Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P02794 (ferritin)
 17,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

From August 1989 to May 1991, 52 patients with transfusion dependent thalassaemia major received L1 (1,2-dimethyl-3- hydroxypyrid-4-one), the oral iron chelator, for a period of 3-21 months (mean +/- SD: 14.2 +/- 6.8). Mean (+/- SD) urinary iron excretion varied from 6.2 +/- 4.6 mg/d on 25 mg/kg/d of L1 to 42.3 +/- 37.1 mg/d on 100 mg/kg/d of L1. Mean (+/- SD) drop in S ferritin was 1465 +/- 990 micrograms/l after 5.0 +/- 0.8 months to 3641.2 +/- 2299.3 micrograms/l after 20.1 +/- 0.9 months of therapy. There was no evidence of neutropenia, thrombocytopenia, ear or eye toxicity. L1-related arthralgia, which was reversible on dose reduction or stoppage, was seen in 20 patients (38.5%), while minor gastrointestinal (GI) tract symptoms occurred in seven (3.5%) cases. We conclude that although L1 is an effective iron chelator, further studies are required to understand the mechanism of L1 related arthralgia and also to find a safer but effective dose on which incidence of L1 related arthralgia is minimal.
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PMID:Long-term assessment of efficacy and safety of L1, an oral iron chelator, in transfusion dependent thalassaemia: Indian trial. 828 Jun 22

Erythrocyte basic ferritin (EF) concentration was determined in 64 normal subjects, 123 patients with anemia and 12 patients with leukopenia and thrombocytopenia. There was a significant difference between males and females. Other iron indices, including plasma iron (PI), total iron binding capacity (TIBC), zinc protoporphyrin (ZnPP) and plasma ferritin (PF) were also determined in all the subjects and bone marrow iron stain was determined in the 135 patients. The lowest EF concentration was seen in patients with iron deficiency anemia, being significantly lower than that in normal subjects. EF concentration in patients with iron deficiency erythropoiesis was also lower than that in normal subjects and at the same time significantly different from that in patients with iron deficiency anemia. EF concentration increased prior to PF concentration in patients with iron deficiency anemia who had been treated for a period of 1-8 weeks. EF concentration in patients with anemia of chronic diseases had a significant difference as compared with that in normal subjects and in patients with iron deficiency anemia, but EF concentration in those patients who were accompanied by iron deficiency was similar to that in patients with simple iron deficiency anemia. EF concentration in some iron overloaded patients (aplastic anemia, megaloblastic anemia, MDS etc.) was significantly higher than that in normal subjects. It was demonstrated that there was a good correlation between EF concentration and bone marrow sideroblastic iron in the rank correlation analysis of the iron indices in 135 patients (rs 0.893, P less than 0.01). PF concentration had the best correlation with marrow iron (rs 0.948, P less than 0.01).
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PMID:[Evaluation of erythrocyte basic ferritin in the diagnosis of anemia]. 208

We report a case of infantile acute leukemia with t(16; 21) (p11; q22). The patient was a phenotypically normal one-year-old girl without lymphadenopathy or hepatosplenomegaly. Her peripheral blood at diagnosis showed anemia, thrombocytopenia, and many circulating blasts. Bone marrow blasts were monocytoid with fine reticular nuclear chromatin, abundant grayish-blue cytoplasm with occasional pseudopods or cytoplasmic projections and active hemophagocytosis. Serum levels of lysozyme and ferritin were normal. These blasts were not stained with butyrate esterase and immunologic study showed KOR-P77+ (anti-megakaryocyte monoclonal antibody), MY9+, Ia-. Electron microscopic examination failed to show platelet peroxidase activity. Remission was not induced by mini-COAP or VP-16 and the patient died of measles pneumonitis. The patient's blasts took typical appearance of megakaryoblasts later in the course, although some of them retained the ability of hemophagocytosis observed in the original blasts. This case is considered to be quite atypical since leukemic cells with active hemophagocytosis, megakaryoblastic appearance and t(16; 21) (p11; q22) have not been reported in the literature.
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PMID:[Acute leukemia with active hemophagocytosis, positive immunologic markers for the megakaryocyte-platelet lineage, and translocation (16; 21) (p11; q22]. 231 8

