PLASMA CELL LEUKAEMIA: A RARE YET AGGRESSIVE PLASMA CELL DYSCRASIA WITH A VERY POOR RESPONSE TO CONVENTIONAL THERAPY
AbstractPlasma cell leukaemia (PCL) is a very rare plasma cell dyscrasia with a significant number of monoclonal plasma cells in the peripheral blood. It is diagnosed by the presence of ≥ 2x109 /L plasma cells in the blood or by plasma cells making up ≥20% of the leukocyte count. It can arise from a leukemic transformation of multiple myeloma, or more commonly it can be primary. Regardless of its origin, it carries a very dire prognosis. It responds very poorly to the traditional chemotherapy regimens used for multiple myeloma. We present the case of a 50 years old female who presented to our hospital with a complicated UTI and severe generalized body aches. She was diagnosed as a case of plasma cell leukaemia and was treated with cyclophosphamide and dexamethasone, however she failed to go into remission. Her condition deteriorated and she ultimately passed away 1.5 months after diagnosis. The recommended treatment for PCL is aggressive combination chemotherapy followed by stem cell transplantation. However, there is no consensus regarding the treatment of plasma cell leukaemia, and treatment should be individualized based on the patient profile. Once diagnosed, the prognosis is poor.Keywords: Plasma Cell Leukaemia; Multiple Myeloma; Plasma Cell Dyscrasia; Bortezomib
Gluzinski A, Reichenstein M. Myeloma and leukaemia lymphatic a plasmocellularis. Wien Klin Wochenschr 1906;19:336.
Grogan TM, Van Camp B, Kyle RA. Plasma cell neoplasms. In: Jaffe ES, Harris NL, Stein H, Vardiman JW, editors. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. IARC Press: Lyon, 2001; p.142.
Bladé J, Kyle RA. Nonsecretory myeloma, immunoglobulin D myeloma, and plasma cell leukemia. Hematol Oncol Clin North Am 1999;13(6):1259–72.
Dimopoulos MA, Palumbo A, Delasalle KB, Alexanian R. Primary plasma cell leukemia. Br J Haematol 1994;88(4):754–9.
Sant M, Allemani C, Tereanu C, De Angelis R, Capocaccia R, Visser O, et al. Incidence of hematological malignancies in Europe by morphologic subtype: results of the HAEMACARE project. Blood 2010;116(19):3724–34.
Tiedemann RE, Gonzalez-Paz N, Kyle RA, Santana-Davila R, Price-Troska T, Van Wier SA, et al. Genetic aberrations and survival in plasma cell leukemia. Leukemia 2008;22(5):1044–52.
Pagano L, Valentini CG, De Stefano V, Venditti A, Visani G, Petrucci MT, et al. Primary plasma cell leukemia: a retrospective multicenter study of 73 patients. Ann Oncol 2011;22(7):1628–35.
Fernández de Larrea C, Kyle RA, Durie BG, Ludwig H, Usmani S, Vesole DH, et al. Plasma cell leukemia: consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group. Leukemia 2013;27(4):1–12.
Craig FE, Foon KA. Flow cytometric immunophenotyping for hematologic neoplasms. Blood 2008;111(8):3941–67.
Ioannou MG, Stathakis E, Lazaris AC, Papathomas T, Tsiambas E, Koukoulis GK. Immunohistochemical evaluation of 95 bone marrow reactive plasmacytosis. Pathol Oncol Res 2009;15(1):25–9.
Katodritou E, Terpos E, Kelaidi C, Kotsopoulou M, Delimpasi S, Kyrtsonis MC, et al. Treatment with bortezomib-based regimens improves overall survival in patients with primary or secondary plasma cell leukemia: Analysis of the Greek myeloma study group. Am J Hematol 2014;89(2):145–50.
Musto P, Rossini F, Gay F, Pitini V, Guglielmelli T, D’Arena G, et al. Efficacy and safety of bortezomib in patients with plasma cell leukemia. Cancer 2007;109(11):2285–90.
Saccaro S, Fonseca R, Veillon DM, Cotelingam J, Nordberg ML, Bredeson C, et al. Primary plasma cell leukemia: report of 17 new cases treated with autologous or allogeneic stem-cell transplantation and review of the literature. Am J Hematol 2005;78(4):288–94.
Jameel A. Plasma cell leukemia: case report of a rare and aggressive variant of multiple myeloma. J Pak Med Assoc 2005;55(10):452–3.