Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.1.3.1 (alkaline phosphatase)
 47,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 72-year-old male had complained of right back pain and bleeding from his tongue. He was admitted to our department on May 18, 1989. Physical examination revealed hepatosplenomegaly. Peripheral blood findings were as follows: RBC was 3.80 x 10(6)/microliters. Hb 12.2 g/dl, Ht 36.5%, platelet count 735 x 10(3)/microliters, WBC 22,100/microliters, leukoerythroblastosis present. Neutrophil alkaline phosphatase score was normal. Serum vitamin B12 and plasma platelet-derived growth factor level were elevated. Skeletal X-ray revealed multiple punched-out lesions at the 8th thoracic vertebra, and 6th and 8th ribs. Serum IgG level was 3,900 mg/dl. Serum immunoelectrophoresis revealed IgG lambda-type M-protein. Because he complained of severe cervical pain, and skeletal X-ray examination revealed the fracture of 6th cervical vertebra, the operation was performed to remove the lesion. Biopsy of cervical lesion revealed plasmacytoma. M-protein was decreased and the size of the tumor was reduced after treatment with VCAP (vincristine, cyclophosphamide, adriamycin, prednisolone) regimen and interferon-alpha for multiple myeloma.
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PMID:[IgG lambda-type multiple myeloma associated with myelofibrosis accompanied by thrombocytosis]. 194 35

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

We demonstrated the clinical effectiveness of recombinant interferon-gamma (rIFN gamma) (Biogen) in 18 patients with Philadelphia-positive chronic myeloid leukemia. Sequential cytogenetic studies and molecular analyses of the breakpoint cluster region and for immunoglobulin and T cell rearrangements were performed every 3-4 months. In 13 patients who received treatment for a minimum of 3 months, the majority were treated with 1.5 mg/m2, t.i.w., i.v. Nonhematologic effects--particularly chills, rigors, myalgia, fatigue, headaches, and nausea--were significant. Complete or partial hematologic responses were observed in six patients, two of whom had approximately 20% normal metaphases after an average of 74 weeks of treatment. However, reversion to 100% Ph+ cells occurred 30 weeks later. In these two patients, in whom normal metaphases were found, no changes were observed in the presence of rearrangements of the breakpoint cluster region. In addition, the marrows remained hypercellular, and the leukocyte alkaline phosphatase score and B12 levels remained abnormal. No immunoglobulin or T cell beta-chain gene rearrangements were found. These data indicate the clinical effectiveness of rIFN gamma in some patients with chronic myeloid leukemia, although the fundamental nature of the disease is unaltered by this form of treatment.
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PMID:Recombinant gamma-interferon has activity in chronic myeloid leukemia. 215 24

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

Tetranectin, a protein recently identified in a wide variety of human secretory cells (Christensen, L., and I. Clemmensen. 1989. Histochemistry. 92:29-35) was found to colocalize with latent alkaline phosphatase activity in fractions well separated from azurophil granules, specific granules, gelatinase-containing granules, and plasma membranes when postnuclear supernatants of nitrogen-cavitated neutrophils were fractionated on discontinuous Percoll density gradients. Stimulation of intact neutrophils with nanomolar concentrations of FMLP, leukotriene B4, 10-100 U/ml of tumor necrosis factor, and granulocyte-macrophage colony-stimulating factor resulted in parallel release of tetranectin and translocation of alkaline phosphatase to the plasma membrane. Furthermore, intracellular pools of tetranectin and latent alkaline phosphatase were completely released from neutrophils under conditions that barely induced release of specific granules containing B12-binding protein. These findings indicate that tetranectin and latent alkaline phosphatase define an easily mobilizable population of cytoplasmic storage organelles in human neutrophils which are functionally distinguishable from azurophil, specific, and gelatinase-containing granules. These organelles may play an important role as stores of membrane proteins that are mobilized to the cell surface during stimulation by inflammatory mediators.
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PMID:Identification of a highly mobilizable subset of human neutrophil intracellular vesicles that contains tetranectin and latent alkaline phosphatase. 229 16

This prospective study was undertaken in patients scheduled for gastrectomy for peptic ulcer disease to determine the effect of partial gastrectomy with either Roux-en-Y (n = 11) or Billroth II anastomosis (n = 11) on the function of the small intestine. Patients were studied before and at 6 months (blood and small-intestinal function tests) and at 24 months (blood tests) postoperatively. Median postoperative body weights at 6 months (70.5 kg; p less than 0.01) and 12 months (70.3 kg; NS) were lower than preoperatively (73.0 kg). Haemoglobin concentrations at 6 months (8.9 mM; p less than 0.01) and at 24 months (9.1 mM; p less than 0.05) were also significantly reduced compared with the preoperative value (9.5 mM). However, neither at 6 nor at 24 months postoperatively were there significant changes for serum iron, iron saturation, folic acid, vitamin B12, protein, albumin, alkaline phosphatase, and calcium concentrations. Whereas no significant deterioration of the absorption of D-xylose and vitamin B12 or of faecal fat excretion was observed, the orocoecal transit time was significantly shortened from 98 to 50 min (p less than 0.01), the expiratory hydrogen excretion after a 50-g oral glucose load was significantly increased from 8 to 54 ppm (p less than 0.01), as was indicanuria from 257 to 368 mumol/24 h (p less than 0.01). Apart from a lower serum iron concentration and iron saturation index in the Roux-en-Y patients 6 months postoperatively (p less than 0.05), no significant differences between the two types of anastomosis were observed. It is therefore concluded that both in patients with Roux-en-Y and in those with Billroth II anastomosis most abnormalities observed after gastrectomy are secondary to an accelerated small-intestinal transit.
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PMID:Effect of gastrectomy with either Roux-en-Y or Billroth II anastomosis on small-intestinal function. 230 15

