Persistent Eosinophilia: A Diagnostic Dilemma
Keywords:
Eosinophilia, chronic eosinophilic leukaemia, idiopathic hypereosinophilic syndrome
Abstract
Chronic eosinophilic leukaemia-not otherwise specified (CEL-NOS) is a myeloproliferative neoplasm associated with an autonomous, clonal proliferation of eosinophilic precursors resulting in persistent eosinophilia. A 50-year-old female presented with easy fatiguability, cough and generalised swelling of the body. Investigations revealed anaemia with leucocytosis (56.150 x 103/ul) and 88% eosinophils (absolute eosinophil count was 49412/ul). Peripheral smear showed abnormal eosinophils exhibiting abnormal granulation and nuclear lobation. Reactive causes were ruled out and a bone marrow aspiration/biopsy revealed mildly hypercellular marrow with increased number of eosinophils and their precursors, 7% blasts along with dysplastic megakaryocytes - hypolobated and occasional segmented forms. Molecular studies including chromosomal and gene analysis were done. A combination of the clinical picture, laboratory and molecular studies led us to a diagnosis of CEL-NOS. The causes for eosinophilia are myriad and range from reactive causes like parasitic infestations to neoplasms in which eosinophils are a part of the neoplastic population/ are cytokine-mediated reactive component in the background of another neoplasm. The incidence of CEL-NOS is obscure due to significant overlap with Idiopathic Hypereosinopihlic eosinophilia (IHES). While CEL-NOS is a myeloproliferative neoplasm and its diagnosis can be made provided evidence of a clonality is present, IHES is a diagnosis of exclusion. It is important to differentiate the two entities as they carry different prognosis and modes of treatment.References
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2. Bain B, Clark D, Wilkins B. Bone Marrow Pathology. Newark: John Wiley & Sons, Incorporated; 2019.
3. Tefferi A, Patnaik MM, Pardanani A. Eosinophilia: secondary, clonal and idiopathic. British journal of haematology. 2006 Jun;133(5):468-92.
4. Valent P, Klion AD, Horny HP, Roufosse F, Gotlib J, Weller PF et al. Contemporary consensus proposal on criteria and classification of eosinophilic disorders and related syndromes. Journal of Allergy and Clinical Immunology. 2012 Sep 1;130(3):607-12.
5. Brigden M, Graydon C. Eosinophilia detected by automated blood cell counting in ambulatory North American outpatients: incidence and clinical significance. Archives of pathology & laboratory medicine. 1997 Sep 1;121(9):963.
6. Gotlib J, Cools J, Malone JM, Schrier SL, Gilliland DG, Coutré SE. The FIP1L1-PDGFRα fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia: implications for diagnosis, classification, and management. Blood. 2004 Apr 15;103(8):2879-91.
7. Gleich GJ. Mechanisms of eosinophil-associated inflammation. Journal of Allergy and Clinical Immunology. 2000 Apr 1;105(4):651-63.
8. Klion AD, Law MA, Riemenschneider W, McMaster ML, Brown MR, Horne M et al. Familial eosinophilia: a benign disorder?. Blood. 2004 Jun 1;103(11):4050-5.
9. Tefferi A. Blood eosinophilia: a new paradigm in disease classification, diagnosis, and treatment. InMayo Clinic Proceedings 2005 Jan 1 (Vol. 80, No. 1, pp. 75-83). Elsevier.
10. Hujoel IA, Jaeger TM. 65-Year-Old Woman With Chronic Eosinophilia. InMayo Clinic Proceedings 2018 May 1 (Vol. 93, No. 5, pp. 646-650). Elsevier.
11. Tefferi A, Gotlib J, Pardanani A. Hypereosinophilic syndrome and clonal eosinophilia: point-of-care diagnostic algorithm and treatment update. InMayo Clinic Proceedings 2010 Feb 1 (Vol. 85, No. 2, pp. 158-164). Elsevier.
12. Kumar A, Sinha S, Tripathi AK. Chronic eosinophilic leukemia: a case report and review of literature. Indian Journal of Hematology and Blood Transfusion. 2007 Dec 1;23(3-4):112-5.
13. Bain BJ. Eosinophilic leukemia and idiopathic hypereosinophilic syndrome are mutually exclusive diagnoses. Blood. 2004 Dec 1;104(12):3836-7.
14. Wang SA, Hasserjian RP, Tam W, Tsai AG, Geyer JT, George TI et al. Bone marrow morphology is a strong discriminator between chronic eosinophilic leukemia, not otherwise specified and reactive idiopathic hypereosinophilic syndrome. haematologica. 2017 Aug 1;102(8):1352-60.
Published
2020-05-28
Issue
Section
Case Report
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