Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In this study, we investigated a possible association between the degree of macrophage activation - as measured by serum neopterin concentrations - and disturbances of iron metabolism, determined by the concentrations of ferritin and serum iron, in patients with malignant disorders. Additionally we evaluated correlations between these factors and the degree and type of anaemia. Seventy-three patients, who suffered from non-Hodgkin's lymphoma (NHL) (n = 43), Hodgkin's disease (n = 11), myeloma or monoclonal gammopathy of unknown significance (n = 9), myelodysplastic syndrome (n = 1), and solid tumours (n = 9), were examined. Mean neopterin levels were raised in all groups, patients with NHL showing the highest concentrations. Ferritin but not neopterin concentrations were higher in males than in females. A significant correlation was found between neopterin and ferritin concentrations (p less than 0.01). Considering only female patients the strength of the correlation was the same (p less than 0.02). In addition, we found inverse correlations of neopterin with haemoglobin and iron concentrations (all p less than 0.01). Similar relationships existed in patients during follow-up. Our results support the hypothesis of an association between the degree of activation of macrophages and the development of anaemia by a shift or iron towards the storage sites.
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PMID:Association between the activation of macrophages, changes of iron metabolism and the degree of anaemia in patients with malignant disorders. 164 56

Ferritin H (heavy) and L (light) subunits in red cells were determined in normal subjects, patients with myelodysplastic syndrome (MDS) and other haematological disorders by means of enzyme-linked immunosorbent assay (ELISA) using monoclonal antibodies to ferritin H and L subunits. The mean contents of ferritin H and L subunits in red cells in healthy individuals were H 8.0 + 0.8 attogram (ag)/cell (lag = 10 x 10(-18)g)(mean +/- SE), L 4.8 +/- 0.4 ag/cell respectively. The values of both subunits in normal male (H 10.5 +/- 1.3 ag/cell, L 5.9 +/- 0.7 ag/cell) were significantly higher than those of normal female (H 5.4 +/- 0.8 ag/cell, L 3.9 +/- 0.5 ag/cell). Significantly elevated H and L subunit contents in red cells were observed in patients with refractory anemia (RA)(H 138.2 +/- 72.0 ag/cell, L 57.0 +/- 20.9 ag/cell) and refractory anemia with excess of blasts (RAEB)(H 97.4 +/- 36.9 ag/cell, L 49.3 +/- 18.4 ag/cell) as compared with those of normal subjects. On the other hand, both parameters decreased in patients with iron deficiency anemia (IDA)(H 2.4 +/- 0.3 ag/cell, L 1.5 +/- 0.3 ag/cell). H/L ratio in patients with RA (2.7 +/- 0.5) was significantly higher than those of normal subjects (1.8 +/- 0.1) indicating relative increase of red cell ferritin H subunit in patient with MDS. The measurement of red cell ferritin H and L subunits by ELISA could be useful for evaluating dyserythropoiesis or ineffective hemopoiesis in MDS.
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PMID:[Clinical significance of red cell ferritin H and L subunits in myelodysplastic syndrome]. 792 83

The main objective of the study was to analyze the incidence of iron overload (IO) and its management in transfusion-dependent patients with low-risk myelodysplastic syndrome (MDS) before the license of deferasirox. This observational, cross-sectional, and multicenter study was conducted from January to May 2007 in 81 Spanish hospitals. Eligible patients had a low or intermediate-1 risk score and had to have received at least ten units of packed red blood cell (PRBC). Of the 549 patients analyzed, 75% had received more than 20 PRBC units since diagnosis; 14% had IO at diagnosis and 58% at last follow-up. Thirty-eight percent of patients received chelation therapy; of those, 92% were treated with desferrioxamine. Ferritin levels at the start of chelation therapy were higher than 1,000 microg/L in 76% and over 2,500 microg/L in 24% of patients. Of the 202 patients who received some form of chelation therapy, ferritin levels increased from a mean +/- SD of 1,986 +/- 1,398 to 2,480 +/- 1,648 microg/L at last follow-up in 86% (p < 0.001). In the remaining 29 patients treated with a minimally effective therapy, ferritin levels did not increase. Of these, only 11 patients received such therapy lasting more than 12 months. In conclusion, most low-risk transfusion-dependent MDS patients develop IO, but only a minority receives a minimally effective and timely iron chelation therapy.
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PMID:Iron overload and chelation therapy in patients with low-risk myelodysplastic syndromes with transfusion requirements. 1969 Aug 57

The risk and clinical significance of cardiac iron overload due to chronic transfusion varies with the underlying disease. Cardiac iron overload shortens the life expectancy of patients with thalassemia, whereas its effect is unclear in those with myelodysplastic syndromes (MDS). In patients with sickle cell anemia (SCA), iron does not seem to deposit quickly in the heart. Our primary objective was to assess through a multicentric study the prevalence of cardiac iron overload, defined as a cardiovascular magnetic resonance T2*<20 ms, in patients with thalassemia, SCA, or MDS. Patient inclusion criteria were an accurate record of erythrocyte concentrates (ECs) received, a transfusion history >8 ECs in the past year, and age older than 6 years. We included from 9 centers 20 patients with thalassemia, 41 with SCA, and 25 with MDS in 2012-2014. Erythrocytapharesis did not consistently prevent iron overload in patients with SCA. Cardiac iron overload was found in 3 (15%) patients with thalassemia, none with SCA, and 4 (16%) with MDS. The liver iron content (LIC) ranged from 10.4 to 15.2 mg/g dry weight, with no significant differences across groups (P = 0.29). Abnormal T2* was not significantly associated with any of the measures of transfusion or chelation. Ferritin levels showed a strong association with LIC. Non-transferrin-bound iron was high in the thalassemia and MDS groups but low in the SCA group (P<0.001). Hepcidin was low in thalassemia, normal in SCA, and markedly elevated in MDS (P<0.001). Two mechanisms may explain that iron deposition largely spares the heart in SCA: the high level of erythropoiesis recycles the iron and the chronic inflammation retains iron within the macrophages. Thalassemia, in contrast, is characterized by inefficient erythropoiesis, unable to handle free iron. Iron accumulation varies widely in MDS syndromes due to the competing influences of abnormal erythropoiesis, excess iron supply, and inflammation.
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PMID:Cardiac iron overload in chronically transfused patients with thalassemia, sickle cell anemia, or myelodysplastic syndrome. 2825 76