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. 2024; 41(4): 225-235 | DOI: 10.4274/tjh.galenos.2024.2023.0450

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

�nal Ata�¹, Ozan Salim², Utku Iltar², Orhan Kemal Y�cel², Ay�e Hilal K��kdiler Ero�lu⁴, Vedat Aslan³, Murat Y�ld�r�m⁵, �zen Dedeo�lu⁶, Sema Se�ilmi�⁷, Turgay Ula�⁷, Burak Deveci⁸, Sedanur Karaman G�lsaran⁹, Ayfer Ged�k¹⁰, Fatih Yaman¹¹, �brahim Ethem P�nar¹², Senem Maral¹³, Ahmet Sar�c�¹⁴, Serhat �elik¹⁵, Hande O�ul¹⁶, S�d�ka G�lkan �zkan¹⁷, Aliihsan Gemici¹⁸, Ahmet Kur�at G�ne�¹⁹, An�l Tombak²⁰, �rfan Yava�o�lu⁴, Volkan Karaku�³, Melda C�mert⁵, Tayfur Topta�⁶, Mehmet Sinan Dal⁷, Rabin Saba⁸, Hakk� Onur K�rk�zlar⁹, �zg�r Mehtap¹⁰, Eren G�nd�z¹¹, Fahir �zkalemka�¹², Murat Albayrak¹³, �lhami Berber¹⁴, Muzaffer Keklik¹⁵, Nil G�ler¹⁶, Hasan Atilla �zkan¹⁷, �m�r G�kmen Sevindik¹⁸, Zahit Bolaman⁴, Erdal Kurto�lu³, Meltem Ayl�⁵, T�lin F�ratl� Tu�lular⁶, Fevzi Altunta�⁷, Levent �ndar²
¹Akdeniz University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Antalya, T�rkiye; University of Health Sciences T�rkiye, Antalya Training and Research Hospital, Clinic of Internal Medicine, Division of Hematology, Antalya, T�rkiye
² Akdeniz University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Antalya, T�rkiye
³ University of Health Sciences T�rkiye, Antalya Training and Research Hospital, Clinic of Internal Medicine, Division of Hematology, Antalya, T�rkiye
⁴Ayd�n Adnan Menderes University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Ayd�n, T�rkiye
⁵University of Health Sciences T�rkiye, Ankara G�lhane Training and Research Hospital, Clinic of Hematology, Ankara, T�rkiye
⁶Marmara University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, �stanbul, T�rkiye
⁷University of Health Sciences T�rkiye, Dr. Abdurrahman Yurtaslan Ankara Oncology Training and Research Hospital, Clinic of Hematology, Ankara, T�rkiye
⁸Antalya Bilim University Faculty of Medicine, Department of Hematology, Antalya, T�rkiye
⁹Trakya University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Edirne, T�rkiye
¹⁰Kocaeli University, Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Kocaeli, T�rkiye
¹¹Eski�ehir Osmangazi University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Eski�ehir, T�rkiye
¹²Bursa Uluda� University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Bursa, T�rkiye
¹³University Health Sciences T�rkiye, D��kap� Y�ld�r�m Beyaz�t Training and Research Hospital, Clinic of Internal Medicine, Division of Hematology, Ankara, T�rkiye
¹⁴�n�n� University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Malatya, T�rkiye
¹⁵Erciyes University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Kayseri, T�rkiye
¹⁶Pamukkale University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Denizli, T�rkiye
¹⁷Yeditepe University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, �stanbul, T�rkiye
¹⁸�stanbul Medipol University, Medipol Mega Hospital, Department of Internal Medicine, Division of Hematology, �stanbul, T�rkiye
¹⁹Ankara Etlik City Hospital, Clinic of Internal Medicine, Division of Hematology, Ankara, T�rkiye
²⁰Mersin University Faculty of Medicine, Department of Internal 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 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.

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 Sa�kal�m 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 ≥2x109/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�. β2 mikroglobulin d�zeyi y�ksek, Uluslararas� Evreleme Sistemi skoru 3 olan ve birinci basamak tedavide nakil yap�lmayan hastalarda OS daha k�sayd� (s�ras�yla, p=0,005, p=0,02 ve 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

�nal Ata�, Ozan Salim, Utku Iltar, Orhan Kemal Y�cel, Ay�e Hilal K��kdiler Ero�lu, Vedat Aslan, Murat Y�ld�r�m, �zen Dedeo�lu, Sema Se�ilmi�, Turgay Ula�, Burak Deveci, Sedanur Karaman G�lsaran, Ayfer Ged�k, Fatih Yaman, �brahim Ethem P�nar, Senem Maral, Ahmet Sar�c�, Serhat �elik, Hande O�ul, S�d�ka G�lkan �zkan, Aliihsan Gemici, Ahmet Kur�at G�ne�, An�l Tombak, �rfan Yava�o�lu, Volkan Karaku�, Melda C�mert, Tayfur Topta�, Mehmet Sinan Dal, Rabin Saba, Hakk� Onur K�rk�zlar, �zg�r Mehtap, Eren G�nd�z, Fahir �zkalemka�, Murat Albayrak, �lhami Berber, Muzaffer Keklik, Nil G�ler, Hasan Atilla �zkan, �m�r G�kmen Sevindik, Zahit Bolaman, Erdal Kurto�lu, Meltem Ayl�, T�lin F�ratl� Tu�lular, Fevzi Altunta�, Levent �ndar. 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. 2024; 41(4): 225-235

Corresponding Author: Ozan Salim

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