Primary Plasma Cell Leukemia: A Retrospective Study of a Rare Disease From Tertiary Cancer Centre From India
- 11 Downloads
Plasma cell leukemia (PCL) is an aggressive rare leukemic variant of multiple myeloma (MM). We aim to present 4 years data on clinical profile and treatment outcomes of Primary PCL (PPCL) patients treated at tertiary care cancer centre from Northern India. To analyse response and safety profile of a PPCL with or without stem cell transplantation. Retrospectively reviewed and analysed PPCL patient’s data at our centre from January-2013 to June-2017. Total 11 PPCL patients diagnosed among 240 MM patients during study period. Eight were males. Only 10 patients were started on treatment. Four (n = 4/10) patients underwent stem cell transplantation. Overall response rate was 70% (n = 7). Eleven culture positive bacterial infections (bloodstream = 2, urinary tract = 3; pulmonary = 6) were recorded. Four patients had fungal infections. One patient had Herpes Zoster infection. Relapse rate of entire cohort was 50% (n = 5). Median PFS and OS of entire cohort was 11 months (95% confidence interval 6.3–15.6) and 21 months (95% C.I. 1–49.8) respectively. The estimated PFS and OS at 1 year of transplanted versus nontransplanted patients were 71% + 24% versus 0% (P = 0.96) and 71% + 24% versus 15% + 19% (P = 0.234) respectively. Treatment with PIs + IMAs followed by transplants (single/double) might improve depth and duration of remission and OS. Patients should be treated with indefinite maintenance therapy to control disease.
KeywordsPrimary plasma cell leukemia Stem cell transplantation Multiple myeloma Overall survival
We would like to acknowledge an entire team of hemato-oncology and bone marrow transplant unit, Rajiv Gandhi Cancer Institute & Research Centre for their continuous support for retrieving records of eligible patients.
Compliance with Ethical Standards
Conflict of interest
All authors have declare that they have no conflict of interest.
Human and Animal Rights
All procedures performed in study involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. The study was approved by our Institutional Review Board (IRB).
This is a retrospective study hence for this type of study formal consent is not required. IRB waived off the need to take consent for the study.
- 1.Fernández de Larrea C, Kyle RA, Durie BG, Ludwig H, Usmani S, Vesole DF et al (2013) International Myeloma Working Group. Plasma cell leukemia: consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group. Leukemia 27:780–791CrossRefGoogle Scholar
- 2.Pasqualethi P, Festuccia V, Collacciani A, Acitelli P, Casale R (1996) Plasma cell leukemia, a report on 11 cases and review of the literature. Panminerra Med. 38:179–184Google Scholar
- 10.International Myeloma Working (IMW) (http://imwg.myeloma.org/)
- 15.Musto P, D’Auria F, Petrucci MT, Levi A, Cascavilla N, Falcone A et al. (2011) A: final results of a phase II study evaluating lenalidomide in combination with low dose dexamethasone as first line therapy for primary plasma cell leukemia. Blood (ASH Annual Meeting Abstracts). p 118 (Abstract 2925)Google Scholar
- 17.D’Arena G, Valentini CG, Pietrantuono G, Guariglia R, Martorelli MC, Mansueto G et al (2012) Frontline chemotherapy with bortezomib-containing combinations improves response rate and survival in primary plasma cell leukemia: a retrospective study from GIMEMA Multiple Myeloma Working Party. Ann Oncol 23:1499–1502CrossRefGoogle Scholar
- 22.Royer B, Minvielle S, Diouf M, Roussel M, Karlin L, Hulin C et al (2016) Bortezomib, doxorubicin, cyclophosphamide, dexamethasone induction followed by stem cell transplantation for primary plasma cell leukemia: a prospective phase II study of the Intergroupe Francophone du Myélome. J ClinOncol 34:2125–2132CrossRefGoogle Scholar