Blood Res 2013; 48(3):
Published online September 25, 2013
https://doi.org/10.5045/br.2013.48.3.228
© The Korean Society of Hematology
1Hematology Unit, S. Eugenio Hospital, Rome, Italy.
2Pathology Department, S. Eugenio Hospital, Rome, Italy.
Correspondence to : Pasquale Niscola. Hematology Unit, S. Eugenio Hospital, Piazzale dell'Umanesimo 10, 00144, Rome, Italy. pniscola@gmail.com
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
The occurrence of monoclonal gammopathy of undetermined significance (MGUS) and essential thrombocytopenia (ET) in the same patient is quite rare [1-3], usually manifesting as an incidental finding. In addition, the coexistence of multiple myeloma (MM) with ET has also been rarely reported [4-8]. Moreover, the evolution of MGUS to MM simultaneously with blastic transformation of ET in the form of acute biphenotypic leukemia, as observed in this report, represents an exceptionally rare occurrence. With an anecdotal purpose, we herein report the long-term clinical history of a patient who presented with concomitant evolution of MGUS to MM and from ET to acute biphenotypic leukemia, with the original diagnosis of MGUS and ET occurring 37 years prior.
In 2010, a 77-year-old man presented to our center with increasing thrombocytosis and monoclonal paraproteinemia (IgG lambda). In 1975, at another center, he was diagnosed with MGUS associated with ET. The patient was managed according to the prevalent clinical guidelines and received low-dose acetylsalicylic acid (LD-ASA). Upon presentation to our clinic (35 years after original diagnosis and treatment), he reported that for several years he had not been followed up by periodic laboratory evaluations and hematologic examinations. Therefore, a comprehensive work-up, including a bone marrow (BM) aspirate and trephine biopsy, was performed. Megakaryocytic hyperplasia and clustering consistent with ET, along with an infiltration of IgG kappa clonally mature plasma cells (PC) consistent with MGUS, was noted.
This case lacks practical therapeutic implications and reliable indications for the management of this uncommon occurrence, and our report has only anecdotal value. However, the overlapping occurrence of acute biphenotypic leukemia transformed from ET and MM is extremely rare. We speculate that the synchronous evolution of ET and MGUS along with coexpression of lymphoid antigens by blastic cells could suggest a common origin of these 2 malignancies, potentially evolving from a common precursor by progressive transformation to more aggressive disorders [5]. However, this hypothesis remains to be investigated.
Blood Res 2013; 48(3): 228-230
Published online September 25, 2013 https://doi.org/10.5045/br.2013.48.3.228
Copyright © The Korean Society of Hematology.
Pasquale Niscola1*, Gianfranco Catalano1, Stefano Fratoni2, Laura Scaramucci1, Paolo de Fabritiis1, and Tommaso Caravita1
1Hematology Unit, S. Eugenio Hospital, Rome, Italy.
2Pathology Department, S. Eugenio Hospital, Rome, Italy.
Correspondence to: Pasquale Niscola. Hematology Unit, S. Eugenio Hospital, Piazzale dell'Umanesimo 10, 00144, Rome, Italy. pniscola@gmail.com
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
The occurrence of monoclonal gammopathy of undetermined significance (MGUS) and essential thrombocytopenia (ET) in the same patient is quite rare [1-3], usually manifesting as an incidental finding. In addition, the coexistence of multiple myeloma (MM) with ET has also been rarely reported [4-8]. Moreover, the evolution of MGUS to MM simultaneously with blastic transformation of ET in the form of acute biphenotypic leukemia, as observed in this report, represents an exceptionally rare occurrence. With an anecdotal purpose, we herein report the long-term clinical history of a patient who presented with concomitant evolution of MGUS to MM and from ET to acute biphenotypic leukemia, with the original diagnosis of MGUS and ET occurring 37 years prior.
In 2010, a 77-year-old man presented to our center with increasing thrombocytosis and monoclonal paraproteinemia (IgG lambda). In 1975, at another center, he was diagnosed with MGUS associated with ET. The patient was managed according to the prevalent clinical guidelines and received low-dose acetylsalicylic acid (LD-ASA). Upon presentation to our clinic (35 years after original diagnosis and treatment), he reported that for several years he had not been followed up by periodic laboratory evaluations and hematologic examinations. Therefore, a comprehensive work-up, including a bone marrow (BM) aspirate and trephine biopsy, was performed. Megakaryocytic hyperplasia and clustering consistent with ET, along with an infiltration of IgG kappa clonally mature plasma cells (PC) consistent with MGUS, was noted.
This case lacks practical therapeutic implications and reliable indications for the management of this uncommon occurrence, and our report has only anecdotal value. However, the overlapping occurrence of acute biphenotypic leukemia transformed from ET and MM is extremely rare. We speculate that the synchronous evolution of ET and MGUS along with coexpression of lymphoid antigens by blastic cells could suggest a common origin of these 2 malignancies, potentially evolving from a common precursor by progressive transformation to more aggressive disorders [5]. However, this hypothesis remains to be investigated.