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Year : 2011  |  Volume : 54  |  Issue : 3  |  Page : 648-649
Persistent neutrophilia progressing to polycythemia vera


Department of Pathology, Lady Hardinge Medical College and Associated Smt. Sucheta Kriplani Hospital, New Delhi - 110 001, India

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Date of Web Publication20-Sep-2011
 

How to cite this article:
Singh S, Sharma S, Tejwani N. Persistent neutrophilia progressing to polycythemia vera. Indian J Pathol Microbiol 2011;54:648-9

How to cite this URL:
Singh S, Sharma S, Tejwani N. Persistent neutrophilia progressing to polycythemia vera. Indian J Pathol Microbiol [serial online] 2011 [cited 2020 Dec 2];54:648-9. Available from: https://www.ijpmonline.org/text.asp?2011/54/3/648/85134


Sir,

Myeloproliferative neoplasms (MPN) are clonal hematopoitic stem cell disorders that can transform to acute leukemia or into each other. However, chronic neutrophilic leukemia (CNL) progressing to polycythemia vera (PV) has not been reported in the literature yet.

A 48-year-old male presented in the Medicine Outpatient Department in the year 2007 with complaints of dragging pain in the left hypochondrium for 1 year. There was no history of fever. On clinical examination, massive splenomegaly measuring 15 cm below the costal margin was found. Hemogram revealed hemoglobin of 14.5 g/dl with normal red cell indices, platelet count of 300 x 103 /μl and neutrophilic leukocytosis [total leukocyte count (TLC) of 25 x 10 3 /μl] with absolute neutrophil count of 22 x 10 3 /μl. The neutrophil alkaline phosphatase score was found to be raised ([Figure 1] inset). Iron studies were within normal limits. Although there were no signs of infection, he was started on broad-spectrum antibiotics and advised bone marrow aspiration with close follow-up. The patient did not turn up for follow-up for the next 2 years. However, after 2 years, he again presented with similar complaints. His hemogram at this time revealed persistent neutrophilia (TLC = 28 x 10 3 /μl and absolute neutrophil count = 24.5 x 10 3 /μl) with mild shift to the left [Figure 1], hemoglobin levels of 17.1 g/dl and platelet count 534 x 103 / μl. Bone marrow aspirate smears showed trilineage hyperplasia [Figure 2] with the myeloid: erythroid ratio being 3.6. Granulocyte series showed normal maturation. Megakaryocytes were increased in number with normal morphology. Iron stores were absent. The neutrophil alkaline phosphatase score was 272 (control = 92). Cytogenetic studies showed presence of JAK2-V617F mutation (homozygous) and absence of BCR-ABL gene rearrangement. Based on the above findings, a final diagnosis of PV with possible transformation from CNL was given.
Figure 1: Neutrophilic leucocytosis (Giemsa stain, ×400) with high neutrophil alkaline phosphatase score (inset, ×400)

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Figure 2: Bone marrow aspirate showing trilineage hyperplasia (Giemsa stain, ×400)

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The World Health Organization has recently acknowledged CNL to be a distinct MPN, with a recommendation that all other secondary causes mimicking CNL (reactive and other myeloproliferative disorders) should be excluded. [1] In rare conditions, it can transform to acute leukemia [2] and other MPN or can be associated with multiple myeloma. [3] However, CNL progressing into PV has not been reported in the literature, as could be the possibility in the present case.

 
   References Top

1.Bain BJ, Brunning RD, Vardiman JW, Thiele J. Chronic neutrophilic leukemia. In: Steven H, Swerdlow, Elias C, Nancy LH, Elaine SJ, Stefano AP, et al ., editor. WHO classification of tumours of haematopoietic and lymphoid tissues, 4 th ed. Lyon, France: IARC; 2008. p. 38-389.  Back to cited text no. 1
    
2.Katsuki K, Shinohara K, Takeda K, Ariyoshi K, Yamada T, Kameda N, et al. Chronic neutrophilic leukemia with acute myeloblastic transformation. Jpn J Clin Oncol 2000;30:362-5.  Back to cited text no. 2
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3.Lugassy G, Farhi R. Chronic neutrophilic leukemia associated with polycythemia vera. Am J Hematol 1990;35:139-40.  Back to cited text no. 3
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Correspondence Address:
Narender Tejwani
Department of Pathology, Lady Hardinge Medical College and Associated Smt. Sucheta Kriplani Hospital, New Delhi - 110 001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.85134

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  [Figure 1], [Figure 2]

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[Pubmed] | [DOI]



 

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