Vol 12, No 1 (2021)
Case report
Published online: 2021-09-29

open access

Page views 3247
Article views/downloads 381
Get Citation

Connect on Social Media

Connect on Social Media

Efficacy of lenalidomide in the treatment of myelodysplastic/myeloproliferative neoplasms, unclassifiable (MDS/MPN-U) with the presence of deletion 5q– and trisomy 8+

Oliwia Bachanek-Mitura1, Aneta Szudy-Szczyrek1, Marek Hus1
Hematology in Clinical Practice 2021;12(1):18-22.

Abstract

Myelodysplastic/myeloproliferative neoplasms (MDS/MPN) are very rare clonal myeloid characterized by the simultaneous presence of both of erythrocytic and/orgranulocytic dysplasia with myeloproliferative features. The etiology of the disease remains unknown. The MDS/MPN group is made up of five disorders: chronic myelomonocytic leukemia, atypical chronic myeloid leukemia, juvenile myelomonocytic leukemia, MDS/MPN ring sideroblasts with thrombocytosis and MDS/MPN, un classifiable (MDS/MPN-U). MDS/MPN-U remains the leastcharacterized entity. It accounts less than 5% of all myeloid disorder. The reare currently no well-established diagnostic criteria. The disease is heterogeneous with outclear management strategies.

The aim of thispaperis to present a case of a 59-year-old female patient diagnosed with MDS/MPN-U with deletion 5q– and trisomy 8+, whom lenalidomide 10 mg daily has been used as monotherapy. The disease has initially manifested as severe cytopenias, multiple transfusions of blood preparations were required. Currently patientis in the 9th month of treatment. Hemoglobin, platelets and white blood cells returned to normal. The patient completely became independent of transfusion of red blood cell concentrates. No adverse events were observed.

Article available in PDF format

View PDF Download PDF file

References

  1. Sochacki AL, Fischer MA, Savona MR. Therapeutic approaches in myelofibrosis and myelodysplastic/myeloproliferative overlap syndromes. Onco Targets Ther. 2016; 9: 2273–2286.
  2. Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016; 127(20): 2391–2405.
  3. DiNardo CD, Daver N, Jain N, et al. Myelodysplastic/myeloproliferative neoplasms, unclassifiable (MDS/MPN, U): natural history and clinical outcome by treatment strategy. Leukemia. 2014; 28(4): 958–961.
  4. Talati C, Padron E. An exercise in extrapolation: clinical management of atypical CML, MDS/MPN-unclassifiable, and MDS/MPN-RS-T. Curr Hematol Malig Rep. 2016; 11(6): 425–433.
  5. Giagounidis AAN, Germing U, Aul C. Biological and prognostic significance of chromosome 5q deletions in myeloid malignancies. Clin Cancer Res. 2006; 12(1): 5–10.
  6. Giagounidis AAN, Germing U, Strupp C, et al. Prognosis of patients with del(5q) MDS and complex karyotype and the possible role of lenalidomide in this patient subgroup. Ann Hematol. 2005; 84(9): 569–571.
  7. List A, Kurtin S, Roe DJ, et al. Efficacy of lenalidomide in myelodysplastic syndromes. N Engl J Med. 2005; 352(6): 549–557.
  8. List A, Ebert BL, Fenaux P. A decade of progress in myelodysplastic syndrome with chromosome 5q deletion. Leukemia. 2018; 32(7): 1493–1499.
  9. Arcioni F, Roncadori A, Di Battista V, et al. MORE Study Centres. Lenalidomide treatment of myelodysplastic syndromes with chromosome 5q deletion: Results from the National Registry of the Italian Drug Agency. Eur J Haematol. 2018; 101(1): 78–85.
  10. Ziarkiewicz M, Dwilewicz-Trojaczek J, Pastwińska A, et al. Refractory anaemia with ringed sideroblasts associated with marked thrombocytosis (RARS-T) with superimposed 5q-syndrome. Pol J Pathol. 2010; 61(2): 105–109.
  11. Alshaban A, Padilla O, Philipovskiy A, et al. Lenalidomide induced durable remission in a patient with MDS/MPN-with ring sideroblasts and thrombocytosis with associated 5q- syndrome. Leuk Res Rep. 2018; 10: 37–40.



Hematology in Clinical Practice