Neoplastic involvement of granulocytic lineage, not granulocytic-monocytic, monocytic, or erythrocytic lineage, in a patient with chronic neutrophilic leukemia case report

Abstract

Chronic neutrophilic leukemia (CNL) is a very rare myeloproliferative disorder. To determine the neoplastic origin of CNL, morphological and cytogenetical studies were made of colonies derived from hematopoietic progenitors of a patient with CNL. The patient's hematopoietic progenitors spontaneously formed colonies consisting of mature granulocytes, and cytogenetical study of the colonies indicated chromosome abnormalities identical to those in the patient's bone marrow cells. Analysis of colonies consisting of granulocytes and macrophages, macrophages, or erythrocytes disclosed a normal karyotype. These results demonstrated that the neoplastic process in this patient with CNL originated in hematopoietic progenitors capable of differentiating only into granulocytes, and not granulocytes and monocytes, monocytes, or erythrocytes.

Chronic neutrophilic leukemia (CNL): a clinical, pathologic and cytogenetic study

“…Molecular studies for the BCR/ABL rearrangement, which must be negative before this diagnosis can be made, have been performed only in cases described since 1992, accounting for fewer than 10 reported cases. [5][6][7][8][9][10][11][12] This study is the largest reported series of wellcharacterized patients with CNL and allows a more detailed description and understanding of this disease.…”

“…6,7,9 Most well-documented cases of CNL have normal cytogenetics. Among cases with cytogenetic abnormalities, chromosome anomalies are heterogeneous, and no consistent abnormality has been associated with CNL.…”

“…Reported isolated abnormalities have included trisomy 8, trisomy 9 with deletion of the long arm of chromosome 20 (20q−), isolated 20q−, deletion of part of the long arm of chromosome 11 with breakpoints at 11q14, trisomy 21, and complex karyotypes. 7,[9][10][11]18 Kwong and Cheng 6 reported a case with clinical and hematologic features consistent with CNL, with normal cytogenetics and absence of the BCR rearrangement. The clonal nature of the myeloid proliferation was demonstrated by the methylation pattern of the X-linked hypoxanthine phosphoribosyl transferase (HPRT) gene.…”

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

“…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].…”

Pathogenetic insight and prognostic information from standard and molecular cytogenetic studies in the BCR-ABL-negative myeloproliferative neoplasms (MPNs)

“…Indeed, it has been suggested that the chromosomal changes identified to date may reflect cytogenetic evolution and be secondary events in the pathogenesis of the disease. 64 Finally, cytogenetics studies have indicated that the origin of the neoplastic clone in CNL may be at the committed stem cell level, 68 although this finding has not been supported by X-linked clonality studies. 69 Specific chromosomal abnormalities…”