Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: EC:3.1.3.1 (alkaline phosphatase)
47,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chronic neutrophilic leukemia is a rare, infrequently recognized, myeloproliferative disorder. It usually manifests as a leukemoid reaction, with mostly mature granulocytes in the peripheral blood, with rare to occasional immature forms, and sometimes with normoblasts. The clinical manifestations also include hepatosplenomegaly, elevated leukocytic alkaline phosphatase, elevated serum vitamin B12 and serum vitamin B12 binder ("R" fraction), and elevated serum uric acid. Distinction from a leukemegaly, the absence of sepsis, usually normal erythrocytic sedimentation, and the absence of fever. Leukemoid reactions may be associated with elevated serum vitamin B12 and uric acid, but the levels are usually lower than those found in chronic neutrophilic leukemia. Many patients have gouty symptoms, especially after treatment with Busulfan, and many have an unexplained hemorrhagic tendency, making major operations a risk. The authors add two cases to the 11 previously described.
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PMID:Chronic neutrophilic leukemia. Report of two cases and review of the literature. 28 88

Two patients with chronic neutrophilic leukemia, a rare myeloproliferative syndrome, are reported with a review of the literature. The major features of the 34 collected cases (including the two patients reported here) were persistent leukocytosis simulating a leukemoid reaction, hepatosplenomegaly, hyperuricemia, increased vitamin B12 blood level, increased leukocyte alkaline phosphatase and absence of the Philadelphia chromosome. Infection was the leading cause of death. Concomitant multiple myeloma was found in eight patients.
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PMID:[Chronic neutrophilic leukemia: apropos of 2 cases and review of the literature]. 208 13

Reactive leukocytosis has been reported in patients with non-Hodgkin's lymphoma of different histologic types. On the other hand, the blastic crisis of chronic myelocytic leukemia (CML) can sometimes be localized outside the bone marrow and simulate lymphoma, particularly when the blasts are of lymphoid lineage and the blastic crisis is the presenting feature of the disease. We report two patients in whom the differential diagnosis between lymphoblastic lymphoma with reactive leukocytosis and blastic crisis of CML outside the bone marrow was raised. They were two males aged 32 and 22 years, respectively, with lymphadenopathy (and one with splenomegaly), who were initially diagnosed of T lymphoblastic lymphoma. In both cases, leukocytosis was detected with myelemia and dysgranulopoiesis in the onset in one of them and when lymphadenopathy reappeared after remission in the other one. In addition, one patient had marked eosinophilia. In the bone marrow there was marked granulopoietic hyperplasia, with a reduction of fatty cells, and the granulocyte alkaline phosphatase index was reduced. However, the cytogenetic study did not disclose the existence of Philadelphia (Ph) chromosome, and bcr/abl molecular rearrangement was also not observed in the molecular study of both cases. We discuss the basic aspects of differential diagnosis between T lymphoblastic lymphoma with leukemoid reaction and T lymphoid lymphadenopathic blastic crisis of Ph-negative, bcr/abl-negative CML.
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PMID:[T lymphoblastic leukemia with leukemoid reaction or the extramedullary blast crisis of Philadelphia chromosome-negative chronic myeloid leukemia? Comments apropos 2 cases]. 209 54

A male patient, aged 78, was found with chronic neutrophilic leukemia (CNL). The patient showed sustained mature neutrophilic leukocytosis, splenomegaly, a high leukocyte alkaline phosphatase score, elevated serum vitamin B12 and uric acid, myeloid hyperplasia and absence of ph' chromosome in the bone marrow, with no evidence suggesting this condition to be a leukemoid reaction to an underlying disease. In addition to the above mentioned features, some functional characteristics of CNL cells were compared with normal cells. CNL is a very rare disease; some thought it as a variant of chronic myelogenous leukemia (CML). In this report a review of the literature is also included.
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PMID:Chronic neutrophilic leukemia--report of a case and review of the literature. 217 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.
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PMID:The discrepancy between chemotaxis and leukotriene B4 production in a patient with chronic neutrophilic leukemia. 256 Dec 96

A case of lepromatous leprosy with erythema nodosum leprosum (ENL) presenting as a myeloid leukemoid reaction is reported. Very high leucocyte count with immaturity of the cells in myeloid series was present in peripheral blood. High leucocyte alkaline phosphatase score, absence of hepatosplenomegaly and transient nature of leukemoid reaction differentiated it from chronic myeloid leukemia and acute myeloblastic leukemia. The possible mechanisms of leukemoid reaction in ENL are discussed.
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PMID:Leukemoid reaction in erythema nodosum leprosum in a leprosy patient. 325 36

