title: An adult patient with systemic mastocytosis and B-acute lymphoblastic leukemia.
abstract: Mastocytosis is a myeloproliferative neoplasm characterized by clonal expansion of abnormal mast cells, ranging from the cutaneous forms of the disease to mast cell leukemia. In a significant proportion of patients, systemic mastocytosis (SM) coexists with another hematologic malignancy, termed systemic mastocytosis with an associated hematologic nonmast cell lineage disorder (SM-AHNMD). Despite the pronounced predominance of concomitant myeloid neoplasms, the much more unusual coexistence of lymphoproliferative diseases has also been reported. Imatinib mesylate (IM) has a role in the treatment of SM in the absence of the KITD816V mutation. In the setting of SM-AHNMD, eradicating the nonmast cell malignant clone greatly affects prognosis. We report a case of an adult patient with SM associated with B-lineage acute lymphoblastic leukemia (B-ALL). Three cases of concurrent adult ALL and mastocytosis have been reported in the literature, one concerning SM and two concerning cutaneous mastocytosis (CM), as well as six cases of concomitant CM and ALL in children.
authors.affiliation: Hematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, Greece.
authors.forename: Theodoros
authors.initials: T
authors.lastname: Iliakis
authors.affiliation: Hematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, Greece.
authors.forename: Niki
authors.initials: N
authors.lastname: Rougkala
authors.affiliation: Hematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, Greece.
authors.forename: Panagiotis T
authors.identifier: 0000-0003-2692-5944
authors.initials: PT
authors.lastname: Diamantopoulos
authors.affiliation: Hematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, Greece.
authors.forename: Vasiliki
authors.initials: V
authors.lastname: Papadopoulou
authors.affiliation: Hematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, Greece.
authors.forename: Fani
authors.initials: F
authors.lastname: Kalala
authors.affiliation: Hematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, Greece.
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Zervakis
authors.affiliation: Hematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, Greece.
authors.forename: Nefeli
authors.initials: N
authors.lastname: Giannakopoulou
authors.affiliation: Hematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, Greece.
authors.forename: Polixeni
authors.initials: P
authors.lastname: Chatzinikolaou
authors.affiliation: Department of Pathology, National and Kapodistrian University of Athens, Athens, Greece.
authors.forename: Georgia
authors.identifier: 0000-0002-1398-178X
authors.initials: G
authors.lastname: Levidou
authors.affiliation: Department of Pathology, National and Kapodistrian University of Athens, Athens, Greece.
authors.forename: Eleftheria
authors.initials: E
authors.lastname: Lakiotaki
authors.affiliation: Department of Pathology, National and Kapodistrian University of Athens, Athens, Greece.
authors.forename: Penelope
authors.initials: P
authors.lastname: Korkolopoulou
authors.affiliation: Department of Pathology, National and Kapodistrian University of Athens, Athens, Greece.
authors.forename: Efstratios
authors.initials: E
authors.lastname: Patsouris
authors.affiliation: Hematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, Greece.
authors.forename: Eleni
authors.initials: E
authors.lastname: Variami
authors.affiliation: Hematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, Greece.
authors.forename: Nora-Athina
authors.initials: NA
authors.lastname: Viniou