title: Coexistence of plasma cell dyscrasia with prefibrotic stage of primary myelofibrosis: a case report.
abstract: Introduction. Coexistence of myeloproliferative neoplasms with lymphoproliferative syndromes has been described in the past, whereas plasma cell dyscrasias seem to be the most common cases. Case Presentation. We present a case of a 59-year-old Caucasian female of Greek origin who presented with thrombocytosis. Clinical and laboratory investigation disclosed the presence of a smoldering myeloma with coexisting histological and molecular characteristics of primary myelofibrosis. The patient had the acquired point mutation V617F in the JAK2 gene but not the bcr-abl rearrangement and was treated for myelofibrosis with subsequent improvement of all haematological parameters without evidence of myelomatic evolution. Conclusion. We present the first case in the literature of a smoldering myeloma coexisting with primary myelofibrosis. The underlying pathogenetic mechanism could be either related to the presence of a pluripotent neoplastic stem cell capable to differentiate into both lymphoid and myeloid cells or be related to two separate nosologic entities.
authors.affiliation: Department of Haematology, University Hospital of Heraklion, 71110 Heraklion Crete, Greece.
authors.forename: George
authors.initials: G
authors.lastname: Tsirakis
authors.forename: Peggy
authors.initials: P
authors.lastname: Kanellou
authors.forename: Maria
authors.initials: M
authors.lastname: Kaparou
authors.forename: Andrew
authors.initials: A
authors.lastname: Passam
authors.forename: Amalia
authors.initials: A
authors.lastname: Zampoulaki
authors.forename: Kostas
authors.initials: K
authors.lastname: Stylianou
authors.forename: Michael G
authors.initials: MG
authors.lastname: Alexandrakis
references.citation: Lancet. 2005 Mar 19-25;365(9464):1054-61
references.pmid: 15781101
references.citation: Nature. 2005 Apr 28;434(7037):1144-8
references.pmid: 15793561
references.citation: Acta Haematol. 1995;94(2):98-101
references.pmid: 7484021
references.citation: Acta Haematol. 1982;67(2):139-40
references.pmid: 6801897
references.citation: Cancer Cell. 2005 Apr;7(4):387-97
references.pmid: 15837627
references.citation: Haematologica. 2003 Apr;88(4):ECR09
references.pmid: 12681982
references.citation: Baillieres Clin Haematol. 1998 Dec;11(4):721-49
references.pmid: 10640214
references.citation: Br J Haematol. 1999 Mar;104(4):730-7
references.pmid: 10192432
references.citation: Am J Hematol. 1998 Sep;59(1):79-82
references.pmid: 9723582
references.citation: Cancer. 1994 Feb 1;73(3):625-8
references.pmid: 8299083
references.citation: Br J Haematol. 1993 Nov;85(3):514-20
references.pmid: 7510989
references.citation: Oncology. 1981;38(6):369-72
references.pmid: 7301283
references.citation: J Clin Invest. 1985 Apr;75(4):1388-90
references.pmid: 3921571
references.citation: Acta Haematol. 2004;111(3):155-9
references.pmid: 15034237
references.citation: Am J Clin Pathol. 1988 Jan;89(1):63-8
references.pmid: 3337052
references.citation: Blood. 2005 Oct 15;106(8):2920-1
references.pmid: 16204151
references.citation: Am J Med. 1985 Sep;79(3):297-302
references.pmid: 4036981
references.citation: Am J Clin Pathol. 2003 Jan;119(1):152-8
references.pmid: 12520711
references.citation: Blood. 1996 Mar 1;87(5):1928-38
references.pmid: 8634441
references.citation: N Engl J Med. 2005 Apr 28;352(17):1779-90
references.pmid: 15858187