Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
 ...
PMID:A case of chronic neutrophilic leukemia with trisomy 8. 249 62

Chronic neutrophilic leukemia (CNL) is a rare type of leukemia. We diagnosed a 81-year-old woman as CNL because she showed that sustained leukocytosis dominated by mature neutrophils, hepatosplenomegaly, high neutrophilic alkaline phosphatase (NAP) score, absence of the Ph1 chromosome and no evidence of leukemoid reaction. During the clinical course, she did not manifest hemorrhagic tendency or infection. We also examined neutrophilic function including chemotaxis, chemiluminescence, nitroblue tetrazolium (NBT) dye reduction, which all indicated normal neutrophil function. Using a reversed phase-high pressure liquid chromatography (HPLC), we detected the production of leukotriene B4 (LTB4) in neutrophils. We found that the LTB4 production was decreased in neutrophils whereas they showed normal chemotaxis. This discrepancy has never, to our knowledge, been reported before in case of CNL.
 ...
PMID:The discrepancy between chemotaxis and leukotriene B4 production in a patient with chronic neutrophilic leukemia. 256 Dec 96

The supernatants of dispase-treated platelets from normal persons and patients with myeloproliferative disorders were analysed by capillary isotachophoresis. The examined myeloproliferative disorders were three cases of chronic myelogeneous leukemia, one case of chronic neutrophilic leukemia, one case of essential thrombocythemia and one case of polycythemia vera. An additional peak was revealed in the samples from the patients with chronic myelogeneous leukemia. Platelets from the other three patients showed no additional bands. Isotachophoresis will be useful not only to analyse the superficial peptide on the cell membrane but also to define the peptide associated with malignant transformation.
 ...
PMID:Application of analytical isotachophoresis in the study of platelet membrane proteins in patients with chronic myeloproliferative disorders. 276 Jan 57

Chronic myeloid leukaemia (CML) includes five subtypes, and the term should be used in the same way as the term chronic lymphoid leukaemia to refer to a group of related conditions. The subtypes of CML are: 1. Chronic granulocytic leukaemia (CGL) (95% of all CML; 90% are Ph+, BCR+, 5% are Ph-, BCR+); 2. Juvenile CML (extremely rare; Ph-, BCR- in the few so far examined); 3. Chronic neutrophilic leukaemia (CNL) (extremely rare; Ph-, BCR- in the few so far examined); 4. Chronic myelomonocytic leukaemia (CMML). CMML with low or normal leukocyte counts is classified as a myelodysplastic syndrome; CMML with high leukocyte count is both myelodysplastic and myeloproliferative. Ph-, BCR-; 5. Atypical CML (aCML). Intermediate between CGL and CMML but has distinctive features. Ph-, mostly BCR-. Significance of few reported BCR+ uncertain. Markedly worse survival than CGL and probably worse than CMML. Definition needs refining. Types 2, 3, 4 and 5 account for 5% of all CML. CGL, CMML, aCML and CNL can be diagnosed in the great majority of cases from the morphological profile of presentation peripheral blood films, but high-quality Romanowsky staining is essential.
 ...
PMID:Haematological classification of the chronic myeloid leukaemias. 333 55

A patient with fairly typical chronic neutrophilic leukemia, as represented by some two dozen such reported cases, had been given Thorotrast more than 20 years before. Typical myeloblastic crisis developed with remarkable terminal leukocytosis. Mature blood neutrophils had normal function with respect to phagocytosis, bacterial killing, metabolic activation, and chemotactic response. The number of cells producing colonies of neutrophils and monocytes in in vitro semisolid cultures was normal in the blood and increased in marrow. Colony size was smaller than is usually observed in normal patients or in typical patients with chronic myeloid leukemia. Termination in blast crisis, also seen in a few other patients with chronic neutrophilic leukemia, indicates that this is indeed a form of leukemia and not a "leukemoid" reaction of obscure cause. The differential diagnosis of extreme neutrophilia is discussed.
 ...
PMID:Cytobiologic and clinical aspects in a patient with chronic neutrophilic leukemia after thorotrast exposure. 346 38

A case of chronic neutrophilic leukemia associated with multiple myeloma is described. The patient appears to be the longest surviving case reported in the literature. Since myeloma developed several years after leukemia, the possible precancerous role of myeloproliferative syndromes is proposed.
 ...
PMID:Chronic neutrophilic leukemia and myeloma. Report on long survival. 658 81

