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

Silva,Patrícia  Rocha; Ferreira,Cristina; Bizarro,Susana; Cerveira,Nuno; Torres,Lurdes; Moreira,Ilídia; Mariz,José  Mário

doi:10.3892/ol.2015.3148

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

Authors:
- Patrícia Rocha Silva
- Cristina Ferreira
- Susana Bizarro
- Nuno Cerveira
- Lurdes Torres
- Ilídia Moreira
- José Mário Mariz
View Affiliations

Affiliations: Department of Oncohaematology, The Francisco Gentil Portuguese Institute of Oncology, Porto 4200‑072, Portugal, Department of Genetics, The Francisco Gentil Portuguese Institute of Oncology, Porto 4200‑072, Portugal
Published online on: April 24, 2015 https://doi.org/10.3892/ol.2015.3148
Pages: 2657-2660

Metrics: Total Views: 0 (Spandidos Publications: | PMC Statistics: )

Metrics: Total PDF Downloads: 0 (Spandidos Publications: | PMC Statistics: )

Cited By (CrossRef): 0 citations Loading Articles...

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 (51.33x109/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.

View Figures

View References

1	Bain B, Brunning R and Vardiman J: Chronic neutrophilic leukaemiaIn: WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Swerdlow S, Campo E and Harris NL: IARC Press; France: pp. 38–39. 2008
2	Vardiman J and Hyjek E: World health organization classification, evaluation and genetics of the myeloproliferative neoplasm variants. Hematology Am Soc Hematol Educ Program. 2011:250–256. 2011. View Article : Google Scholar : PubMed/NCBI
3	Reilly JT: Chronic neutrophilic leukaemia: a distinct clinical entity? Br J Haematol. 116:10–18. 2002. View Article : Google Scholar : PubMed/NCBI
4	Bohm J and Schaefer HE: Chronic neutrophilic leukaemia: 14 new cases of an uncommon myeloproliferative disease. J Clin Pathol. 55:862–864. 2002. View Article : Google Scholar : PubMed/NCBI
5	List AF, Kopecky KJ, Qillman CL, et al: Benefit of cyclosporine modulation of drug resistance in patients with poor-risk acute myeloid leukemia: a Southwest Oncology Group study. Blood. 98:3212–3220. 2001. View Article : Google Scholar : PubMed/NCBI
6	Tuohy EL: A case of splenomegaly with polymorphonuclear neutrophil hyperleukocytosis. Am J Med Sci. 160:18–25. 1920. View Article : Google Scholar
7	Elliott MA: Chronic neutrophilic leukemia and chronic myelomonocytic leukemia: WHO defined. Best Pract Res Clin Haematol. 19:571–593. 2006. View Article : Google Scholar : PubMed/NCBI
8	Sakka V, Tsiodras S, Giamarellos-Bourboulis EJ and Giamarellou H: An update on the etiology and diagnostic evaluation of a leukemoid reaction. Eur J Intern Med. 17:394–398. 2006. View Article : Google Scholar : PubMed/NCBI
9	Melo JV: The diversity of BCR-ABL fusion proteins and their relationship to leukemia phenotype. Blood. 88:2375–2384. 1996.PubMed/NCBI
10	Pane F, Frigeri F, Sindona M, et al: Neutrophilic-chronic myeloid leukemia: a distinct disease with a specific molecular marker (BCR/ABL with C3/A2 junction). Blood. 88:2410–2414. 1996.PubMed/NCBI
11	Klampfl T, Gisslinger H, Harutyunyan AS, et al: Somatic mutations of calreticulin in myeloproliferative neoplasms. N Engl J Med. 369:2379–2390. 2013. View Article : Google Scholar : PubMed/NCBI
12	Nangalia J, Massie CE, Baxter EJ, et al: Somatic CALR mutations in myeloproliferative neoplasms with nonmutated JAK2. N Engl J Med. 369:2391–2405. 2013. View Article : Google Scholar : PubMed/NCBI
13	Ortiz-Cruz K, Amog-Jones G and Salvatore JR: Chronic neutrophilic leukemia with JAK2 gene mutation. Commun Oncol. 9:127–131. 2012. View Article : Google Scholar
14	Maxson JE, Gotlib J, Pollyea DA, et al: Oncogenic CSF3R mutations in chronic neutrophilic leukemia and atypical CML. N Engl J Med. 368:1781–1790. 2013. View Article : Google Scholar : PubMed/NCBI
