Gingival myeloid sarcoma in myelodysplastic syndrome

Abstract

To the Editor, Myeloid sarcoma (MS) of the gingiva is an extremely rare observation in the context of different myeloproliferative disorders [1–3], including chronic myelomonocytic leukemia (CMML) [1, 4] and acute myeloid leukemia (AML). In CMML patients, gingival hypertrophy is often associated with tumor progression and a more aggressive disease [4]. Although the evolution of myelodysplastic syndrome (MDS) in overt AML is a common occurrence, isolated gingival MS in patients with stable MDS without AML progression has been only exceptionally observed [3]. Herein, we report on a case of gingival MS occurred as isolated AML progression in a previously diagnosed MDS. The patient was a 63year-old Caucasian male who was diagnosed as having MDS, subtype refractory cytopenia with multilineage dysplasia; he presented with trilinear cytopenia (transfusion-dependent severe anemia, thrombocytopenia, and neutropenia), bone marrow (BM) hypocellularity with 3 % of blasts and a cytogenetic abnormality consistent with trisomy 8. According to the IPSS [5] and WPSS [6], the MDS risk was classified as Int-1 and intermediate categories, respectively. The patient had always been healthy and had no significant comorbidities; in particular, no oral and/or gastrointestinal diseases were reported. Given the immune-mediated pathogenesis likely involved in most forms of hypoplastic MDS [7], the patient was managed by cyclosporine. This treatment effort resulted in a substantially maintained stable disease without any significant toxicity. Six months after the diagnosis, the patient complained a well-circumscribed little enlargement of the right upper gingiva surrounded by a painful ulcer. An excision biopsy of the intraoral mass was performed. Histological analysis showed submucosal infiltration by aggregates of myeloperoxidase and CD68 positive myeloid blast cells. A comprehensive hematological revaluation, including BM aspirate and trephine biopsy and a karyotype analysis, revealed a stable MDS without any evidence of AML progression in the BM; in particular, the percentage of blast cells was the same of that found at the MDS diagnosis. Cytogenetic analysis confirmed the presence of trisomy 8. Two courses of fludarabine and high dose of cytosine arabinoside regimen were given resulting in the disappearance of the gingival swelling and the BM blast cells. A suitable donor was not available, for which autologous stem cell transplantation (ASCT) with busulfan and cyclophosfamide conditioning regimen was given as consolidation treatment. However, 3 months after ASCT, he presented a relapse of AML, without any extramedullary localization. AML rapidly progressed until the patient’s death. The unusual intraoral localization of MS and the presence of the trisomy 8 in our case, may suggest a possible link between these findings. Indeed, recurrent oral ulcers and the excess of chromosome The authors have no affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties. No writing assistance was utilized in the production of this manuscript. The authors declare that they have full control of all primary data and they agree to allow the journal to review their data if requested. A. Tendas : L. Scaramucci :M. Giovannini : L. Cupelli : A. Perrotti : P. de Fabritiis Hematology Unit, S. Eugenio Hospital, Rome, Italy