A 52 yr old Caucasian female (F. E.) had hemolytic anemia, a leukemoid reaction, and fatal sepsis due to Escherichia coli. Her leukocytes ingested bacteria normally but did not kill catalase positive Staphylococcus aureus, Escherichia coli, and Serratia marcescens. An H(2)O(2)-producing bacterium, Streptococcus faecalis, was killed normally. Granule myeloperoxidase, acid and alkaline phosphatase, and beta glucuronidase activities were normal, and these enzymes shifted normally to the phagocyte vacuole (light and electron microscopy). Intravacuolar reduction of nitroblue tetrazolium did not occur. Moreover, only minimal quantities of H(2)O(2) were generated, and the hexose monophosphate shunt (HMPS) was not stimulated during phagocytosis. These observations suggested the diagnosis of chronic granulomatous disease. However, in contrast to control and chronic granulomatous disease leukocytes, glucose-6-phosphate dehydrogenase activity was completely absent in F. E. leukocytes whereas NADH oxidase and NADPH oxidase activities were both normal. Unlike chronic granulomatous disease, methylene blue did not stimulate the hexose monophosphate shunt in F. E. cells. Thus, F. E. and chronic granulomatous disease leukocytes appear to share certain metabolic and bactericidal defects, but the metabolic basis of the abnormality differs. Chronic granulomatous disease cells lack oxidase activity which produces H(2)O(2); F. E. cells had normal levels of oxidase activity but failed to produce NADPH due to complete glucose-6-phosphate dehydrogenase deficiency. These data indicate that a complete absence of leukocyte glucose-6-phosphate dehydrogenase with defective hexose monophosphate shunt activity is associated with low H(2)O(2) production and inadequate bactericidal activity, and further suggest an important role for NADPH in the production of H(2)O(2) in human granulocytes.
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PMID:Complete deficiency of leukocyte glucose-6-phosphate dehydrogenase with defective bactericidal activity. 440 Dec 71

Early chronic myeloid leukemia (CML) and leukemoid reaction (LR) sometimes show similar histological pictures. In order to assess the efficacy of immunohistochemistry in the discrimination of the two forms, twenty bone marrow (BM) trephines of patient with CML and twenty with LR were immunostained and studied. A wide spectrum of antibodies effective on paraffin-embedded tissues (NP 57 anti-neutrophil elastase, Leu M1, MAC 387, KP1, Y2/51, LCA, UCHL1, L26, BerH2 and Glycophorin A) and directed against granulopoietic, erythropoietic, megakaryocytic, monocytic and lymphoid cells was tested by means of the alkaline phosphatase anti-alkaline phosphatase (APAAP) method. Expression of neutrophil elastase in CML and LR showed a different pattern of reactivity in normal and neoplastic granulocytic cells and Y2/51 put in evidence significant discrepancies of megakaryocytes in the two groups. Moreover, a greater number of histiocytic, lymphoid and erythropoietic cells were detected in LR after immunostaining with KP1, LCA, UCHL1, L26 and Glycophorin A. The different immunophenotypical pictures observed, suggest the value of immunohistochemistry as a supplementary diagnostic tool for the differential diagnosis between early CML and LR.
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PMID:[Immunophenotyping of early-phase chronic myeloid leukemia and leukemoid reaction]. 770 38

A case of appendiceal abscess presenting leukemoid reaction is reported. A seven-month-old male infant was admitted because of fever and diarrhea. Anemic conjunctiva and mild abdominal distension were the only physical findings on arrival. Progressive leukocytosis with left shift maturation was noted during hospitalization. The maximal white blood cell count was 178,000/cumm, with a high leukocyte alkaline phosphatase score. On the fourth day of admission, a palpable right lower quadrant abdominal mass with local tenderness was found. Appendiceal abscess was diagnosed by both ultrasonography and computed tomography. The infant received triple antibiotic therapy consisting of ampicillin, gentamicin, and clindamycin for 10 days; the mass disappeared two weeks later. As appendiceal abscess is a rare occurrence in infants and its production of a leukemoid reaction is even rarer, the case described here may cause a diagnostic challenge, and deserves attention from clinicians.
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PMID:Leukemoid reaction in a child with appendiceal abscess: a case report. 803 47

A 30-year-old man with chronic neutrophilic leukemia (CNL) in association with monoclonal gammopathy is presented. Physical examination on admission revealed moderate hepatosplenomegaly. Initial blood count showed neutrophilic leukocytosis (42.2 x 10(9)/1 with 90% mature neutrophils). Leukocyte alkaline phosphatase (LAP) score was elevated. Bone marrow aspiration showed myeloid hyperplasia without dysplastic features. Karyotypic and molecular analyses of bone marrow cells showed the absence of Philadelphia (Ph1) chromosome and bcr gene rearrangement. Because there was no underlying infection or neoplasm, he was diagnosed as having CNL associated with IgG kappa-type monoclonal gammopathy (IgG, 1,269 mg/dl). In addition to its association with monoclonal gammopathy of undetermined significance (MGUS), the present case was also characterized by spontaneous remission of CNL during the 12-year follow-up, accompanied by a gradual increase in serum IgG levels up to 3,000 mg/dl. As far as we know, there have been 19 cases of CNL associated with monoclonal gammopathy in the literature. The median survival of these cases was 5 years. Although there have been only 6 cases of CNL associated with MGUS, survival of these cases was particularly favorable. Taken together with the observation that leukocytosis and hepatosplenomegaly in the present case subsided without specific treatment, we speculate that myeloid proliferation in the present case may have been a leukemoid reaction to underlying monoclonal gammopathy.
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PMID:Chronic neutrophilic leukemia associated with monoclonal gammopathy of undetermined significance. 863 44


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