15	Elliott MA and Tefferi A: Chronic neutrophilic leukemia 2014: Update on diagnosis, molecular genetics and management. Am J Hematol. 89:651–658. 2014. View Article : Google Scholar : PubMed/NCBI
16	Terré C, Garcia I, Bastie JN, et al: A case of chronic neutrophilic leukemia with deletion (11)(q23). Cancer Genet Cytogenet. 110:70–71. 1999. View Article : Google Scholar : PubMed/NCBI
17	Chen Y, Wang S and Wang W: Deletion of chromosome 17 as a novel cytogenetic finding in chronic neutrophilic leukemia: A case report. Oncol Lett. 5:1704–1706. 2013.PubMed/NCBI
18	Frank MB, Norwood TH and Willerford DM: Chimeric del20q in a case of chronic neutrophilic leukemia. Am J Hemat. 64:229–231. 2000. View Article : Google Scholar : PubMed/NCBI
19	Cohen PR: Sweet's syndrome - a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis. 2:342007. View Article : Google Scholar : PubMed/NCBI
20	Ratzinger G, Burgdorf W, Zelger BG and Zelger B: Acute febrile neutrophilic dermatosis: a histopathologic study of 31 cases with review of literature. Am J Dermatopathol. 29:125–133. 2007. View Article : Google Scholar : PubMed/NCBI
21	Gan GG, Pasagna JF, Eow GI and Nadarajan VS: Chronic neutrophilic leukaemia. Singapore Med J. 48:e74–e76. 2007.PubMed/NCBI
22	Castanet J, Lacour JP, Garnier G, et al: Neutrophilia dermatosis associated with chronic neutrophilia leukemia. J Am Acad Dermatol. 29:290–292. 1993. View Article : Google Scholar : PubMed/NCBI
23	Zittoun R, Réa D, Ngoc LH and Ramond S: Chronic neutrophilic leukemia. A study of four cases. Ann Hematol. 68:55–60. 1994. View Article : Google Scholar : PubMed/NCBI
24	Shigekiyo T, Miyagi J, Chohraku M, et al: Bleeding tendency in chronic neutrophilic leukemia. Int J Hematol. 88:240–242. 2008. View Article : Google Scholar : PubMed/NCBI
25	Kako S, Kanda Y, Sato T, et al: Early relapse of JAK2 V617F-positive chronic neutrophilic leukemia with central nervous system infiltration after unrelated bone marrow transplantation. Am J Hematol. 82:386–390. 2007. View Article : Google Scholar : PubMed/NCBI
26	Noguchi T, Ikeda K, Yamamoto K, et al: Severe bleeding tendency caused by leukemic infiltration and destruction of vascular walls in chronic neutrophilic leukemia. Int J Hematol. 74:437–441. 2001. View Article : Google Scholar : PubMed/NCBI
27	Elliott MA, Hanson CA, Dewald GW, Smoley SA, Lasho TL and Tefferi A: WHO-defined chronic neutrophilic leukemia: a long-term analysis of 12 cases and a critical review of the literature. Leukemia. 19:313–317. 2005. View Article : Google Scholar : PubMed/NCBI
28	Goto H, Hara T, Tsurumi H, Tanabashi S and Moriwaki H: Chronic neutrophilic leukemia with congenital Robertsonian translocation successfully treated with allogeneic bone marrow transplantation in a young man. Intern Med. 48:563–567. 2009. View Article : Google Scholar : PubMed/NCBI
29	Pardanani A and Tefferi A: Imatinib targets other than bcr/abl and their clinical relevance in myeloid disorders. Blood. 104:1931–1939. 2004. View Article : Google Scholar : PubMed/NCBI
30	Apperley JF, Gardembas M, Melo JV, et al: Response to imatinib mesylate in patients with chronic myeloproliferative diseases with rearrangements of the platelet-derived growth factor receptor beta. N Engl J Med. 347:481–487. 2002. View Article : Google Scholar : PubMed/NCBI
31	Choi IK, Kim BS, Lee KA, et al: Efficacy of imatinib mesylate (STI571) in chronic neutrophilic leukemia with t(15;19): case report. Am J Hematol. 77:366–369. 2004. View Article : Google Scholar : PubMed/NCBI
32	Elliott MA, Dewald GW, Tefferi A and Hanson CA: Chronic neutrophilic leukemia (CNL): a clinical, pathologic and cytogenetic study. Leukemia. 15:35–40. 2001. View Article : Google Scholar : PubMed/NCBI
33	Amato D, Memon S and Wang C: Myeloblastic transformation of chronic neutrophilic leukaemia. Br J Haematol. 142:1482008. View Article : Google Scholar : PubMed/NCBI