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

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

Turk J Hematol. Ahead of Print: TJH-27448 | DOI: 10.4274/tjh.galenos.2024.2024.0450

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

Unal Atas¹, Ozan Salim², Utku Iltar², Orhan Kemal Yucel², Ayse Hilal Kucukdiler Eroglu³, Vedat Aslan⁴, Murat Yildirim⁵, Ozen Dedeoglu⁶, Sema Secilmis⁷, Turgay Ulas⁷, Burak Deveci⁸, Sedanur Karaman Gulsaran⁹, Ayfer Geduk¹⁰, Fatih Yaman¹¹, Ibrahim Ethem Pinar¹², Senem Maral¹³, Ahmet Sarici¹⁴, Serhat Celik¹⁵, Hande Ogul¹⁶, Sidika Gulkan Ozkan¹⁷, Aliihsan Gemici¹⁸, Ahmet Kursat Gunes¹⁹, Anil Tombak²⁰, Irfan Yavasoglu³, Volkan Karakus Et Al.⁴
¹Akdeniz University, Faculty of Medicine, Division of Hematology, Antalya, T�rkiye; Health Sciences University, Antalya Training and Research Hospital, Division of Hematology, Antalya, T�rkiye
²Akdeniz University, Faculty of Medicine, Division of Hematology, Antalya, T�rkiye
³Aydin Adnan Menderes University, Faculty of Medicine, Division of Hematology, Ayd�n, T�rkiye
⁴Health Sciences University, Antalya Training and Research Hospital, Division of Hematology, Antalya, T�rkiye
⁵Health Sciences University, Ankara Gulhane Training and Research Hospital, Division of Hematology, Ankara, T�rkiye
⁶Marmara University, Faculty of Medicine, Division of Hematology, �stanbul, T�rkiye
⁷Health Sciences University, Dr Abdurrahman Yurtaslan Ankara Oncology Training and Research Hospital, Division of Hematology, Ankara, T�rkiye
⁸Antalya Bilim University, Antalya, T�rkiye
⁹Trakya University, Faculty of Medicine, Division of Hematology, Edirne, T�rkiye
¹⁰Kocaeli University, Faculty of Medicine, Division of Hematology, Kocaeli, T�rkiye
¹¹Eskisehir Osmangazi University, Faculty of Medicine, Division of Hematology, Eski�ehir, T�rkiye
¹²Bursa Uludag University, Faculty of Medicine, Division of Hematology, Bursa, T�rkiye
¹³Health Sciences University, Diskapi Yildirim Beyazit Training and Research Hospital, Division of Hematology, Ankara, T�rkiye
¹⁴Inonu University, Faculty of Medicine, Division of Hematology, Malatya, T�rkiye
¹⁵Erciyes University, Faculty of Medicine, Division of Hematology, Kayseri, T�rkiye
¹⁶Pamukkale University, Faculty of Medicine, Division of Hematology, Denizli, T�rkiye
¹⁷Yeditepe University, Faculty of Medicine, Division of Hematology, �stanbul, T�rkiye
¹⁸Istanbul Medipol University, Medipol Mega Hospital, Division of Hematology, �stanbul, T�rkiye
¹⁹Ankara City Hospital, Division of Hematology, Ankara, T�rkiye
²⁰Mersin University, Faculty of Medicine, Division of Hematology, Mersin, T�rkiye

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 a poor prognosis.
Materials and Methods: Data from 53 patients who were diagnosed with pPCL between 2011�2020 and who used at least one proteasome inhibitor (PI) and/or immunomodulatory (IMID) agent were analyzed retrospectively. Depending on the year of pPCL diagnosis, 20% leukocytes or ≥2�109/L plasma cells in the peripheral blood were used.
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 in 31 (58.5%) patients, and PI and IMID were used simultaneously in 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 (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 of 15 months. Only 7 (13.2%) of the patients were alive at the last follow-up visit. Those with higher beta-2 microglobulin levels and ISS stage 3 and nontransplant patients receiving first-line treatment had shorter OS (p=0.005, p=0.02 and p=0.008, respectively). Otherwise, 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 our study, as in previous similar studies, we could not see the increased survival trend in pPCL which is observed in MM. New studies are needed for pPCL patients, which is likely to increase with the new diagnostic criteria, based on current agents and information in MM.

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

Proteazom �nhibit�rleri ve �mm�nomod�lat�r Ajanlar �a��nda Primer Plazma H�creli L�semili Hastalar�n Hayatta Kalma Sonu�lar�: Ger�ek Ya�am Verilerinden �ok Merkezli Bir Analiz

Ama�: Bu �al��ma ile multipl miyelom (MM) hastalar�nda genel sa�kal�mda (OS) anlaml� bir art�� sa�layan antimiyeloma ajanlar�n�n, prognozu daha k�t� olan primer plazma h�creli l�semi (pPHL) hastalar�nda kullan�m�na ili�kin ger�ek hayat verilerini ortaya koymak istedik.
Gere� ve Y�ntem: 2011�2020 y�llar� aras�nda pPHL tan�s� alan ve en az bir proteazom inhibit�r� (PI) ve/veya imm�nomod�lat�r (IMID) ajan kullanan 53 hastan�n verileri retrospektif olarak analiz edildi. Hastalar�n tan� y�llar�ndan kaynakl� olarak, periferik kanda plazma h�cresinin l�kositlerin %20�sinden fazla veya ≥2�109/L olmas� pPHL tan� kriteri kabul edildi.
Bulgular: Hastalar�n ortanca ya�� 58 olup, 23 (%43,4) hasta 65 ya� �zerindeydi. �lk s�ra tedavide 31 (%58,5) hastada PI veya IMID tek ba��na kullan�l�rken, 15 (%28,3) hastada PI ve IMID e� zamanl� kullan�ld�. Ayr�ca 21 (%39,6) hastaya nakil, 13 (%24,5) hastaya ise idame tedavisi uyguland�. Hastalar�n ortanca progresyonsuz sa�kal�m s�resi 4 (1�42) ayd�. �lk s�ra tedaviye primer refrakter hastalar d��land��nda ise 6,5 ayd�. Ortanca takip s�resi 15 ay olan hastalar�n, ortanca OS s�resi de 15 ayd�. Son kontrolde hastalar�n sadece 7'si (%13,2) hayattayd�. Beta-2 mikroglobulin d�zeyi y�ksek, ISS skoru 3 olan ve birinci basamak tedavide nakil yap�lmayan hastalarda OS daha k�sayd� (s�ras�yla, p=0.005, p=0.02 and p=0.008). �te yandan ind�ksiyon tedavisinde PI ve IMID ajanlar�n�n birlikte kullan�lmas�n�n, kemoterapi eklenmesinin, ind�ksiyon tedavisine yan�t�n ve idame tedavisinin OS �zerine etkisi olmad�� g�r�ld�.
Sonu�: �nceki benzer �al��malarda oldu�u gibi, �al��mam�zda pPHL�de MM'de g�zlenen artan sa�kal�m e�ilimini g�remedik. Yeni tan� kriteri ile birlikte artmas� olas� pPHL hastalar� i�in, MM�daki g�ncel ajanlar ve bilgiler dahilinde, yap�lacak yeni �al��malara ihtiya� vard�r.

Anahtar Kelimeler: Primer plazma h�creli l�semi, Antimiyelom ajanlar, Proteazom inhibit�rleri, �mm�nomod�lat�r ajanlar, Hematopoietik k�k h�cre nakli

Corresponding Author: Ozan Salim

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