Ünal Ataş, Ozan Salim, Utku Iltar, Orhan Kemal Yücel, Ayşe Hilal Küçükdiler Eroğlu, Vedat Aslan, Murat Yıldırım, Özen Dedeoğlu, Sema Seçilmiş, Turgay Ulaş, Burak Deveci, Sedanur Karaman Gulsaran, Ayfer Gedük, Fatih Yaman, İbrahim Ethem Pınar, Senem Maral, Ahmet Sarıcı, Serhat Çelik, Hande Oğul, Sıdıka Gülkan Özkan, Aliihsan Gemici, Ahmet Kurşat Güneş, Anil Tombak, İrfan Yavaşoğlu, Volkan Karakuş, Melda Cömert, Tayfur Toptaş, Mehmet Sinan Dal, Rabin Saba, Hakkı Onur Kırkızlar, Özgür Mehtap, Eren Gündüz, Fahir Özkalemkaş, Murat Albayrak, İlhami Berber, Muzaffer Keklik, Nil Güler, Hasan Atilla Özkan, Ömür Gökmen Sevindik, Zahit Bolaman, Erdal Kurtoğlu, Meltem Aylı, Tülin Fıratlı Tuğlular, Fevzi Altuntaş, Levent Ündar
{"title":"蛋白酶体抑制剂和免疫调节药物时代原发性浆细胞白血病患者的生存结果：多中心实际分析","authors":"Ünal Ataş, Ozan Salim, Utku Iltar, Orhan Kemal Yücel, Ayşe Hilal Küçükdiler Eroğlu, Vedat Aslan, Murat Yıldırım, Özen Dedeoğlu, Sema Seçilmiş, Turgay Ulaş, Burak Deveci, Sedanur Karaman Gulsaran, Ayfer Gedük, Fatih Yaman, İbrahim Ethem Pınar, Senem Maral, Ahmet Sarıcı, Serhat Çelik, Hande Oğul, Sıdıka Gülkan Özkan, Aliihsan Gemici, Ahmet Kurşat Güneş, Anil Tombak, İrfan Yavaşoğlu, Volkan Karakuş, Melda Cömert, Tayfur Toptaş, Mehmet Sinan Dal, Rabin Saba, Hakkı Onur Kırkızlar, Özgür Mehtap, Eren Gündüz, Fahir Özkalemkaş, Murat Albayrak, İlhami Berber, Muzaffer Keklik, Nil Güler, Hasan Atilla Özkan, Ömür Gökmen Sevindik, Zahit Bolaman, Erdal Kurtoğlu, Meltem Aylı, Tülin Fıratlı Tuğlular, Fevzi Altuntaş, Levent Ündar","doi":"10.4274/tjh.galenos.2024.2023.0450","DOIUrl":null,"url":null,"abstract":"<p><strong>Objective: </strong>In this study, we aimed to obtain real-life data on the use of antimyeloma agents, which significantly increase overall survival (OS) in multiple myeloma (MM) patients, in primary plasma cell leukemia (pPCL) patients with poor prognosis.</p><p><strong>Materials and methods: </strong>Data from 53 patients who were diagnosed with pPCL between 2011 and 2020 and who used at least one proteasome inhibitor (PI) and/or immunomodulatory (IMID) agent were analyzed retrospectively. Depending on the year of the pPCL diagnosis, 20% leukocytes or ≥2x10<sup>9</sup>/L plasma cells in the peripheral blood was used as a diagnostic criterion.</p><p><strong>Results: </strong>The median age of the patients was 58 years and 23 (43.4%) patients were over 65 years of age. For first-line treatment, PI or IMID alone was used by 31 (58.5%) patients, and PI and IMID were used simultaneously by 15 (28.3%) patients. Additionally, 21 (39.6%) patients received transplantation and 13 (24.5%) patients received maintenance treatment. The median progression-free survival was 4 (range: 1-42) months. When patients whose primary disease was refractory to first-line therapy were excluded, the duration of treatment was 6.5 months. The median OS was 15 months with a median follow-up duration of 15 months. Only 7 (13.2%) of the patients were alive at the last follow-up visit. Those with higher β2-microglobulin levels and International Staging System stage 3 and non-transplant patients receiving first-line treatment had shorter OS (p=0.005, p=0.02, and p=0.008, respectively). The concomitant use of PIs and IMIDs, the addition of chemotherapy to induction therapy, and the response to induction therapy or maintenance therapy did not affect OS.</p><p><strong>Conclusion: </strong>In this study, as in previous similar studies, we could not see an increased survival trend in pPCL, which is observed in MM. New studies are needed for pPCL, which is likely to increase with new diagnostic criteria, based on current agents and information from MM.