A Case of Chronic Neutrophilic Leukemia with Deletion (11)(q23)

Terré, Christine; Garcia, Isabelle; Bastie, Jean-Noël; Mayeur, Didier; Decombe, L; Gruyer, Ph; Berger, Roland; Castaigné, Sylvie

doi:10.1016/s0165-4608(98)00177-0

Cited by 15 publications

(10 citation statements)

References 6 publications

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“…At that time, only 150 cases had been reported in the literature [29,31,32]. A critical review of the literature applying the then recently developed WHO diagnostic criteria, could only confirm this diagnosis in 40 cases, indicating the true rate of occurrence of what is now considered CNL was even lower [5,[32][33][34][35][36][37][38][39][40][41][42][43][44][45][46][47]. In this data set of WHO-defined CNL, the median age at diagnosis was 66 years (range: 15 -86) and 56% were males [5].…”

Section: Epidemiology and Demographicsmentioning

confidence: 99%

“…Despite this, relatively few cases have been reported in the literature, reflecting the rarity of this disease and the older age of many affected. As such most of the information regarding this procedure is derived from single case reports and seven such cases, performed at ages 15,23,26,40,49,49, and 60 years, have been reported [5,32,37,45,57,79]. All patients received myeloablative conditioning (cyclophosphamide and total body irradiation), most had sibling donors, although two more recent cases received matched unrelated transplants [57,79].…”

Section: Hematopoietic Stem Cell Transplantationmentioning

confidence: 99%

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Chronic neutrophilic leukemia 2016: Update on diagnosis, molecular genetics, prognosis, and management

Elliott

Tefferi

2016

American J Hematol

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Chronic neutrophilic leukemia (CNL) is a potentially aggressive myeloproliferative neoplasm, for which current WHO diagnostic criteria include leukocytosis of ≥25 × 109/L (of which >80% are neutrophils) and with <10 and <1% circulating immature granulocytes and blasts, respectively without dysplasia, clinical, or molecular criteria for other myeloproliferative disorders, nor an identifiable cause for physiologic neutrophilia in the absence of markers of myeloid clonality. Such a pathogenic clonal marker has now been identified as a somatic activating mutation of CSF3R, most commonly CSF3R T618I, thus demanding revision of the current WHO diagnostic classification to include the molecular criterion of mutated CSF3R. The clinical presentation, disease course and prognosis of CSF‐R mutated CNL have been recently outlined. Co‐operative mutations in SETBP1 and ASXL1 appear to be of prognostic significance and correlate with disease progression. Advances in the understanding of the molecular pathogenesis of CNL, have not yet fully translated into satisfactory therapeutic strategies, but the foundations for these are strengthening. Am. J. Hematol. 91:342–349, 2016. © 2015 Wiley Periodicals, Inc.

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Section: Epidemiology and Demographicsmentioning

confidence: 99%

Section: Hematopoietic Stem Cell Transplantationmentioning

confidence: 99%

Chronic neutrophilic leukemia 2016: Update on diagnosis, molecular genetics, prognosis, and management

Elliott

Tefferi

2016

American J Hematol

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“…Unfortunately, platelet function was not evaluated in any of the 40 patients [9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25]. Qualitative platelet abnormalities are commonly found in patients with myeloproliferative disorders (MPDs) such as chronic myelogenous leukemia, myeloid metaplasia and myelofibrosis, polycythemia vera, and essential thrombocythemia [26,27].…”

mentioning

confidence: 99%

Bleeding tendency in chronic neutrophilic leukemia

et al. 2008

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“…Recently, Maxson et al reported the association between receptor for colony-stimulating factor 3 (CSF3R) mutations and CNL and atypical CML (14), which could be a promising marker of the disease (15). In the present case, the karyotype was normal, but nearly 10% of CNL cases are associated with cytogenetic abnormalities, including trisomy 8, 9, 21 and deletion of 11(q), 12(p), 17(p) and 20(q), the latter possibly being associated with a more favorable prognosis (16)(17)(18).…”

Section: Discussionmentioning

confidence: 51%

Diagnosis, complications and management of chronic neutrophilic leukaemia: A case report

et al. 2015

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Abstract. Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative neoplasm characterized by sustained neutrophilia and the absence of the Philadelphia chromosome or the BCR-ABL1 fusion 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 (51.33x10 9 /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 the BCR-ABL1 fusion 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.

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A Case of Chronic Neutrophilic Leukemia with Deletion (11)(q23)

Cited by 15 publications

References 6 publications

Chronic neutrophilic leukemia 2016: Update on diagnosis, molecular genetics, prognosis, and management

Chronic neutrophilic leukemia 2016: Update on diagnosis, molecular genetics, prognosis, and management

Bleeding tendency in chronic neutrophilic leukemia

Diagnosis, complications and management of chronic neutrophilic leukaemia: A case report

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