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1/5. A case of chronic neutrophilic leukemia with trisomy 8.

    The authors describe a case of chronic neutrophilic leukemia (CNL) showing trisomy of chromosome 8. This anomaly, particularly common in cases of myelodysplastic syndrome (MDS), has never been previously found to be associated with this rare type of leukemia. Thus CNL may occasionally be cytogenetically indistinguishable from cases of MDS.
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ranking = 1
keywords = myelodysplastic syndrome, myelodysplastic
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2/5. Chronic neutrophilic leukemia with dysplastic features. A new variant of the myelodysplastic syndromes.

    We are reporting four patients who presented with a persistent increase of mature neutrophils and band forms, no monocytosis in repeated examinations and with dysplastic features in blood and bone marrow. Two of them developed acute myeloid leukemia (AML; FAB classification M1) and the other two were characterised by a progressively aggravating myelodysplasia. All the patients showed a poor response to treatment and died within a period of time ranging from 14 to 80 months after diagnosis. We are proposing the term chronic neutrophilic leukemia with dysplastic features (CNL-D) for this entity, and we believe that it represents a variant of the myelodysplastic syndromes.
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ranking = 5
keywords = myelodysplastic syndrome, myelodysplastic
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3/5. Chronic neutrophilic leukemia. A study of four cases.

    Chronic neutrophilic leukemia (CNL) is a very rare entity, which has to be included among the chronic myeloid leukemias. Once an underlying cause of neutrophilia is excluded, the diagnosis of CNL is based on exclusion of chronic granulocytic and other types of chronic myeloid leukemias. The classification proposed by Sheperd et al. has proven to be helpful, but it must be completed by cytogenetic analysis and the search for bcr rearrangement by molecular biology methods, in order to confirm the absence of philadelphia chromosome and of bcr-abl hybrid gene. We report here four cases of CNL, with confirmed absence of bcr rearrangement in two cases. Two patients died, 12 and 8 years after diagnosis, the second one following transformation into myelofibrosis with myeloid metaplasia. The other two died of acute myelogenous leukemia, the first one, 25 years after diagnosis of CNL, following a 3-year phase of acceleration. The last patient presented combined features of CNL and refractory anemia with excess of blasts, and was characterized by both progressive leukocytosis and severe thrombocytopenia; acute transformation into acute myelogenous leukemia occurred 6 months after diagnosis and death 1 month later. Among the 30 cases reported so far, plus the four presented here, combined myelodysplastic features were observed in five cases and transformation into acute myelogenous leukemia in six. Chronic neutrophilic leukemias should be reported regularity, in view of the uncertain and low frequency of this hematological disease.
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ranking = 0.30447367434506
keywords = myelodysplastic
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4/5. Chronic neutrophilic leukemia with dysplastic features mimicking myelodysplastic syndromes.

    A 52-year-old male patient with chronic neutrophilic leukemia (CNL) with dysplastic features is described. He had a 2-year history of anemia followed by marked leukocytosis up to 57.5 x 10(9)/l with 88% segmented neutrophils. bone marrow aspiration and biopsy showed hypercellular marrow with myeloid hyperplasia and 16% myeloblasts. There were also significant morphological abnormalities which included neutrophils with few granules, hypersegmented nucleus or Pelger-Heut anomaly, and micromegakaryocytes. cytogenetic analysis disclosed a deletion of the long arm of chromosome 7 (7q-). He was diagnosed as having CNL with dysplastic features and was treated conservatively. However, leukemic transformation to acute myelogenous leukemia occurred within a year and he died 16 months after diagnosis. Neutrophilia is a feature not of myelodysplastic syndromes (MDS) but rather of myeloproliferative disorders such as CNL. However, this patient was considered to have MDS with increased proliferation and differentiation of neutrophilic lineage, because of marked myelodysplasia and poor prognosis.
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ranking = 2.2178946973802
keywords = myelodysplastic syndrome, myelodysplastic
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5/5. Chronic neutrophilic leukemia evolving from a myelodysplastic syndrome.

    Chronic neutrophilic leukemia (CNL) is a rare hematologic disorder usually presenting with a persistent neutrophilia in the leukemoid range (WBC > 40-50 x 10(9)/1) and consisting largely of mature neutrophils. patients have no obvious cause for an elevated white count and typically have an elevated leukocyte alkaline phosphatase score, hepatosplenomegaly, elevated vitamin B12 and are philadelphia chromosome-negative. CNL has occasionally been associated with paraproteinemia or outright myeloma. Dysplastic features within the neutrophils in CNL have rarely been reported. We report the clinical, pathological and cytogenetic features of a case of CNL in an elderly white female initially diagnosed with refractory anemia with excess blasts, which subsequently progressed to CNL.
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ranking = 4
keywords = myelodysplastic syndrome, myelodysplastic
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