scholarly journals Novel Mutations in a Lethal Case of Lymphomatous Adult T Cell Lymphoma with Cryptic Myocardial Involvement

2021 ◽  
Vol 28 (1) ◽  
pp. 818-824 ◽  
Author(s):  
Taraneh Hashemi Zonouz ◽  
Rami Abdulbaki ◽  
Bidhan C. Bandyopadhyay ◽  
Victor E. Nava
Keyword(s):  
T Cell ◽  
Novel Mutations ◽  
Reduced Ejection Fraction ◽  
Adult T Cell Leukemia ◽  
Diagnostic Pitfall ◽  
Positron Emission ◽  
Pet Ct ◽  
Lymphomatous Involvement ◽  
Myocardial Involvement ◽  
Generation Sequencing

The autopsy of a 65-year-old diabetic African American male revealed significant left myocardial involvement by adult T-cell leukemia/lymphoma (ATLL) despite normal pre-mortem fluorodeoxyglucose (FDG) uptake by positron emission tomography/computed tomography (PET/CT). Due to pre-existing diabetic cardiomyopathy with reduced ejection fraction (EF) and compatible imaging studies, cardiac lymphomatous involvement was not suspected. While peripheral blood was negative for leukemia, next-generation sequencing of a lymph node revealed at least eight novel mutations (AXIN1, R712Q, BARD1 R749K, CTNNB1 I315V, CUX1 P102T, DNMT3A S199R, FGFR2 S431L, LRP1B Y2560C and STAG2 I771M). These findings underscore a diagnostic pitfall in a rare lymphomatous variant of ATLL infiltrating myocardium and contribute to its molecular characterization.

scholarly journals The value of complete remission according to positron emission tomography prior to autologous stem cell transplantation in lymphoma: a population-based study showing improved outcome

2020 ◽  
Author(s):  
Kristina Noring ◽  
Mattias Carlsten ◽  
Kristina Sonnevi ◽  
Björn Wahlin
Keyword(s):  
Positron Emission Tomography ◽  
Stem Cell ◽  
T Cell ◽  
Stem Cell Transplantation ◽  
B Cell ◽  
Cell Lymphoma ◽  
Emission Tomography ◽  
Positron Emission ◽  
Pet Ct ◽  
Improved Outcome

Abstract Background Chimeric antigen-receptor T-cell and bispecific antibody therapies will likely necessitate a reconsideration of the role of autologous stem-cell transplantation (ASCT) in lymphoma. Patients who are likely to profit from ASCT need to be better identified. Methods Here, we investigated the value of positron emission tomography/computerized tomography (PET/CT) before ASCT. All 521 patients transplanted for lymphoma 1994–2019 at Karolinska (497 conditioned with BEAM) were included. Results Outcome improved over three calendar periods 1994–2004, 2005–2014, 2015–2019 (2-year overall survival [OS]: 66%, 73%, 83%; P = 0.018). Non-relapse mortality (NRM) at 100 days over the three periods were 9.8%, 3.9%, 2.9%, respectively. The OS improvement between 1994–2004 and 2005–2014 was due to lower NRM (P = 0.027), but the large OS advance from 2015 was not accompanied by a significant reduction in NRM (P = 0.6). The fraction of PET/CT as pre-ASCT assessment also increased over time: 1994–2004, 2%; 2005–2014, 24%; 2015–2019, 60% (P < 0.00005). Complete responses (PET/CT-CR) were observed in 77% and metabolically active partial responses (PET/CT-PR) in 23%. PET/CT-CR was a predictor for survival in the entire population (P = 0.0003), also in the subpopulations of aggressive B-cell (P = 0.004) and peripheral T-cell (P = 0.024) lymphomas. Two-year OS and progression-free survival (OS/PFS) for patients in PET/CT-CR were in relapsed/refractory aggressive B-cell lymphoma 87%/75% and peripheral T-cell lymphoma 91%/78%. The corresponding figures in PET/CT-PR were 43%/44% and 33%/33%. Patients with solitary PET/CT-positive lesions showed acceptable outcome with ASCT followed by local irradiation (2-year OS/PFS 80%/60%). CT was less discriminative: 2-year OS/PFS: CT-CR, 76%/66%; CT-PR, 62%/51%. Outcome was inferior after BEAC compared with BEAM conditioning. Conclusions We conclude that the improved outcome reflects better, PET/CT-informed, identification of patients who should proceed to ASCT. The excellent survival of patients in PET/CT-CR indicates that ASCT should remain part of standard therapy for lymphoma.

Aberrant TTF-1 Expression in Peripheral T-Cell Lymphomas: A Diagnostic Pitfall

2020 ◽  
pp. 106689692094683
Author(s):  
Sheng-Tsung Chang ◽  
Shang-Wen Chen ◽  
Bo-Jung Chen ◽  
Shien-Tung Pan ◽  
Kennosuke Karube ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Unknown Origin ◽  
B Cell Lymphoma ◽  
Unknown Primary ◽  
Specific Marker ◽  
Lung Cancers ◽  
Adult T Cell Leukemia ◽  
Diagnostic Pitfall ◽  
T Cell Lymphomas

