Chronic neutrophil leukaemia in adolescence and young adulthood

Abstract

Chronic neutrophil leukaemia (CNL) is a rare myeloproliferative disorder predominantly reported in elderly patients. We present a 15-year-old girl and a 25-year-old male with CNL. Clonal cytogenetic abnormalities were detected in both patients. One showed trisomy 21 evolving into tetrasomy 21. The second patient showed a unique chromosome aberration during blast crisis: t(2;2)(q32;p24). Both patients were successfully treated with allogeneic bone marrow transplantation (BMT). CNL should also be considered as a differential diagnosis in adolescence and young adulthood. BMT represents a potentially curative treatment option in such patients.

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

“…Two cases each underwent SCT in stable chronic phase, in accelerated phase, or following treated blast transformation, respectively, with no details provided for the remaining case. The procedure was successful in five cases, with ongoing remission at the time of reporting of more than 78, 73, 54, 36, and 7 months post-transplantation [32,37,45,79]. Two patients who received transplants following blast transformation or in accelerated phase, died shortly after the procedure due to regimen related toxicity and relapse, respectively [5,57].…”

“…For blast phase, standard induction chemotherapy was successful at attaining a second chronic phase in one patient only [37]. In the remaining patients the leukemia was either refractory, the marrow remained hypoplastic following induction chemotherapy or death occurred during attempts at remission induction [5,32,38,41].…”

“…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].…”

“…In the earliest reported cases, splenic irradiation and splenectomy were used to reduce tumor bulk and relieve abdominal discomfort [78]. Splenectomy has resulted in worsening of neutrophilic leukocytosis and cannot be recommended [5,7,37]. Treatment of CNL has consisted largely of cytoreductive agents, most commonly oral hydroxyurea and to a much lesser extent, parenteral alpha interferon to control the myeloproliferation.…”

“…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].…”