Clinical and histopathologic overlap between subcutaneous panniculitis-like T-cell lymphoma with systemic features and small cell CD8+ ALK+ systemic anaplastic large cell lymphoma with cutaneous involvement

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.

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The Role of IgM Antibodies in T Cell Lymphoma Protection in a Novel Model Resembling Anaplastic Large Cell Lymphoma

The Journal of Immunology ◽

10.4049/jimmunol.2001279 ◽

2021 ◽

pp. ji2001279

Author(s):

Chuancang Jiang ◽

Ming-Lang Zhao ◽

Luis Ramos ◽

Katarzyna Dobaczewska ◽

Ronald Herbert ◽

...

Keyword(s):

T Cell ◽

Anaplastic Large Cell Lymphoma ◽

Cell Lymphoma ◽

Large Cell ◽

T Cell Lymphoma ◽

Igm Antibodies ◽

Large Cell Lymphoma ◽

Novel Model

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Primary cutaneous anaplastic large-cell lymphoma simulating an aggressive angiocentric T-cell lymphoma

Journal of the American Academy of Dermatology ◽

10.1016/j.jaad.2008.11.571 ◽

2009 ◽

Vol 60 (3) ◽

pp. AB130

Keyword(s):

T Cell ◽

Anaplastic Large Cell Lymphoma ◽

Cell Lymphoma ◽

Large Cell ◽

T Cell Lymphoma ◽

Large Cell Lymphoma

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ALK-negative anaplastic large-cell lymphoma demonstrates similar poor prognosis to peripheral T-cell lymphoma, unspecified

Histopathology ◽

10.1046/j.1365-2559.2003.01726.x ◽

2003 ◽

Vol 43 (5) ◽

pp. 462-469 ◽

Cited By ~ 66

Author(s):

R L ten Berge ◽

P C de Bruin ◽

J J Oudejans ◽

G J Ossenkoppele ◽

P van der Valk ◽

...

Keyword(s):

T Cell ◽

Anaplastic Large Cell Lymphoma ◽

Poor Prognosis ◽

Cell Lymphoma ◽

Large Cell ◽

T Cell Lymphoma ◽

Peripheral T Cell Lymphoma ◽

Large Cell Lymphoma

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FDG-PET Scans as a Staging Study for T-Cell Lymphomas: High Rates of Positivity Do Not Result in Frequent Changes in Stage.

Blood ◽

10.1182/blood.v108.11.2399.2399 ◽

2006 ◽

Vol 108 (11) ◽

pp. 2399-2399 ◽

Cited By ~ 1

Author(s):

Steven M. Horwitz ◽

Francine Foss ◽

Shari Goldfarb ◽

Ana Molina ◽

Paul A. Hamlin ◽

...

Keyword(s):

Lymph Node ◽

T Cell ◽

Anaplastic Large Cell Lymphoma ◽

Fdg Pet ◽

Cell Lymphoma ◽

Large Cell ◽

T Cell Lymphoma ◽

T Cell Lymphomas ◽

Large Cell Lymphoma ◽

Pet Scans

Abstract FDG-PET is emerging as a standard staging study for diffuse large B-cell lymphoma and Hodgkin’s disease. There is little experience with PET in staging T-cell lymphomas (TCL). TCL often involve extranodal sites not well imaged by standard CT. We hypothesized that PET would add to staging by detecting additional sites of disease. To address this question we reviewed our TCL database to identify patients (pt) who had PET as part of complete staging at initial diagnosis or at relapse. Each pt was included once. Staging included physical exam, CT scan of the chest/abdomen/pelvis alone or as part of a PET/CT combination, and bone marrow (BM) biopsy. Studies such as MRI of the sinus or ultrasound of the testes were done as clinically indicated. We reviewed 107 pt who met the above criteria. Histologies were as follows: peripheral T-cell lymphoma NOS (PTCL), angioimmunoblastic T-cell lymphoma (AILT), anaplastic large cell lymphoma, ALK-1-, (ALCL−), anaplastic large cell lymphoma, ALK-1+, (ALCL+), mycosis fungoides (MF), subcutaneous panniculitis-like T-cell lymphoma (SPTCL), adult T-cell lymphoma (ATL), NK lymphoma nasal type (NK-Nas), lymphoblastic lymphoma (LL), enteropathy associated T-cell lymphoma (EATCL), blastic NK lymphoma (BLNK), primary cutaneous ALCL (ALCL-Cut), and hepatosplenic T-cell lymphoma (HSPTCL). All pt with MF had suspicion of extracutaneous disease. Overall 95/107 (89%) had a PET interpreted as positive by visual review. Standard uptake values (SUV) varied from 1.1–20.5 g/ml. Of the 12 pt with negative PET scans, 7(58%) had no evidence of disease on CT including PTCL (n=3, stage I resected), HSPTCL (n=2, liver, spleen, BM disease only), ALCL-Cut (n=2, skin only). T-cell Lymphoma-PET Results Histology N PET + % positive SUV Range (g/ml) ALL PT 107 95 89% 1.1–20.5 PTCL 27 24 88% 2–20 AILT 19 16 84% 2–11.7 ALCL− 12 12 100% 3–19.6 ALCL+ 4 4 100% 4–12 MF 12 10 83% 1.8–17.6 SPTCL 8 8 100% 1.4–13.1 ATL 5 5 100% 2.9–19.7 NK-Nas 5 5 100% 3.4–13.1 LL 3 3 100% 5.5–20.5 EATCL 3 3 100% 3.5–9.9 ALCL-Cut 5 3 60% 1.1–1.4 BLNK 2 2 100% 1.929.5 HSPTCL 2 0 0 N/A PET detected additional sites of disease in 34/107 (32%). These sites detected by PET were skin/subcut (n=9), bone (n=7), lymph node (n=6), spleen (n=3), nasopharynx/sinus (n=2), liver (n=2), BM (n=1), bowel (n=1), muscle (n=1), kidney (n=1), tonsil (n=1), testes (n=1). Three new malignancies were incidentally detected by PET including lung cancer (n=1), metastatic renal cell to the parotid (n=1), and mantle cell lymphoma (n=1 in a pt with ALCL-cut). Despite these additional sites, stage was changed in only 10/107 (9%). We did not use negative PET to downstage. Sites resulting in higher stage included bone (n=3), lymph node (n=2), subcut/muscle (n=3), testes (n=1), liver (n=1). Skin lesions were noted on physical exam and therefore did not change stage. In conclusion, TCL are almost universally PET positive. PET often adds information by identifying extranodal disease. However, partly due to many pt being stage IV by other modalities, PET resulted in a change of stage in <10% of pt. PET may be particularly useful in assessing of skin sites. These data suggest that it may be beneficial to include PET in the response assessment of TCL, as has been proposed for DLBCL and HL in revised NHL response criteria.

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