Background Thyroid transcription factor-1 (TTF-1) is a useful marker for identifying thyroid and lung cancers in diagnostic pathology, particularly for the investigation of unknown primary cancers. However, some other tumors such as colorectal cancer might aberrantly express TTF-1, particularly with the less specific clone SPT24. Occasional diffuse large B-cell lymphoma (DLBCL) cases have been reported to be TTF-1-positive, yet there is no information on TTF-1 expression in peripheral T-cell lymphoma (PTCL). Methods We investigated a series of PTCL and DLBCL by immunohistochemistry for TTF-1 expression using 2 commercially available clones. Results We found that 33% (5/15) adult T-cell leukemia/lymphomas (ATLLs) and 25% (2/8) angioimmunoblastic T-cell lymphomas (AITLs) were positive by clone SPT24 and only 2ATLL cases were positive by clone 8G7G3/1. Overall TTF-1 expression rates of PTCL by SPT24 and 8G7G3/1 were 16% (7/43) and 5% (2/43), respectively. All DLBCLs were negative. Conclusion Although TTF-1 is a relatively specific marker for thyroid and lung cancers, it might be expressed in some lymphomas, particularly PTCL when using clone SPT24. Pathologist should be aware of this possible diagnostic pitfall when using TTF-1 in investigating tumors of unknown origin.

scholarly journals Clinical Limitations of Surveillance Fluorine 18 Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography According to Histologic Subtypes in Patients with Malignant Lymphoma Who Achieved Complete Remission

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 1736-1736
Author(s):  
Yu Ri Kim ◽  
Soo-Jeong Kim ◽  
June-Won Cheong ◽  
Yundeok Kim ◽  
Ji Eun Jang ◽  
...  
Keyword(s):  
T Cell ◽  
B Cell ◽  
Predictive Value ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Indolent Lymphoma ◽  
Histologic Subtype ◽  
Positron Emission ◽  
Pet Ct ◽  
Mantle Cell

Abstract Introduction Fluorine 18 Fluorodeoxyglucose (FDG) Positron emission tomography-computed tomography (PET-CT) scan was highly sensitive modality for evaluating the staging or response of malignant lymphoma. The role of PET to detect early relapse in complete remission was still uncertain. We evaluated the clinical implication of surveillance PET-CT scan in lymphoma patients according to histologic subtype of lymphoma. Patients and methods A total 1009 patients of 1553 treated lymphoma patients, who achieved complete response following primary treatment, were retrospectively reviewed. Among these patients, we analyzed 202 patients who showed positive results of PET-CT and performed tissue biopsy of FDG uptake sites. Results Total 202 surveillance PET-CT positive patients, 120 (59.4%) patients were confirmed lymphoma relapse, 82 (40.6%) patients were histologically negative. Histologically negative results of 82 patients as follows: most (59.7%) patients were inflammation, 10 (2.1%) patients were solid tumor and 3 patients were tuberculosis. According to histologic subtype, PPV of Hodgkin's lymphoma was 25.0% (4/16), diffuse large B-cell lymphoma patients was 51.0% (47/92), mantle cell lymphoma (MCL) was 86.6% (13/15) and extranodal NK/T cell lymphoma (ENKL) was 85.7% (12/14) (Table 1). There was significant difference between PPV of aggressive B-cell non-Hodgkin lymphoma (NHL) except MCL and aggressive T-cell NHL (51.0% vs 78.5%, P=0.040). Indolent NHL was related with high PPV compared to aggressive B-cell NHL (70.2% vs 51.0%, P=0.032). Six (12.7%) patients of 47 indolent lymphoma were transformed to DLBCL at relapse. Four (8.5%) patients of 47 DLBCL patients showed histologic change to indolent lymphoma. There was no difference of predictive value according to stage, the number of extranodal involvement, lactic dehydrogenase and international prognostic index. PPV of FDG uptake in lymph node was 58.3% (63/108) and other sites except lymph node were 60.6% (57/94). Biopsy of stomach was 53.3% (8/15), small or large bowel was 55.5% (7/12). True positive patients of core biopsy was 61 (65.5%) of 93 patients, operation or excisional biopsy was 59 (67.0%) of 88 patients and aspiration cytology was 4 (19.0%) of 21 patients. Conclusion PPV of surveillance PET-CT in aggressive B-cell lymphoma was lower than indolent lymphoma or aggressive T-cell lymphoma. Surveillance PET-CT had clinical limitations according to histologic subtype of lymphoma. Histologic confirm by re-biospy should be recommended to exclude the false positive in aggressive B-cell lymphoma and distinguish the histologic transformation in indolent lymphoma. Table 1. Positive predictive value according to lymphoma subtype Pathology Lymphoma subtype positive negative Positive predictive value DLBCL (n=92) 47 45 51.0 MZBCL (n=26) 17 9 65.3 Follicular lymphoma (n=20) 16 4 80.0 Mantle cell lymphoma (n=15) 13 2 86.6 Burkitt lymphoma (n=3) 1 2 33.3 Plasmablastic lymphoma (n=1) 1 0 100 Lymphoplasmacytic lymphoma (n=1) 0 1 0 ENKL (n=14) 12 2 85.7 PTCL (n=11) 7 4 63.6 Mycosis fungoides (n=3) 2 1 66.6 Hodgkin's lymphoma (n=16) 4 12 25.0 Total (n=202) 120 82 59.4 Disclosures No relevant conflicts of interest to declare.

scholarly journals Clinical usefulness of FDG–PET/CT for the evaluation of various types of adult T-cell leukemia

Hematology ◽  
2017 ◽  
Vol 22 (9) ◽  
pp. 536-543 ◽  
Author(s):  
Sawako Nakachi ◽  
Masahiro Okada ◽  
Satoko Morishima ◽  
Yurika Agarie ◽  
Sakiko Kitamura ◽  
...  
Keyword(s):  
T Cell ◽  
Fdg Pet ◽  
Clinical Usefulness ◽  
T Cell Leukemia ◽  
Cell Leukemia ◽  
Adult T Cell Leukemia ◽  
Pet Ct ◽  
Fdg Pet Ct
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