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Open Access 01-03-2009 | Original Article

Systemic mastocytosis associated with t(8;21)(q22;q22) acute myeloid leukemia

Authors: Sheeja T. Pullarkat, Vinod Pullarkat, Steven H. Kroft, Carla S. Wilson, Arshad N. Ahsanuddin, Karen P. Mann, Maung Thein, Wayne W. Grody, Russell K. Brynes

Published in: Journal of Hematopathology | Issue 1/2009

Abstract

Although KIT mutations are present in 20–25% of cases of t(8;21)(q22;q22) acute myeloid leukemia (AML), concurrent development of systemic mastocytosis (SM) is exceedingly rare. We examined the clinicopathologic features of SM associated with t(8;21)(q22;q22) AML in ten patients (six from our institutions and four from published literature) with t(8;21) AML and SM. In the majority of these cases, a definitive diagnosis of SM was made after chemotherapy, when the mast cell infiltrates were prominent. Deletion 9q was an additional cytogenetic abnormality in four cases. Four of the ten patients failed to achieve remission after standard chemotherapy and seven of the ten patients have died of AML. In the two patients who achieved durable remission after allogeneic hematopoietic stem cell transplant, recipient-derived neoplastic bone marrow mast cells persisted despite leukemic remission. SM associated with t(8;21) AML carries a dismal prognosis; therefore, detection of concurrent SM at diagnosis of t(8;21) AML has important prognostic implications.

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Title: Systemic mastocytosis associated with t(8;21)(q22;q22) acute myeloid leukemia
Authors: Sheeja T. Pullarkat
Vinod Pullarkat
Steven H. Kroft
Carla S. Wilson
Arshad N. Ahsanuddin
Karen P. Mann
Maung Thein
Wayne W. Grody
Russell K. Brynes
Publication date: 01-03-2009
Publisher: Springer-Verlag
Published in: Journal of Hematopathology / Issue 1/2009
Print ISSN: 1868-9256
Electronic ISSN: 1865-5785
DOI: https://doi.org/10.1007/s12308-009-0023-2

At a glance: The STEP trials

Springer Medicine

Systemic mastocytosis associated with t(8;21)(q22;q22) acute myeloid leukemia

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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