entity based on genetic abnormalities, and breast implant–associated anaplastic large cell lymphoma is recognized as a provisional entity. The gastrointestinal T-cell lymphomas are reclassified, with addition of a new provisional entity, indolent T-cell lymphoproliferative disorder of the gastrointestinal tract, characterized by an indolent clinical course. Objective.— To review the diagnostic approaches of reclassified and newly established entities of mature T-cell lymphomas, focusing on significant immunophenotypic features and molecular genetic abnormalities. Relevant new discoveries after the publication of the 2017 World Health Organization classification are included. Data Sources.— Information from the literature most relevant to 2017 World Health Organization revised classification and publications after 2016. Conclusions.— Incorporating clinical, morphologic, and immunophenotypic features usually provides sufficient evidence to reach a preliminary diagnosis of mature T-cell lymphoma. Molecular genetic studies can be very helpful for the final diagnosis and classification, especially in challenging cases. Some molecular genetic features have been found in breast implant–associated anaplastic large cell lymphoma, distinct from anaplastic large cell lymphoma, ALK negative. Immunohistochemical staining of 4 markers may enable further subtyping of peripheral T-cell lymphomas.

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