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Multicenter Study
. 2024 Dec 2;41(4):225-235.
doi: 10.4274/tjh.galenos.2024.2023.0450. Epub 2024 Nov 6.

Survival Outcomes of Patients with Primary Plasma Cell Leukemia in the Era of Proteasome Inhibitors and Immunomodulatory Agents: A Real-Life Multicenter Analysis

Ünal Ataş  1   2 , Ozan Salim  1 , Utku Iltar  1 , Orhan Kemal Yücel  1 , Ayşe Hilal Küçükdiler Eroğlu  3 , Vedat Aslan  2 , Murat Yıldırım  4 , Özen Dedeoğlu  5 , Sema Seçilmiş  6 , Turgay Ulaş  6 , Burak Deveci  7 , Sedanur Karaman Gulsaran  8 , Ayfer Gedük  9 , Fatih Yaman  10 , İbrahim Ethem Pınar  11 , Senem Maral  12 , Ahmet Sarıcı  13 , Serhat Çelik  14 , Hande Oğul  15 , Sıdıka Gülkan Özkan  16 , Aliihsan Gemici  17 , Ahmet Kurşat Güneş  18 , Anil Tombak  19 , İrfan Yavaşoğlu  3 , Volkan Karakuş  2 , Melda Cömert  4 , Tayfur Toptaş  5 , Mehmet Sinan Dal  6 , Rabin Saba  7 , Hakkı Onur Kırkızlar  8 , Özgür Mehtap  9 , Eren Gündüz  10 , Fahir Özkalemkaş  11 , Murat Albayrak  12 , İlhami Berber  13 , Muzaffer Keklik  14 , Nil Güler  15 , Hasan Atilla Özkan  16 , Ömür Gökmen Sevindik  17 , Zahit Bolaman  3 , Erdal Kurtoğlu  2 , Meltem Aylı  4 , Tülin Fıratlı Tuğlular  5 , Fevzi Altuntaş  6 , Levent Ündar  1
Affiliations
Multicenter Study

Survival Outcomes of Patients with Primary Plasma Cell Leukemia in the Era of Proteasome Inhibitors and Immunomodulatory Agents: A Real-Life Multicenter Analysis

Ünal Ataş et al. Turk J Haematol. .

Abstract
in English, Turkish

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.

Amaç: Bu çalışma ile multipl miyelom (MM) hastalarında genel sağkalımda (OS) anlamlı bir artış sağlayan antimiyeloma ajanlarının, prognozu daha kötü olan primer plazma hücreli lösemi (pPHL) hastalarında kullanımına ilişkin gerçek hayat verilerini ortaya koymak istedik.

Gereç ve yöntemler: 2011-2020 yılları arasında pPHL tanısı alan ve en az bir proteazom inhibitörü (PI) ve/veya immünomodülatör (IMID) ajan kullanan 53 hastanın verileri retrospektif olarak analiz edildi. Hastaların tanı yıllarından kaynaklı olarak, periferik kanda plazma hücresinin lökositlerin %20’sinden fazla veya ≥2x109/L olması pPHL tanı kriteri kabul edildi.

Bulgular: Hastaların ortanca yaşı 58 olup, 23 (%43,4) hasta 65 yaş üzerindeydi. İlk sıra tedavide 31 (%58,5) hastada PI veya IMID tek başına kullanılırken, 15 (%28,3) hastada PI ve IMID eş zamanlı kullanıldı. Ayrıca 21 (%39,6) hastaya nakil, 13 (%24,5) hastaya ise idame tedavisi uygulandı. Hastaların ortanca progresyonsuz sağkalım süresi 4 (1-42) aydı. İlk sıra tedaviye primer refrakter hastalar dışlandığında ise 6,5 aydı. Ortanca takip süresi 15 ay olan hastaların, ortanca OS süresi de 15 aydı. Son kontrolde hastaların sadece 7’si (%13,2) hayattaydı. β2 mikroglobulin düzeyi yüksek, Uluslararası Evreleme Sistemi skoru 3 olan ve birinci basamak tedavide nakil yapılmayan hastalarda OS daha kısaydı (sırasıyla, p=0,005, p=0,02 ve p=0,008). Öte yandan indüksiyon tedavisinde PI ve IMID ajanlarının birlikte kullanılmasının, kemoterapi eklenmesinin, indüksiyon tedavisine yanıtın ve idame tedavisinin OS üzerine etkisi olmadığı görüldü.

Sonuç: Önceki benzer çalışmalarda olduğu gibi, çalışmamızda pPHL’de MM’de gözlenen artan sağkalım eğilimini göremedik. Yeni tanı kriteri ile birlikte artması olası pPHL hastaları için, MM’daki güncel ajanlar ve bilgiler dahilinde, yapılacak yeni çalışmalara ihtiyaç vardır.

Keywords: Antimyeloma agents; Hematopoietic stem cell transplantation; Immunomodulatory agents; Primary plasma cell leukemia; Proteasome inhibitors.

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Conflict of interest statement

Conflict of Interest: No conflict of interest was declared by the authors.

Figures

Figure 1
Figure 1
Progression-free survival in patients with and without bone marrow transplantation in first-line therapy. n: Number of patients.
Figure 2
Figure 2
Comparison of overall survival according to International Staging System (ISS) stages 1-2 and 3. n: Number of patients.
Figure 3
Figure 3
(A) Proteasome inhibitor (PI) and/or immunomodulatory (IMID) agents used together and not used in induction therapy; (B) in induction treatment, with or without intensive chemotherapy with an antimyeloma agent; (C) after induction therapy, patients with or without at least partial response; (D) patients with or without consolidative bone marrow transplantation in first-line therapy. n: Number of patients.
See this image and copyright information in PMC

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