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Myelomastocytic Leukemia: Evidence for the Origin of Mast Cells from the Leukemic Clone and Eradication by Allogeneic Stem Cell Transplantation

Authors' Affiliations: ¹ Department of Internal Medicine I, Division of Hematology and Hemostaseology; ² Department of Internal Medicine I, Division of Oncology-the Center of Excellence in Clinical and Experimental Oncology (CLEXO); ³ Department of Internal Medicine I, Division of Bone Marrow Transplantation; ⁴ Clinical Institute of Medical and Chemical Laboratory Diagnostics; ⁵ Institute of Medical Biology; and ⁶ Institute for Clinical Pathology, Medical University of Vienna, Austria

Requests for reprints: Wolfgang R. Sperr, Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Währinger Gürtel 18-20, A-1090 Vienna, Austria. Phone: 43-1-404-6085; Fax: 43-1-402-6930; E-mail: wolfgang.r.sperr{at}meduniwien.ac.at.

Purpose: Myelomastocytic leukemia is a term used for patients with advanced myeloid neoplasms, in whom elevated numbers of immature atypical mast cells are found, but criteria for a primary mast cell disease are not met. The origin of mast cells in these patients is presently unknown.

Patient and Methods: We have analyzed clonality of mast cells in an 18-year-old patient suffering from acute myeloid leukemia with a complex karyotype including a t(8;21) and mastocytic transformation with a huge increase in immature mast cells and elevated serum tryptase level, but no evidence for a primary mast cell disease/mastocytosis.

Results: As assessed by in situ fluorescence hybridization combined with tryptase staining, both the tryptase-negative blast cells and the tryptase-positive mast cells were found to contain the t(8;21)-specific AML1/ETO fusion gene. Myeloablative stem cell transplantation resulted in complete remission with consecutive disappearance of AML1/ETO transcripts, decrease of serum tryptase to normal range, and disappearance of neoplastic mast cells.

Conclusion: These data suggest that mast cells directly derive from the leukemic clone in patients with myelomastocytic leukemia.

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