title: Diagnosis, complications and management of chronic neutrophilic leukaemia: A case report.
abstract: Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative neoplasm characterized by sustained neutrophilia and the absence of the Philadelphia chromosome or theBCR-ABL1fusion gene. The present study reports the case of a 59-year-old Caucasian female that was referred to The Francisco Gentil Portuguese Institute of Oncology (Porto, Portugal) with constitutional symptoms (mainly asthenia), marked leukocytosis (51.33×109/l with 90% neutrophils), macrocytic anemia and splenomegaly. Bone marrow aspiration and biopsy revealed hypercellular marrow with clear predominance of segmented neutrophils. The karyotype was normal and theBCR-ABL1fusion gene was not detected. After excluding a leukemoid reaction, a diagnosis of CNL was established. The clinical follow-up was complicated by hemorrhagic brain lesions and relapsing episodes of erythematous, well-demarcated and painful subcutaneous nodular lesions, consistent with Sweet's syndrome (SS). Multiple treatment strategies were administered, including use of hydroxyurea, imatinib and intensive chemotherapy. Nevertheless, progression was documented and the patient succumbed at 28 months post-diagnosis. The clinical course of CNL varies, and can be complicated by cerebral hemorrhage, blastic transformation or infection. Dermatological manifestations such as SS have seldom been reported in association. No evidence-based treatment currently exists and the majority of our knowledge is based on results from case reports and small series.
authors.affiliation: Department of Oncohaematology, The Francisco Gentil Portuguese Institute of Oncology, Porto 4200-072, Portugal.
authors.forename: Patrícia Rocha
authors.initials: PR
authors.lastname: Silva
authors.affiliation: Department of Oncohaematology, The Francisco Gentil Portuguese Institute of Oncology, Porto 4200-072, Portugal.
authors.forename: Cristina
authors.initials: C
authors.lastname: Ferreira
authors.affiliation: Department of Genetics, The Francisco Gentil Portuguese Institute of Oncology, Porto 4200-072, Portugal.
authors.forename: Susana
authors.initials: S
authors.lastname: Bizarro
authors.affiliation: Department of Genetics, The Francisco Gentil Portuguese Institute of Oncology, Porto 4200-072, Portugal.
authors.forename: Nuno
authors.initials: N
authors.lastname: Cerveira
authors.affiliation: Department of Genetics, The Francisco Gentil Portuguese Institute of Oncology, Porto 4200-072, Portugal.
authors.forename: Lurdes
authors.initials: L
authors.lastname: Torres
authors.affiliation: Department of Oncohaematology, The Francisco Gentil Portuguese Institute of Oncology, Porto 4200-072, Portugal.
authors.forename: Ilídia
authors.initials: I
authors.lastname: Moreira
authors.affiliation: Department of Oncohaematology, The Francisco Gentil Portuguese Institute of Oncology, Porto 4200-072, Portugal.
authors.forename: José Mário
authors.initials: JM
authors.lastname: Mariz
keywords: Sweet's syndrome; atypical chronic myeloid leukaemia; chronic neutrophilic leukaemia; myeloproliferative disorders