We report a case of malignant histiocytosis diagnosed by liver-spleen biopsy under laparoscopy. A 49-year-old woman was admitted to our hospital with thrombocytopenia, moderate anemia and hypoproteinemia. Her bone marrow findings revealed erythroid and megakaryocyte hyperplasia, and the serum ferritin concentration was 2,250 ng/ml though she had not received any blood transfusions. Ferrokinetics analysis showed the pattern of ineffective erythropoiesis, and the half-lives of erythrocytes and platelets were both shortened. Her hepatosplenomegaly gradually increased accompanied by increasing serum ferritin level to 10,000 ng/ml. Liver-spleen biopsy was carried out under laparoscopy and revealed infiltration of atypical histiocytes with erythrophagocytosis, which were positive for S-100 and ferritin but negative for lysozyme. The rate of glycosylation in whole serum ferritin, analyzed by using concanavalin-A binding method, showed that her glycosylated ferritin content was only 8.3%, whereas in sera after iron overloading, it was about 70%. Serum isoferritin profiles by isoelectric focussing were studied, and isoferritin pattern from malignant histiocytosis was the same as that in iron overloading after neuraminidase treatment. These findings suggest that serum ferritin is synthesized in proliferating histiocytes and released in the plasma as a nonsecretory type (nonglycosylated ferritin) in this case.
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PMID:[Mechanism of hyperferritinemia in a case of malignant histiocytosis]. 238 9

We have examined the efficacy of various drugs in 44 patients with MDS and found the different effectiveness which depends on the type of MDS. Namely, RA appears to respond to steroid hormone, androgen, and/or vitamin D3, regardless of single or combined use. In particular, it is obvious in androgen, and as our previous reports, high content of acidic ferritin in RBC with RA have changed to more basic ones by treatment with androgen. On the contrary, these drugs were not effective on RAEB, RAEB-T, and CMML. A long-term observation is needed to determine whether the prolonged or decreased occurrence of leukemia could be obtained in the effective cases with RA. Most of the cases who did not develop overt leukemia during this study died of bleeding or infections due to thrombocytopenia or leukocytopenia, thus indicating that supportive therapies are important in patients with MDS. Since it has recently been reported that recombinant G-CSF or GM-CSF is helpful to increase the number of leucocyte and to enhance their functional recovery in MDS, these factors may be powerful agents against infections when they are carefully used with regard to the activation of leukemic clones.
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PMID:[Therapy of the preleukemic state: effect of androgens on refractory anemia]. 283 1

We examined hematopoietic disorders in 74 patients with various malignancies. The proportions of patients with anemia in the case of esophageal carcinoma, gastric carcinoma, colorectal carcinoma, hepatoma, and pancreatic carcinoma were 75%, 87.5%, 77%, 64% and 87.5%, respectively. Hypochromic microcytic anemia was mainly observed in showed patients with carcinoma of the gastro-intestinal tract which showed severe bleeding. The major proportion of patients with hepatoma and pancreatic carcinoma showed normochromic normocytic anemia. The WBC count was usually within the normal range except for patients with liver cirrhosis or generalized metastasis who showed decreased WBC counts. The mean lymphocyte count in the peripheral blood, which is thought to be correlated with the prognosis of cancer patients, was less than 1,500/microliter except for patients with gastric carcinoma, whose five-year survival rate was 28.6%. Monocytosis was mainly observed in patients with colorectal carcinoma and pancreatic carcinoma, accounting for 24.5% of the total cases. This finding may suggest some relationship between cancer and monocytes. Thrombocytosis was seen in patients with severe bleeding, but thrombocytopenia was seen in patients with liver cirrhosis. Most cancer patients showed normo-cellular marrow and normal M/E. We also examined the ferrokinetics and ferritin levels in cancer patients.
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PMID:[Hematological disorders in malignancy]. 301 96

Seventy-four patients with beta-thalassemia major were studied to test the hypothesis that a deficiency of protein C (PC) and antithrombin III (AT III), both antithrombotic proteins, could contribute to the pathogenesis of CNS thromboembolic lesions. In 70 patients, PC levels were found to be significantly lower than normal, whereas AT III activity was found to be lower only in 41 patients. The lowest values of PC and AT III were found in older splenectomized patients, a low PC value only was found in chronic hepatitis patients. Prothrombin time and fibrinogen were found to be particularly abnormal in patients with chronic hepatitis and without spleen. A relatively poor correlation was observed between PC and AT III (p less than 0.02). PC correlated with age (p less than 0.001), transfusional iron (p less than 0.001) and ferritin (p less than 0.001). It also correlated with serum albumin (p less than 0.001), prothrombin time (p less than 0.001) and fibrinogen (p less than 0.02) and with serum transaminases (GPT) (p less than 0.001). The same indexes correlated less significantly with AT III activity. Nevertheless, only 2 of our patients had CNS thromboembolic complications. It is probable that low clotting factors, hyperfibrinolysis and thrombocytopenia (which are common in chronic liver disease) could have the opposite effect on hemostasis from that of low levels of anticoagulant proteins such as PC and AT III.
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PMID:Protein C and antithrombin III in polytransfused thalassemic patients. 310 18