The membrane-proximal cytoplasmic region of the granulocyte colony-stimulating factor receptor (G-CSFR) is known to be essential for the proliferation signal, with a more distal region being required for the differentiation signal. Such a separation of functional domains raises the possibility that mutations occurring at these regions may contribute to cell proliferation in the absence of differentiation, this being the most important characteristic in acute leukemia cells. Therefore, we analysed the structural abnormalities at the transmembrane and cytoplasmic region of G-CSFR in a significant number of patients with various myeloid malignancies. When we examined the genomic DNA of G-CSFR obtained from 41 patients with acute myelogenous leukemia (AML), 18 with chronic myelogenous leukemia (CML), 7 with myelodysplastic syndrome (MDS), 2 with chronic myelomonocytic leukemia and 1 with chronic neutrophilic leukemia, we found a polymorphism in 3 patients, but no significant pathogenic mutations in any patients. The screening for this polymorphism in 100 hematologically normal controls revealed that it may be useful as a linkage marker for population and family studies, because the heterozygosity index is at a high level (0.055). While there have been several reports discussing the leukemogenic potential of mutations in the cytokine/hematopoietin receptor superfamily, genetic alterations in the transmembrane and cytoplasmic region of G-CSFR do not seem to play a pathogenic role in leukemia.
 ...
PMID:Analysis of the granulocyte colony-stimulating factor receptor gene structure using PCR-SSCP in myeloid leukemia and myelodysplastic syndrome. 954 19

A patient previously diagnosed with chronic neutrophilic leukemia (CNL) was studied using fluorescent in situ hybridization (FISH) to determine clonality of neutrophils. By cytogenetic studies the patient's blood and bone marrow had an 11q14 deletion and were negative for the Philadelphia (Ph) chromosome. FISH was performed on peripheral blood smears using probes for the bcr/abl translocation and a probe for 11q23 (MLL). The patient's white blood cells were negative for the bcr/abl translocation; neutrophils and eosinophils, but not lymphocytes, were monosomic for the 11q23 probe indicating a clonal population within the neutrophil population.
Leukemia 1998 Apr
PMID:Demonstration of clonality in neutrophils using FISH in a case of chronic neutrophilic leukemia. 955 23

Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative disorder. Less than 50 cases have been reported. We report the first case of CNL with an associated leukemia cutis. CNL was diagnosed in a 74-year-old white woman in 1998, based on neutrophilic infiltration of the bone marrow and absence of the Philadelphia chromosome. The patient presented to the dermatology service in August 1998 with a 2-week history of a pruritic eruption on the arms, hands, and legs. Physical examination revealed red to violaceous plaques on both thighs and knees, in addition to purpuric patches and plaques on the dorsal hands, arms, and legs. Leukemia cutis was demonstrated on biopsy specimens of several lesional sites. The eruption progressed, despite treatment with topical and systemic corticosteroids. Treatment with systemic chemotherapy did affect partial resolution of the eruption, with parallel decreases in bone pain and white blood cell count, but the disease progressed and the patient ultimately died 5 months after her initial skin findings. Only one other case of CNL with dermatologic manifestations has been reported, CNL associated with a reactional neutrophilic dermatosis. Comparison to and differentiation from this case is discussed. The importance of distinguishing the specific infiltrates of leukemia from the nonspecific infiltrates of reactional dermatoses, such as Sweet's syndrome, is illustrated.
 ...
PMID:Leukemia cutis in a patient with chronic neutrophilic leukemia. 1117 17

This report describes a single institution's recent experience with six patients fulfilling the diagnostic criteria of chronic neutrophilic leukemia. No patient had the Philadelphia chromosome or the BCR/ABL fusion gene. None of the common cytogenetic abnormalities characteristic of myeloid disorders were detected. Two patients demonstrated clonal evolution during the course of the disease. All responded initially to therapy with hydroxyurea with control of leukocytosis and reduction in splenomegaly. Three patients eventually became refractory to hydroxyurea, manifesting progressive neutrophilia without blastic transformation. Aggressive chemotherapy to control progressive leukocytosis resulted in death due to cytopenias in two of these patients. The third patient received less intensive chemotherapy and died of progressive disease. One patient died after transformation of the disease into undifferentiated acute myeloid leukemia. Two patients remain alive with stable disease on hydroxyurea therapy, 12 and 54 months after initial diagnosis. Chronic neutrophilic leukemia is a rare clinicopathologic entity that can be distinguished from chronic myelogenous leukemia, the recently described neutrophilic-chronic myelogenous leukemia, and myelodysplastic syndrome. The clinical course is heterogeneous, with a definite risk of death from either blastic transformation or progressive neutrophilic leukocytosis. Continued study and reporting of these cases must be encouraged.
Leukemia 2001 Jan
PMID:Chronic neutrophilic leukemia (CNL): a clinical, pathologic and cytogenetic study. 1124 96


1 2 3 4 Next >>