No abstract available.
Leukemia, Neutrophilic, Chronic*

No abstract available.
Leukemia, Neutrophilic, Chronic*

No abstract available.
Amyloidosis* ; Leukemia, Neutrophilic, Chronic* ; Splenic Rupture*

Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative neoplasm (MPN) characterized by persistent peripheral blood neutorphilia, bone marrow hypercellularity of neutrophilic granulocyte proliferation and hepatosplenomegaly. Acquired somatic mutation, JAK2 V617F, is the only molecular marker known to have association with classic BCR-ABL1 negative MPNs. However, JAK2 V617F has been detected occasionally in other MPNs such as CNL or other disease entities. We experienced a case of CNL with JAK2 V617F mutation. The patient was diagnosed according to the 2008 WHO classification criteria, and developed AML 9 months after the diagnosis of CNL. The JAK2 V617F was detected in the bone marrow throughout the clinical course. More cases are needed to establish the role of JAK2 V617F in the pathogenesis, prognosis and disease course of CNL.
Bone Marrow ; Granulocytes ; Humans ; Leukemia, Myeloid, Acute ; Leukemia, Neutrophilic, Chronic ; Neutrophils ; Prognosis

Cutaneous lesions of leukemia cutis (LC) by chronic neutrophilic leukemia (CNL) have been merely reported due to the rare occurrences of CNL. Furthermore cutaneous lesions in relation to clinical severity have been far less studied. A 70-year-old man presented with multiple violaceous papules and excoriations on both lower extremities. The diagnosis was LC based on histologic and laboratory evaluation and the origin was elaborated as CNL with the confirmation of colony stimulating factor 3 receptor (CSF3R) mutation. Interestingly, the patient presented clinical severity in a parallel manner to the hematologic abnormality. To the best of our knowledge, there has been no reported case of CSF3R confirmed LC in CNL featuring explicit skin eruption in relation to laboratory findings.
Aged ; Colony-Stimulating Factors ; Diagnosis ; Humans ; Leukemia ; Leukemia, Neutrophilic, Chronic ; Lower Extremity ; Skin

Chronic Neutrophilic Leukemia (CNL) is a rare myeloproliferative disorder characterized by a persistent increase of mature peripheral neutrophils, myeloid hyperplasia in bone marrow, hepatosplenomegaly, elevated neutrophil alkaline phosphatase (NAP) and absence of Philadelphia chromosome, with no evidence of infection or malignancy sufficient to mimic a leukemoid reaction. CNL has been associated with multiple myelomas in many reported cases, but transition of Polycythemia Vera (PV) to CNL is very rare. An 81-year-old female patient, who had undergone intermittent phlebotomy following the diagnosis of PV 8 years previously, was admitted to our hospital due to lower back pain. A physical examination showed a splenomegaly 2cm below the costal margin, with tenderness of the thoracic and lumbar spine area. A peripheral blood examination showed a WBC count of 91, 800/micro L (neutrophil 88%) with a rare immature form, hemoglobin of 9.1 g/dL and a platelet count of 1, 661, 000/micro L. Her NAP score was 58. The bone marrow examination showed 95% cellularity, with an M: E ratio of 10: 1, increased megakaryocytes with normal morphology and the absence of myelofibrosis. Chromosomal studies showed no Philadelphia chromosome. A radiological examination showed compression fractures of the vertebrae and spinal cord compression. No underlying disease causing a leukemoid reaction was detected. With iron replacement, the hemoglobin level failed to increase over 12 g/dL. Therefore, it was concluded to be a transition of PV to CNL. After administration of hydroxyurea and vertebroplasty, the symptom improved and the WBC count was sustained below 40, 000/micro L.
Aged ; Aged, 80 and over ; Female ; Humans ; Leukemia, Neutrophilic, Chronic/*etiology ; Polycythemia Vera/*complications

Humans ; Leukemia, Neutrophilic, Chronic/genetics* ; Multiple Myeloma/genetics* ; Mutation ; Receptors, Colony-Stimulating Factor/genetics* ; Signal Transduction

Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative disorder characterized by clonal proliferation of mature neutrophils, hepatosplenomegaly, elevated leukocyte alkaline phosphatase score (ALP score) and a negative Philadelphia chromosome. To date, approximately 150 cases have been reported in the literature, including some cases presenting with a 'leukemic' state reflected by a neutrophilic reaction. The term 'true' CNL, recently introduced by Reilly, highlights the need for more experience with CNL cases to improve the diagnostic criteria. In Korea, about 10 cases have been reported in the literature and some of those cases did not meet the WHO diagnostic criteria for CNL. We present a typical case of CNL in a 66-year-old man who complained of general weakness and weight loss. On admission, the white blood cell count from the peripheral blood was 175,600/L with 80% segmented neutrophils. The cytogenic study was negative for the Philadelphia chromosome and had a normal karyotype.
Aged ; Alkaline Phosphatase ; Humans ; Karyotype ; Korea ; Leukemia, Neutrophilic, Chronic* ; Leukocyte Count ; Leukocytes ; Myeloproliferative Disorders ; Neutrophils ; Philadelphia Chromosome ; Weight Loss

OBJECTIVETo review the implications for diagnosis, pathogenesis and potential for new therapeutic option for chronic neutrophilic leukemia (CNL).

DATA SOURCESData cited in this review were obtained mainly from PubMed and Medline from 1993 to 2013 and highly regarded older publications were also included. The terms "chronic neutrophilic leukemia" and "diagnosis" were used for the literature search.

STUDY SELECTIONWe identified, retrieved and reviewed the information on the clinical and laboratory features, the new genetic findings, prognosis and disease evolution and management of CNL.

RESULTSThe discovery of high-frequency granulocyte-colony stimulating factor receptor (CSF3R) mutations in CNL identifies a new major diagnostic criterion, and lends more specificity to the World Health Organization (WHO) diagnostic criteria for CNL, which are variably applied in routine clinical practice.

CONCLUSIONSIn patients for whom the cause of neutrophilia is not easily discerned, the incorporation of CSF3R mutation testing can be a useful point-of-care diagnostic to evaluate the presence of a clonal myeloid disorder, as well as providing the potential for genetically informed therapy. The oncogenic CSF3R mutations are molecular markers of sensitivity to inhibitors of the SRC family-TNK2 and JAK kinases and may provide a new avenue for therapy.

Carrier Proteins ; genetics ; Female ; Humans ; Leukemia, Neutrophilic, Chronic ; diagnosis ; genetics ; Male ; Mutation ; Nuclear Proteins ; genetics ; Receptors, Colony-Stimulating Factor ; genetics

OBJECTIVETo observe the CSF3R, ASXL1, SETBP1, JAK2 V617F and CALR mutations in patients with chronic neutrophilic leukemia (CNL).

METHODSTwelve suspected "CNL" patients were retrospectively reviewed according the WHO criteria (2008). CSF3R,ASXL1,SETBP1 and CALR mutations were sequenced, and JAK2 V617F was tested by allele specific (AS)-PCR.

RESULTS6 of 12 cases were diagnosed as CML, and all of the 6 carried. 4 of 6 patients also had ASXL1 and SETBP1 mutations and one had a CALR mutation (c.1154-1155insTTGTC). Two patients with monoclonal gammopathy with uncertain significance (MGUS) combined with CNL-like symptoms had no CSF3R, ASXL1, SETBP1, JAK2 V617F or CALR mutation. The same results were also seen in other 4 cases with secondary neutrophilic leukocytosis.

CONCLUSIONCSF3R, ASXL1 and SETBP1 mutations differential diagnosis of CNL, and should be included in the diagnostic protocol so as to improve diagnostic accuracy for CNL.

Carrier Proteins ; Humans ; Janus Kinase 2 ; Leukemia, Neutrophilic, Chronic ; Mutation ; Nuclear Proteins ; Polymerase Chain Reaction ; Receptors, Colony-Stimulating Factor ; Repressor Proteins ; Retrospective Studies