Mild leukopenia and thrombocytopenia are common in multitransfused hemophiliacs. Because little attention has previously been directed to measurements of erythropoiesis in these patients, we prospectively examined hemoglobin concentration and RBC indices in 94 children and young adults with hemophilia during comprehensive clinic visits. Additional studies performed in many included serum transferrin saturation, ferritin, haptoglobin, and free erythrocyte protoporphyrin measurements. Hemoglobin concentrations were recorded as age-related percentile values. Hemophiliacs of all ages and degrees of severity often had lower than average values for hemoglobin; 31% had values less than the third percentile, 46% less than the tenth percentile, and 83% less than the mean value. Reduced hemoglobin percentile values were unrelated to age, severity of disease, or human immunodeficiency virus antibody status. Only five patients had an obvious cause for anemia. Serum ferritin, transferrin saturation, and erythrocyte protoporphyrin values were usually normal, indicating that iron deficiency and anemia of chronic disease were uncommon. Although serum haptoglobin was reduced in 44% of the patients, reticulocyte count was infrequently increased. We conclude that hemoglobin values are frequently less than the mean normal values for age in hemophiliacs. Although frank anemia is common, it is usually mild and without obvious cause. Hemophiliac individuals with slightly reduced hemoglobin values probably do not routinely require detailed investigation for occult blood loss, iron deficiency, or inflammation.
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PMID:Reduced hemoglobin values in children and young adults with hemophilia. 336 83

Coulter profiles with differential white cell counts, serum ferritin, and haptoglobin levels were determined in venous blood samples obtained from 90 males (M) and 25 females (F) immediately before and after completion of a competitive marathon (42.2 km) race. In an additional 20 male runners, the same measurements were performed serially during the 24 h following their completion of the race. In the pre-race samples from 90 M and 25 F, hypoferritinemia was present in 4/22 M and 1 F found to be mildly anemic. Neutropenia was detected in 4 M and 3 F and mild thrombocytopenia in 2 M. Haptoglobin levels were normal in all the female runners but reduced (less than 0.3 g/l) in 6 M. All post-race samples (88 M and 25 F) were characterized by a reactive neutrophilia and thrombocytosis including those with pre-race neutropenia or thrombocytopenia. An unexpected and incompletely explained sex difference in packed cell volume (PCV) response was observed. In males, the mean PCV increased from 0.425 +/- 0.021 to 0.444 +/- 0.028 (P less than 0.0001) whereas in females it decreased from 0.437 +/- 0.029 to 0.423 +/- 0.036 (P less than 0.05). In the post-race samples, anhaptoglobinemia was found in 13/88 M and 4/25 F. In the 20 male runners studied serially for 24 h after the race, the major changes involved a progressive increase in mean plasma volume (17.4% +/- 12.2% at 24 h) compared with the pre-race value, a progressive and significant increase in MCH and MCHC probably indicating a loss in red cell water and the gradual reversion of the reactive neutrophilia and thrombocytosis to basal levels.
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PMID:Hematological changes associated with marathon running. 355 78

A 32 year old woman with severe aplastic anaemia required frequent transfusions and consequently developed hyperferrioxaemia (54 microMol/l) and hyperferritinaemia (1,700 ng/ml). For the treatment of transfusion siderosis she was given 18 high dose courses each comprising 35 g of desferrioxamine. Because of pre-existing thrombocytopenia (platelet count 5 X 10(9)/l) the iron chelating agent was given by continuous intravenous infusion over 3 1/2 days. High dose desferrioxamine had to be abandoned because of severe bone pain. The desferrioxamine infusions achieved a negative iron balance, iron loss after each infusion being 100 to 200 mg in the urine and 400 mg in the faeces. Serum iron and ferritin concentrations fell almost to normal. This report shows that faecal iron excretion must be taken into account in assessing the balance of iron input and output during desferrioxamine treatment.
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PMID:High dosage desferrioxamine therapy in a female patient with acquired aplastic anaemia and transfusion siderosis. 363 32


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