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Extranodal natural killer/T-cell lymphoma involving the gastrointestinal tract: analysis of clinical features and outcomes from the Asia Lymphoma Study Group.

Kim SJ, Jung HA, Chuang SS, Hong H, Guo CC, Cao J, Hong XN, Suzuki R, Kang HJ, Won JH, Chng WJ, Kwong YL, Suh C, Song YQ, Zhu J, Tay K, Lim ST, Suzumiya J, Lin TY, Kim WS, Asia Lymphoma Study Gro - J Hematol Oncol (2013)

Bottom Line: The most common GI site was the small intestine, including the jejunum and ileum (n = 57, 70.3%).Thus, the median overall survival was 7.8 months (95% Confidence interval: 3.9 - 11.7 months).Thus, more effective treatment strategies are required for this disease entity.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Medicine, Division of Hematology and Oncology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. TongyuLim@hotmail.com.

ABSTRACT

Background: The gastrointestinal (GI) tract is one of the most common extranasal sites in extranodal NK/T-cell lymphoma (ENKTL). However, data regarding ENKTL involving the GI tract are relatively scarce. Thus, we performed a multicenter, multinational retrospective study to analyze clinical features and treatment outcomes of ENKTL involving the GI tract.

Patients and methods: Patients with ENKTL involving the GI tract diagnosed in twelve participating centers between 1991 and 2012 were retrospectively analyzed from five Asian countries.

Results: The analysis of 81 patients with ENKTL involving the GI tract revealed that more than 60% of patients presented as advanced disease with B symptoms. 55 patients (68%) had GI manifestations including abdominal pain (n = 26, 32%), GI tract bleeding (n = 17, 21%) and bowel perforation (n = 12, 15%). The most common GI site was the small intestine, including the jejunum and ileum (n = 57, 70.3%). There were 34 patients (42%) who received systemic chemotherapy while 33 patients (41%) underwent surgery plus chemotherapy. However, 35 patients (43%) died due to disease progression, and treatment-related mortality including sepsis occurred in 17 patients (21%). Thus, the median overall survival was 7.8 months (95% Confidence interval: 3.9 - 11.7 months). Patients who could undergo surgery plus chemotherapy showed a trend of better survival than those treated with chemotherapy alone.

Conclusion: Overall, the data indicated that ENKTL involving the GI tract has a dismal prognosis despite active treatment including chemotherapy and surgery. Thus, more effective treatment strategies are required for this disease entity.

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Summary of treatment approaches.
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Figure 2: Summary of treatment approaches.

Mentions: Out of the 55 patients presenting with GI manifestations, 37 patients first underwent resection of their primary mass lesion mainly for complications such as bleeding or perforation (Figure 2). All but one patients with bowel perforation underwent surgery. One patient presenting as abdominal pain and the other patient pretesting as perforation received only supportive care because they could not undergo surgery owing to their poor health status s (Figure 2). After surgery, 26 patients received systemic chemotherapy, whereas 11 patients did not. Sixteen patients who had widespread disease including GI tract and other sites received systemic chemotherapy (Figure 2). Among the 26 patients presenting without GI manifestations, 18 received systemic chemotherapy while 8 underwent surgery first. Therefore, the initial treatment for these patients included chemotherapy alone (n = 34, 42%), surgery plus chemotherapy (n = 33, 41%), and surgery alone (n = 12, 15%). CHOP (cyclophosphamide 750 mg/m2 on day1, doxorubicin 50 mg/m2 on day 1, vincristine 1.4 mg/m2 on day 1, and prednisone 100 mg on days 1–5) or CHOP-like chemotherapy regimens were used in 37 patients whereas non-anthracycline-based or intensified chemotherapy such as SMILE (Methotrexate 2 g/m2 on day 1, ifosfamide 1500 mg/m2 on days 2–4, etoposide 100 mg/m2 on days 2–4, dexamethasone 40 mg on days 2 – 4, and Escherichia coli L-asparaginase 6,000 U/m2 on days 8, 10, 12, 14, 16, 18, 20, n = 17), EPOCH (etoposide 50 mg/m2 on days 1–4, doxorubicin 10 mg/m2 on days 1–4, vincristine 0.4 mg/m2 on days 1–4, cyclophosphamide 750 mg/m2 on day 5, and prednisolone 60 mg/m2 on days 1–5, n = 5), VIPD (etoposide 100 mg/m2 on days 1–3, ifosfamide 1500 mg/m2 on days 1–3, cisplatin 33 mg/m2 on days 1–3, and dexamethasone 40 mg on days 1 – 4, n = 2), ESHAP (etoposide 40 mg/m2 on days 1–4, methylprednisolone 500 mg on days 1–4, cisplatin 25 mg/m2 on days 1–4, and cytarabine 2 g/m2 on day 5, n = 1), gemcitabine-containing chemotherapy (n = 2), and others (n = 3) were used in 30 patients. However, among the 34 patients treated with systemic chemotherapy, 9 patients underwent surgery due to GI complications during chemotherapy (Figure 2) including bowel perforation (n = 4), GI bleeding (n = 2), and GI obstruction (n = 3). Autologous (n = 3) or allogeneic hematopoietic stem cell transplantation (HSCT, n = 5) was done in eight patients as a consolidation treatment after chemotherapy.


