Short communicationsA Case of Chronic Neutrophilic Leukemia with Deletion (11)(q23)
Introduction
In February 1995, a 68-year-old man was referred to our hospital for anemia. He had a past history of 38 years of professional exposure to benzene and trichlorethylene in a printing office. His blood cell count showed a regenerative anemia with 11.8 g/dL of hemoglobin and 288 × 109/L reticulocytes, with a slight elevation of the platelet count and a normal white blood cell (WBC) count with a normal differential. There was no evidence of a hemorrhagic syndrome. Ferritin level was normal. Coombs test was negative. During the following year, modifications of his blood cell counts were observed with an increased neutrophilic leucocytosis (Table 1). There were no signs or symptoms of infection. Bone marrow aspiration revealed a hypercellular marrow. Treatment with hydroxyurea was started in January 1996. Despite this treatment, the evolution was characterized by a rapid increase of leucocytosis, up to 344 × 109/L with 87% polymorphonuclear (PMN). The liver and spleen were enlarged with cholestasis. Treatment with intermediate doses of cytosine arabinoside (1g/m2/12 hr for 2 d) led to a transient decrease of leucocytosis, but with worsening of the thrombocytopenia with intestinal bleeding. His general condition worsened rapidly and the patient died in August 1996. Cytogenetic analyses were performed twice, in October 1995 and January 1996, and showed the same results. Karyotype was 46,XY,del(11)(q23). Chromosome 11 painting showed no translocation involving chromosome 11. Only one spot was present in morphologically well identified granulocyte nuclei. Hybridization with Yac 742F9 encompassing the MLL locus confirmed this deletion. No rearrangement of the MLL gene was found by Southern blot analysis. Reverse transcriptase polymerase chain reaction (RT-PCR) analysis with primers specific to the M-BCR and m-BCR (b2a2 or b3a2 junction) and with primers specific to the μ-BCR (c3a2 junction) did not reveal any fusion of BCR-ABL.
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Results and discussion
Our patient fulfills the diagnosis criteria for chronic neutrophilic leukemia (CNL). He presented with chronic neutrophilic leucocytosis without an excess of immature myeloid cell, monocytosis, myeloid hyperplasia, hepatomegaly and splenomegaly, and absence of a Philadelphia chromosome [1]. Using these criteria, CNL appears to be a very rare chronic myeloproliferative disorder that generally affects patients more than 50 years of age, with a slowly progressive fatal course. Recently, Pane et
Acknowledgements
We thank J.M. Cayuela for PCR analysis for the detection of BCR-ABL rearrangement.
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