title: Mast cell leukemia associated with undefined morphology and chronic basophilic leukemia.
abstract: BACKGROUND
  Mast cell leukemia (MCL) is rare type of neoplasia with an incidence of 1% in a large series of 342 adult patients with systemic mastocytosis (SM). Chronic basophilic leukemia (CBL) is an extremely rare type of leukemia with appearance of 7 cases in the literature.
  
  
  METHODS
  A 73 year-old female patient who presented with weaknes, had a prolonged duration of hematologic remission after treatment of her CBL by hydroxyurea (HU). Evolution of SM occurring as a second neoplasia concurrently with relapse of de novo CBL was demonstrated by mast cells (MCs) infiltration in the bone marrow (BM) biopsy and smear and increase in tryptase level. Transformation to MCL with simultaneous occurrance of accelerated phase of CBL were documented by the appearance of MCs in both BM and peripheral blood (PB) smears, antigen expressions detected by flow cytometry and spesific stains. Sequence analysis of c-kit gene revealed c-kit exon 11 K550N mutation. Undefined associations of MCL with different mast cell morphology, increase in IL-6 level and accelerated phase of de novo CBL was described.
  
  
  CONCLUSIONS
  Elevations in CRP and IL-6 levels occurring with increases in basophil counts to high levels revealed that febrile episodes with abdominal pain seen in our patient were induced by increase in IL-6 levels released from neoplastic basophils. Neoplastic basophils with diffuse and coarse basophilic granules possibly mimic neutrophils with toxic granules and cause wrong characterization of neoplastic basophils as neutrophils by the automated blood cell counters and misleaded physicians.
authors.affiliation: Division of Hematology, Dokuz Eylul University School of Medicine, 35330 Inciralti, Izmir, Turkey.
authors.forename: Cavit
authors.initials: C
authors.lastname: Cehreli
authors.affiliation: Division of Hematology, Dokuz Eylul University School of Medicine, 35330 Inciralti, Izmir, Turkey.
authors.forename: Inci
authors.initials: I
authors.lastname: Alacacioglu
authors.affiliation: Division of Hematology, Dokuz Eylul University School of Medicine, 35330 Inciralti, Izmir, Turkey.
authors.forename: Ozden
authors.initials: O
authors.lastname: Piskin
authors.affiliation: Division of Hematology, Dokuz Eylul University School of Medicine, 35330 Inciralti, Izmir, Turkey.
authors.forename: Halil
authors.initials: H
authors.lastname: Ates
authors.affiliation: Institute of Oncology, Dokuz Eylul University School of Medicine, Izmir, Turkey.
authors.forename: Ruksan
authors.initials: R
authors.lastname: Cehreli
authors.affiliation: Department of Basic Oncology, Dokuz Eylul University Institute of Oncology, İzmir, Turkey.
authors.forename: Gizem
authors.initials: G
authors.lastname: Calibasi
authors.affiliation: Departments of Medical Biology and Genetics, Dokuz Eylul University School of Medicine, Izmir, Turkey.
authors.forename: Erdinc
authors.initials: E
authors.lastname: Yuksel
authors.affiliation: Institute of Pathology Dokuz Eylul University School of Medicine, Izmir, Turkey.
authors.forename: Sermin
authors.initials: S
authors.lastname: Ozkal
authors.affiliation: Division of Hematology, Dokuz Eylul University School of Medicine, 35330 Inciralti, Izmir, Turkey.
authors.forename: Guner H
authors.initials: GH
authors.lastname: Ozsan
keywords: Basophilia; Dysplasia; Interleukin-6; Mast cell leukemia; Mastocytosis