Extranodal natural killer/T-cell lymphoma involving the gastrointestinal tract: analysis of clinical features and outcomes from the Asia Lymphoma Study Group.

Kim SJ, Jung HA, Chuang SS, Hong H, Guo CC, Cao J, Hong XN, Suzuki R, Kang HJ, Won JH, Chng WJ, Kwong YL, Suh C, Song YQ, Zhu J, Tay K, Lim ST, Suzumiya J, Lin TY, Kim WS, Asia Lymphoma Study Gro - J Hematol Oncol (2013)

Summary of treatment approaches.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4225665&req=5

Figure 2: Summary of treatment approaches.
Mentions: Out of the 55 patients presenting with GI manifestations, 37 patients first underwent resection of their primary mass lesion mainly for complications such as bleeding or perforation (Figure 2). All but one patients with bowel perforation underwent surgery. One patient presenting as abdominal pain and the other patient pretesting as perforation received only supportive care because they could not undergo surgery owing to their poor health status s (Figure 2). After surgery, 26 patients received systemic chemotherapy, whereas 11 patients did not. Sixteen patients who had widespread disease including GI tract and other sites received systemic chemotherapy (Figure 2). Among the 26 patients presenting without GI manifestations, 18 received systemic chemotherapy while 8 underwent surgery first. Therefore, the initial treatment for these patients included chemotherapy alone (n = 34, 42%), surgery plus chemotherapy (n = 33, 41%), and surgery alone (n = 12, 15%). CHOP (cyclophosphamide 750 mg/m2 on day1, doxorubicin 50 mg/m2 on day 1, vincristine 1.4 mg/m2 on day 1, and prednisone 100 mg on days 1–5) or CHOP-like chemotherapy regimens were used in 37 patients whereas non-anthracycline-based or intensified chemotherapy such as SMILE (Methotrexate 2 g/m2 on day 1, ifosfamide 1500 mg/m2 on days 2–4, etoposide 100 mg/m2 on days 2–4, dexamethasone 40 mg on days 2 – 4, and Escherichia coli L-asparaginase 6,000 U/m2 on days 8, 10, 12, 14, 16, 18, 20, n = 17), EPOCH (etoposide 50 mg/m2 on days 1–4, doxorubicin 10 mg/m2 on days 1–4, vincristine 0.4 mg/m2 on days 1–4, cyclophosphamide 750 mg/m2 on day 5, and prednisolone 60 mg/m2 on days 1–5, n = 5), VIPD (etoposide 100 mg/m2 on days 1–3, ifosfamide 1500 mg/m2 on days 1–3, cisplatin 33 mg/m2 on days 1–3, and dexamethasone 40 mg on days 1 – 4, n = 2), ESHAP (etoposide 40 mg/m2 on days 1–4, methylprednisolone 500 mg on days 1–4, cisplatin 25 mg/m2 on days 1–4, and cytarabine 2 g/m2 on day 5, n = 1), gemcitabine-containing chemotherapy (n = 2), and others (n = 3) were used in 30 patients. However, among the 34 patients treated with systemic chemotherapy, 9 patients underwent surgery due to GI complications during chemotherapy (Figure 2) including bowel perforation (n = 4), GI bleeding (n = 2), and GI obstruction (n = 3). Autologous (n = 3) or allogeneic hematopoietic stem cell transplantation (HSCT, n = 5) was done in eight patients as a consolidation treatment after chemotherapy.

Bottom Line: The most common GI site was the small intestine, including the jejunum and ileum (n = 57, 70.3%).Thus, the median overall survival was 7.8 months (95% Confidence interval: 3.9 - 11.7 months).Thus, more effective treatment strategies are required for this disease entity.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Medicine, Division of Hematology and Oncology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. TongyuLim@hotmail.com.

ABSTRACT

Background: The gastrointestinal (GI) tract is one of the most common extranasal sites in extranodal NK/T-cell lymphoma (ENKTL). However, data regarding ENKTL involving the GI tract are relatively scarce. Thus, we performed a multicenter, multinational retrospective study to analyze clinical features and treatment outcomes of ENKTL involving the GI tract.

Patients and methods: Patients with ENKTL involving the GI tract diagnosed in twelve participating centers between 1991 and 2012 were retrospectively analyzed from five Asian countries.

Results: The analysis of 81 patients with ENKTL involving the GI tract revealed that more than 60% of patients presented as advanced disease with B symptoms. 55 patients (68%) had GI manifestations including abdominal pain (n = 26, 32%), GI tract bleeding (n = 17, 21%) and bowel perforation (n = 12, 15%). The most common GI site was the small intestine, including the jejunum and ileum (n = 57, 70.3%). There were 34 patients (42%) who received systemic chemotherapy while 33 patients (41%) underwent surgery plus chemotherapy. However, 35 patients (43%) died due to disease progression, and treatment-related mortality including sepsis occurred in 17 patients (21%). Thus, the median overall survival was 7.8 months (95% Confidence interval: 3.9 - 11.7 months). Patients who could undergo surgery plus chemotherapy showed a trend of better survival than those treated with chemotherapy alone.

Conclusion: Overall, the data indicated that ENKTL involving the GI tract has a dismal prognosis despite active treatment including chemotherapy and surgery. Thus, more effective treatment strategies are required for this disease entity.

Show MeSH
Related in: MedlinePlus