Primary plasma cell leukemia is a rare disorder accounting for less than 5% of malignant plasma cell diseases. It has a poor prognosis compared to multiple myeloma, with a median survival of 8–12 months. The results of conventional therapy are disappointing though autologous stem cell transplantation improve survival. In studies of this approach, both patients who achieved CR and those falling short of CR obtained a benefit. In one study (Gowda et al), best post auto-HCT response was CR in 6 (26%), nCR in 1 (4%), VGPR in 4 (17%) and PR in 6 (26%) with an overall response rate of 74%. After a median follow up of 18.1 months, the median progression-free survival (PFS) and overall survival (OS) was 5.5 and 18.1 months, respectively. As such, even without knowing the level of response, an autologous stem cell transplant would be considered medically necessary.
Drake MB, Iacobelli S, van Biezen A, Morris C, Apperley JF, Niederwieser D, Björkstrand B, Gahrton G; European Group for Blood and Marrow Transplantation and the European Leukemia Net. Primary plasma cell leukemia and autologous stem cell transplantation. Haematologica. 2010 May; 95(5): 804-9.
Gowda L, Shah M, Badar I, Bashir Q, Shah N, Patel K, Kanagal-Shamanna R, Mehta R, Weber DM, Lee HC, Manasanch EE, Shah A, Thomas SK, Parmar S, Nieto Y, Orlowski RZ, Champlin R, Qazilbash MH. Primary plasma cell leukemia: autologous stem cell transplant in an era of novel induction drugs. Bone Marrow Transplant. 2019 Jul; 54(7): 1089-1093.
Jung S., Lee J., Kim K., Suh C., Yoon D. Hyun, Min C., Sohn S. Kyun, Choi C. Won, Lee H. Sup, Kim H. Jung, Shin H., Bang S., Yoon S., et al The role of frontline autologous stem cell transplantation for primary plasma cell leukemia: a retrospective multicenter study (KMM160). Oncotarget. 2017; 8: 79517-79526