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"225-235"},"PeriodicalIF":1.5000,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11628765/pdf/","citationCount":"0","resultStr":"{\"title\":\"Survival Outcomes of Patients with Primary Plasma Cell Leukemia in the Era of Proteasome Inhibitors and Immunomodulatory Agents: A Real-Life Multicenter Analysis\",\"authors\":\"Ünal Ataş, Ozan Salim, Utku Iltar, Orhan Kemal Yücel, Ayşe Hilal Küçükdiler Eroğlu, Vedat Aslan, Murat Yıldırım, Özen Dedeoğlu, Sema Seçilmiş, Turgay Ulaş, Burak Deveci, Sedanur Karaman Gulsaran, Ayfer Gedük, Fatih Yaman, İbrahim Ethem Pınar, Senem Maral, Ahmet Sarıcı, Serhat Çelik, Hande Oğul, Sıdıka Gülkan Özkan, Aliihsan Gemici, Ahmet Kurşat Güneş, Anil Tombak, İrfan Yavaşoğlu, Volkan Karakuş, Melda Cömert, Tayfur Toptaş, Mehmet Sinan Dal, Rabin Saba, Hakkı Onur Kırkızlar, Özgür Mehtap, Eren Gündüz, Fahir Özkalemkaş, Murat Albayrak, İlhami Berber, Muzaffer Keklik, Nil Güler, Hasan Atilla Özkan, Ömür Gökmen Sevindik, Zahit Bolaman, Erdal Kurtoğlu, Meltem Aylı, Tülin Fıratlı Tuğlular, Fevzi Altuntaş, Levent Ündar\",\"doi\":\"10.4274/tjh.galenos.2024.2023.0450\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Objective: </strong>In this study, we aimed to obtain real-life data on the use of antimyeloma agents, which significantly increase overall survival (OS) in multiple myeloma (MM) patients, in primary plasma cell leukemia (pPCL) patients with poor prognosis.</p><p><strong>Materials and methods: </strong>Data from 53 patients who were diagnosed with pPCL between 2011 and 2020 and who used at least one proteasome inhibitor (PI) and/or immunomodulatory (IMID) agent were analyzed retrospectively. Depending on the year of the pPCL diagnosis, 20% leukocytes or ≥2x10<sup>9</sup>/L plasma cells in the peripheral blood was used as a diagnostic criterion.</p><p><strong>Results: </strong>The median age of the patients was 58 years and 23 (43.4%) patients were over 65 years of age. For first-line treatment, PI or IMID alone was used by 31 (58.5%) patients, and PI and IMID were used simultaneously by 15 (28.3%) patients. Additionally, 21 (39.6%) patients received transplantation and 13 (24.5%) patients received maintenance treatment. The median progression-free survival was 4 (range: 1-42) months. When patients whose primary disease was refractory to first-line therapy were excluded, the duration of treatment was 6.5 months. The median OS was 15 months with a median follow-up duration of 15 months. Only 7 (13.2%) of the patients were alive at the last follow-up visit. Those with higher β2-microglobulin levels and International Staging System stage 3 and non-transplant patients receiving first-line treatment had shorter OS (p=0.005, p=0.02, and p=0.008, respectively). The concomitant use of PIs and IMIDs, the addition of chemotherapy to induction therapy, and the response to induction therapy or maintenance therapy did not affect OS.</p><p><strong>Conclusion: </strong>In this study, as in previous similar studies, we could not see an increased survival trend in pPCL, which is observed in MM. 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引用次数: 0
摘要
研究目的在这项研究中，我们旨在获得有关预后不良的原发性浆细胞白血病（pPCL）患者使用抗骨髓瘤药物的真实数据，这些药物可显著提高多发性骨髓瘤（MM）患者的总生存期（OS）：回顾性分析了 2011-2020 年间确诊为 pPCL 的 53 例患者的数据，这些患者至少使用过一种蛋白酶体抑制剂（PI）和/或免疫调节剂（IMID）。根据确诊 pPCL 的年份，采用外周血中 20% 的白细胞或≥2×109/L 的浆细胞：患者的中位年龄为 58 岁，23 名（43.4%）患者年龄超过 65 岁。在一线治疗中，31 名患者（58.5%）单独使用了 PI 或 IMID，15 名患者（28.3%）同时使用了 PI 和 IMID。此外，21 例（39.6%）患者接受了移植，13 例（24.5%）患者接受了维持治疗。无进展生存期的中位数为 4（1-42）个月。如果排除原发疾病对一线治疗难治的患者，治疗时间为6.5个月。中位生存期为15个月，中位随访时间为15个月。最后一次随访时，只有 7 名（13.2%）患者存活。β-2微球蛋白水平较高、ISS3期和接受一线治疗的非移植患者的OS较短（分别为p=0.005、p=0.02和p=0.008）。除此之外，同时使用PIs和IMIDs、在诱导治疗中增加化疗、对诱导治疗或维持治疗的反应均不影响OS：结论：在我们的研究中，与之前的类似研究一样，我们没有看到在 MM 中观察到的 pPCL 存活率增加的趋势。需要对 pPCL 患者进行新的研究，根据 MM 的现有药物和信息，随着新诊断标准的出台，pPCL 患者可能会增加。
