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Relapse of postpolycythemia myelofibrosis after allogeneic stem cell transplantation in a polycythemic phase: successful treatment with donor lymphocyte infusion directed by quantitative PCR test for V617F-JAK2 mutation

Leukemia volume 22, pages 1961–1963 (2008)Cite this article

Allogeneic stem cell transplantation (SCT) with reduced-intensity conditioning (RIC) has been demonstrated to be effective in achieving long-term disease-free survival with an acceptable toxicity in older patients with myelofibrosis, and patients often demonstrate reversal of marrow fibrosis and cytopenias.1, 2 When disease relapses, patients may benefit from donor lymphocyte infusion (DLI) and achieve remission.3 The accepted mechanism of this therapy is harnessing of the graft-versus-tumor effect (GvT); however, it may be associated with graft-versus-host disease (GVHD) and related morbidity and mortality. Here, we describe a patient with postpolycythemic myeloid metaplasia (PPMM) who relapsed after SCT with a clinical presentation of polycythemia vera (PV) and responded to DLI without demonstrating GVHD. Moreover, analysis of the JAK2-V617F mutation levels correlated with his disease course and demonstrated a ‘molecular remission’ after receiving DLI.

This case presentation shows a few unique aspects. Patients suffering from PV usually enjoy prolonged survival compared with other myeloproliferative disorders. After a number of years, the erythrocytic phase of the disease frequently becomes inactive and subsequently patients may develop a spent phase or PPMM indistinguishable from idiopathic myelofibrosis. These patients have short survival and their quality of life is compromised by asthenia, increasing splenomegaly, weight loss and anorexia. Chemotherapy can result in return of a malignant disease to the preceding less-advanced phase. Chronic myeloid leukemia in blastic phase may return to chronic phase and secondary acute myeloid leukemia to myelodysplastic syndrome following chemotherapy or at relapse after SCT. However, only rare cases of PPMM have been described in which chemotherapy treatment has resulted in reversed direction of the disease course, with significant decrease in marrow fibrosis associated with the development of an elevated red cell mass, a state indistinguishable from PV.5

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  1. Division of Hematology and Bone Marrow Transplantation, Chaim Sheba Medical Center, Tel Hashomer, Israel

    O Benjamini, M Koren-Michowitz, N Amariglio, N Kroger, A Nagler & A Shimoni

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  1. O Benjamini
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  2. M Koren-Michowitz
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  3. N Amariglio
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  4. N Kroger
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  6. A Shimoni
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Correspondence to A Shimoni.

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Benjamini, O., Koren-Michowitz, M., Amariglio, N. et al. Relapse of postpolycythemia myelofibrosis after allogeneic stem cell transplantation in a polycythemic phase: successful treatment with donor lymphocyte infusion directed by quantitative PCR test for V617F-JAK2 mutation. Leukemia 22, 1961–1963 (2008). https://doi.org/10.1038/leu.2008.215

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