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A case of JAK‐2 positive essential thrombocythemia followed by mantle cell lymphoma
Author(s) -
Sheikh Umer N,
Socec Catherine,
Snower Daniel
Publication year - 2012
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.26.1_supplement.657.17
Subject(s) - medicine , mantle cell lymphoma , essential thrombocythemia , malignancy , cancer research , lymphoma , oncology , gastroenterology , polycythemia vera
A switch from chronic myeloproliferative neoplasm (MPN) to lymphoid malignancy is not an uncommon phenomenon. However, progression from a JAK‐2 positive essential thrombocythemia (ET) to mantle cell lymphoma (MCL) has not been reported. Methods We describe the case of a 69 year old man who developed colonic MCL, 44 months after the diagnosis of an MPN, which was classified according to WHO criteria as JAK‐2 positive ET. The patient was treated for ET with hydroxyurea (HU) for 66 months and with etanercept (anti‐TNF‐α) for ankylosing spondylitis for approximately six years, during which time he was diagnosed with MCL. Results The transformation from ET to MCL was accompanied by clonal evolution from a normal to a complex karyotype that included deletions of 6q and 11q, loss of 12p and 20p, monosomies 13 and 14, and structural rearrangement of 14q32. Conclusions Diverse pathways must be considered in this transformation process. Although therapeutic induction of MCL by anti‐TNF‐α therapy is extremely rare, MCL in this case might have been related to anti‐TNF‐α therapy. Chance coincidence of both disorders seems improbable, especially given the short interval from the diagnosis of ET to the development of MCL, but nonetheless may be considered. A third explanation might be provided by a spontaneous switch, which would further support the theory that MPNs are disorders of a pluripotent stem cell.