Survival Outcomes of Patients with Primary Plasma Cell Leukemia in the Era of Proteasome Inhibitors and Immunomodulatory Agents: A Real-Life Multicenter Analysis
Objective: In this study, we aimed to obtain real-life data on the use of antimyeloma agents, which significantly increase overall survival (OS) in multiple myeloma (MM) patients, in primary plasma cell leukemia (pPCL) patients with poor prognosis.
Materials and methods: Data from 53 patients who were diagnosed with pPCL between 2011 and 2020 and who used at least one proteasome inhibitor (PI) and/or immunomodulatory (IMID) agent were analyzed retrospectively. Depending on the year of the pPCL diagnosis, 20% leukocytes or ≥2x109/L plasma cells in the peripheral blood was used as a diagnostic criterion.
Results: The median age of the patients was 58 years and 23 (43.4%) patients were over 65 years of age. For first-line treatment, PI or IMID alone was used by 31 (58.5%) patients, and PI and IMID were used simultaneously by 15 (28.3%) patients. Additionally, 21 (39.6%) patients received transplantation and 13 (24.5%) patients received maintenance treatment. The median progression-free survival was 4 (range: 1-42) months. When patients whose primary disease was refractory to first-line therapy were excluded, the duration of treatment was 6.5 months. The median OS was 15 months with a median follow-up duration of 15 months. Only 7 (13.2%) of the patients were alive at the last follow-up visit. Those with higher β2-microglobulin levels and International Staging System stage 3 and non-transplant patients receiving first-line treatment had shorter OS (p=0.005, p=0.02, and p=0.008, respectively). The concomitant use of PIs and IMIDs, the addition of chemotherapy to induction therapy, and the response to induction therapy or maintenance therapy did not affect OS.
Conclusion: In this study, as in previous similar studies, we could not see an increased survival trend in pPCL, which is observed in MM. New studies are needed for pPCL, which is likely to increase with new diagnostic criteria, based on current agents and information from MM.
期刊介绍：
The Turkish Journal of Hematology is published quarterly (March, June, September, and December) by the Turkish Society of Hematology. It is an independent, non-profit peer-reviewed international English-language periodical encompassing subjects relevant to hematology.
The Editorial Board of The Turkish Journal of Hematology adheres to the principles of the World Association of Medical Editors (WAME), International Council of Medical Journal Editors (ICMJE), Committee on Publication Ethics (COPE), Consolidated Standards of Reporting Trials (CONSORT) and Strengthening the Reporting of Observational Studies in Epidemiology (STROBE).
The aim of The Turkish Journal of Hematology is to publish original hematological research of the highest scientific quality and clinical relevance. Additionally, educational material, reviews on basic developments, editorial short notes, images in hematology, and letters from hematology specialists and clinicians covering their experience and comments on hematology and related medical fields as well as social subjects are published. As of December 2015, The Turkish Journal of Hematology does not accept case reports. Important new findings or data about interesting hematological cases may be submitted as a brief report.
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