Forty-eight patients with polycythemia vera (PV) were retrospectively studied for incidence of acute leukemia over a 12 year period. Initial clinical features, hemogram, RBC mass, B12 levels, neutrophil alkaline phosphatase (NAP), and therapy given were studied for association with development of acute leukemia. There were 25 males and mean age at diagnosis was 61.4 years. Initial Hg was 18.38 +/- 1.86 g/dl, WBC 16.44 +/- 12.92 (x 1,000/mm3), platelets 632.94 +/- 303.81 (x 1,000/mm3), B12 1,030.93 +/- 445.20 pg/ml, and neutrophil alkaline phosphatase (NAP) score 136.63 +/- 55.14. Twenty-three patients were treated with phlebotomy alone and 25 received additional myelosuppressive therapy as follows--2 received p32 alone, 4 alkylating agents alone, 8 hydroxyurea (HU) alone, and 11 received 2 or more (multiple) of these agents. None of those treated with phlebotomy alone but 6 of 25 (24%) patients given myelosuppressive therapy developed acute leukemia (P = .03) after a mean period of 46.8 months from start of myelosuppressive therapy. Four of the 11 patients (36%) receiving multiple agent therapy developed acute leukemia (P = .019). Initial hemoglobin levels, but not the other clinical parameters, were significantly higher in patients who developed acute leukemia (P = .002), and this difference persisted in various subgroups receiving myelosuppressive therapy. Thus, high initial hemoglobin and use of any myelosuppressive therapy are associated with an increased risk of leukemic transformation in polycythemia vera. This risk becomes substantial with the use of two or more myelosuppressive agents. Since myelosuppressive therapy does not prolong survival, its role in the management of polycythemia vera should be reexamined.
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PMID:Leukemic transformation in polycythemia vera: analysis of risk factors. 232 2

A 62-year-old woman with chronic neutrophilic leukemia (CNL) is described. She presented in February 1988 for evaluation of leukocytosis of 3 years' duration with no complaint. Physical examination was normal. The leukocyte count was 20,100/microliters with 70% segmented neutrophils and 12% band forms. A myelogram showed marked myeloid hyperplasia and plasmacytosis (5.9%). Neutrophil alkaline phosphatase score, serum lysozyme and vitamin B12 levels were elevated. Cytogenetic analysis of the marrow aspirate showed normal karyotype, with no Philadelphia chromosome. Total serum protein (TP) was 7.5 g/dl with increased beta-globulin (23.5%), identified as monoclonal IgA kappa (3.3 g/dl) on immunoelectrophoresis. No activity of G-CSF was detected in the serum. A retrospective study revealed that the beta-globulin level was normal (6.3%, TP 6.9 g/dl) in 1980 and that it was slightly increased (11.6%, TP 7.0 g/dl) without leukocytosis (5,900/microliter) in 1981. In 1985, when leukocytosis obviously existed (9,900/microliter), the percentage of beta-globulin was increased to 17.5% (TP 7.2 g/dl). The possibility that monoclonal gammopathy preceded the leukocytosis must be admitted. On the basis of our observation, it is assumed that CNL and monoclonal gammopathy may be blood dyscrasias derived from a common precursor cell or that the immunological abnormality associated with monoclonal gammopathy may be implicated in the development of CNL.
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PMID:[Chronic neutrophilic leukemia associated with monoclonal gammopathy (IgA, kappa type)]. 250 2

The work was aimed at studying enzymes involved in the metabolism of flavin nucleotides, namely, riboflavin kinase (EC 2.7.1.26) and FAD pyrophosphorylase (EC 2.7.7.2), as well as flavin mononucleotide hydrolysis by acid phosphatase (EC 3.1.3.2) and alkaline phosphatase (EC 3.1.3.1) in Streptomyces olivaceus actively producing vitamin B12. No correlation could be established between changes in the activity of the above enzymes during the culture growth and the qualitative composition of flavins. The enzyme activity was assayed using, as an enzyme preparation, both intact cells and a cell-free extract obtained by disintegrating the mycelium with different techniques. The screening effect of phosphatases exerted when the activity of riboflavin kinase was assayed could be partly eliminated by adding sodium fluoride to the incubation medium. The localisation of the above enzymes in the cytoplasm is discussed.
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PMID:[Enzymes involved in the metabolism of flavin nucleotides in Streptomyces olivaceus]. 254 68

A case of blastic transformation of essential thrombocythemia (ET) is reported. A 69-year-old male was first admitted to hospital because of fever in February, 1982. He was diagnosed as having ET because of marked thrombocytosis (205.5 x 10(10)/1), absence of erythrocytosis, absence of splenomegaly, normal karyotype and no increment of blasts in the bone marrow, and normal levels of neutrophil alkaline phosphatase, vitamin B12 and folate. He was treated with busulfan, and subsequently his platelet count was well controlled for about five years. At the second admission, blasts were present in the peripheral blood, and later accounted for 49% of the total leukocyte count. Histological examination of a bone biopsy specimen showed homogeneous proliferation of blastic cells and slight reticulin fibrosis. At autopsy, the degree of bone marrow fibrosis had increased. This was considered to be a very rare case of ET with blastic transformation in the terminal phase.
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PMID:Blastic transformation of essential thrombocythemia. A case report. 258 80


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