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Primary Plasma Cell Leukemia Presenting with Chest Pain
Journal of Hematology & Thromboembolic Diseases

Journal of Hematology & Thromboembolic Diseases
Open Access

ISSN: 2329-8790

+44 20 3868 9735

Letter to Editor - (2015) Volume 3, Issue 6

Primary Plasma Cell Leukemia Presenting with Chest Pain

Gargantilla P1,2*, Arroyo N1 and Pintor E2
1Department of Internal Medicine, Hospital de El Escorial, Madrid, Spain
2European University of Madrid, Madrid, Spain
*Corresponding Author: Gargantilla P, Department of Internal Medicine, Hospital de El Escorial, Madrid Ctra. Guadarrama-Escorial Km 6,255, 28200 El Escorial, Madrid, Spain, Tel: 655525622 Email:

Abstract

The term plasma cell leukemia (PCL) is the most aggressive form of the plasma cell dyscrasias and it`s used when the number of circulating plasma cells is significant. It`s defined by the presence of >2 × 109/L peripheral blood plasma cells or accounting for >20% of the differential white cell count, that does not arise from preexisting multiple myeloma.

Keywords: Plasma cell leukemia; Myeloma; Chest pain

Letter to Editor

Plasma cell leukemia (PCL) is a rare and aggressive form of plasma cell dyscrasias and it`s used when the number of circulating plasma cells is significant. It is defined by an absolute plasmacytosis of greater than 2 × 109 cells/L or greater than 20% plasma cell in the peripheral blood [1]. PCL is classified as primary when it presents “de novo” in patients with no evidence of previous multiple myeloma and as secondary when it is observed as a leukemic transformation of relapsed or refractory diseases in patients with previously recognized multiple myeloma.

A 48-year-old Caucasian male with no significant past medical history was referred to our hospital because left side chest pain for four months. He had no exposure to environmental toxins. On physical exam, he was afebrile and he had no palpable adenopathy or hepatosplenomegaly. Chest X-ray revealed a lytic lesion in the 8th left rib. Initial laboratory investigations revealed the following: white blood cell count 61.3 × 109/L with 3% of plasma cells, hemoglobin 8.7 g/dl and normal platelet count. Beta-2 microglobulin and LDH were elevated (5.2 μg/mL and 717 U/L respectively). Peripheral blood smear showed red blood cell rolls and atypical appearance of white blood cells. A serum protein electrophoresis was performed that demonstrated an IgG lambda monoclonal protein (2.630 mg/dL). A monoclonal peak of 1.57 g/dL was seen in the serum. Urinary analysis showed immunoglobulin free light chains Kappa (Bence-Jones protein). Bone marrow biopsy revealed a diffuse infiltration of atypical plasma cells (85%) lacking blastic features. Flow cytometry immunophenotypic characteristics were as follows: positive for CD38, CD138, CD27, and negative for CD19, CD56, CD117. Patient was referred to the department of hematology where he received chemotherapy according to the protocol Dexamethasone, Lenalidomide and Bortezomib.

Plasma cell leukemia is a rare and aggressive plasma cell disorder. First cases of PCL were described at the beginning of the twentieth century [2]. It`s a different clinic-pathologic entity from myeloma multiple: PCL is observed in younger patients (median age ranged between 52 and 65 years), more common in African Americans than in Caucasians [3], with a increased incidence of urinary free lightchain (Bence-Jones). PCL is characterized by an aggressive clinical presentation, rapid clinical course, leukocytosis, extramedullary involvement, marked bone marrow infiltration by immature plasma cells and high LDH serum levels [3]. Evaluation of the plasma cells in patients with PCL demonstrates differences in marker expression between multiple myeloma and secondary plasma cell leukemia: C38 and CD138 are expressed in both, but CD56; characteristically expressed on myeloma cells, is absent from patients with PCL [4]. Combination therapy of alkylating agents and glucocorticoids are unsatisfactory. Nowadays, effective induction therapy is based in intensive chemotherapy with alkylating agents including anthracyclines such as HyperCVAD (hyper-fractionated cyclophospghamide, vincristine, doxorrubicin, dexamethasone) or PACE (cisplatin, doxorubicin, cyclophosphamide and etoposide) or bortemozib-based combinations [3]. Otherwise, in patients younger than 50 years of age with a suitable donor, a myeloablative allogeneic transplantation can be considered.

References

  1. Igala M, Bopaka RG, Khtabi W, Benchekroun S, Quessar A (2014) Primary plasma cell leukemia presenting as a thoracic mass. Pan Afr Med J 19: 39.
  2. Gluzinski A, Reichenstein M (1906) Myeloma und Leucaemialymphaticaplasmocellularis. Wien KlinWochensch 18: 336-339.
  3. Fernández de Larrea C, Kyle RA, Durie BGM, Usmani S (2013) Plasma cell leukemia: consensus statement on diagnostic requirements, response criteria and treatment recommendations by the Internacional Myeloma Working Group. Leukemia 1-12.
  4. Saccaro S, Fonseca R, Veillon DM, Cotelingam J, Nordberg ML, et al. (2005) 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 78: 288-294.
Citation: Gargantilla P, Arroyo N, Pintor E (2015) Primary Plasma Cell Leukemia Presenting with Chest Pain. J Hematol Thrombo Dis 3:225.

Copyright: © 2015 Gargantilla P, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.