scholarly journals Iron metabolism, sickle cell disease, and response to cyanate

Blood ◽  
1975 ◽  
Vol 46 (4) ◽  
pp. 583-590 ◽  
Author(s):  
CM Peterson ◽  
JH Graziano ◽  
A de Ciutiis ◽  
RW Grady ◽  
A Cerami ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Serum Ferritin ◽  
Iron Metabolism ◽  
Bone Marrow Aspirate ◽  
Binding Capacity ◽  
High Serum ◽  
Cell Disease ◽  
Serum Ceruloplasmin

Abstract In an attempt to understand the variability of the hematologic response to oral sodium cyanate, iron metabolism was studied in a group of 39 patients with sickel cell disease. Eleven of the 39 patients were found to have no stainable iron in the marrow despite the fact that patients with sickle cell disease are generally considered to have hemosiderosis. The mean per cent saturation and total iron-binding capacity were in the low-normal range in sickle cell patients whether or not stainable iron was present in the bone marrow aspirate. Serum ferritin concentrations, on the other hand, were found to be high in both groups (greater than 500 mu g/liter) when compared to controls (60 mu g/liter). The high serum ferritin levels denoted significant total- body iron deposition which may be unavailable for normal metabolic processes. One patient with no stainable iron in the bone marrow aspirate did respond to iron therapy alone with an increase in hemoglobin concentration. Serum ceruloplasmin levels were also found to be high in sickle cell disease patients. The ability to respond to oral cyanate therapy was correlated with the amount of stainable iron in the bone marrow aspirate. These studies emphasize the necessity of a reevaluation of iron metabolism in the pathophysiology and treatment of sickle cell disease.

scholarly journals Iron metabolism, sickle cell disease, and response to cyanate

Blood ◽  
1975 ◽  
Vol 46 (4) ◽  
pp. 583-590
Author(s):  
CM Peterson ◽  
JH Graziano ◽  
A de Ciutiis ◽  
RW Grady ◽  
A Cerami ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Serum Ferritin ◽  
Iron Metabolism ◽  
Bone Marrow Aspirate ◽  
Binding Capacity ◽  
High Serum ◽  
Cell Disease ◽  
Serum Ceruloplasmin

In an attempt to understand the variability of the hematologic response to oral sodium cyanate, iron metabolism was studied in a group of 39 patients with sickel cell disease. Eleven of the 39 patients were found to have no stainable iron in the marrow despite the fact that patients with sickle cell disease are generally considered to have hemosiderosis. The mean per cent saturation and total iron-binding capacity were in the low-normal range in sickle cell patients whether or not stainable iron was present in the bone marrow aspirate. Serum ferritin concentrations, on the other hand, were found to be high in both groups (greater than 500 mu g/liter) when compared to controls (60 mu g/liter). The high serum ferritin levels denoted significant total- body iron deposition which may be unavailable for normal metabolic processes. One patient with no stainable iron in the bone marrow aspirate did respond to iron therapy alone with an increase in hemoglobin concentration. Serum ceruloplasmin levels were also found to be high in sickle cell disease patients. The ability to respond to oral cyanate therapy was correlated with the amount of stainable iron in the bone marrow aspirate. These studies emphasize the necessity of a reevaluation of iron metabolism in the pathophysiology and treatment of sickle cell disease.

Iron Overload in Acute Myelogenous Leukemia after Bone Marrow Transplantation.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 5336-5336
Author(s):  
Zahra Pakbaz ◽  
Roland Fischer ◽  
Nancy Noonan ◽  
Sherrie Shiota ◽  
Paul Harmatz ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Transplantation ◽  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Serum Ferritin ◽  
Marrow Transplantation ◽  
Cell Disease ◽  
Acute Leukemias ◽  
Wet Weight

Abstract Children with acute leukemias typically receive RBC transfusions during the course of their treatment. However, the severity and significance of transfusional iron overload is not known in this patient population. Earlier, we reported elevated serum ferritin (SF) in 5 patients with AML who received HLA-identical sibling bone marrow transplantation (BMT). However, SF has a wide predictive interval for liver iron concentration (LIC) in thalassemia and sickle cell disease and the current recommendation is to measure LIC to estimate total body iron burden. Further exploration of the SF-to-LIC ratio (SF/LIC) to investigate the relationship between SF and LIC has shown ratio differences by specific disease (SCD, thalassemia, genetic hemochromatosis), transfusion status and use of chelation. The reasons for these differences are not presently known. In this study LIC was measured within 2 weeks of serum ferritin (SF), in 8 AML patients after transplantation, to explore the significance of the elevated SF and to determine the range and character of the SF/LIC ratio after BMT for AML. LIC was measured (1–4 year after BMT) by a low temperature SQUID biosusceptometer system (Ferritometer®) under the standardized Hamburg-Torino-Oakland protocol. The range for LIC in healthy individuals measured by SQUID is 90–340 mg/g wet weight. The median serum ferritin was 1227 (582–1723) μg/l and the median LIC was 1284 (751–1612) mg/g wet weight or approximately 4 times greater than the upper limit of normal. ALT was measured in 4 patients of which 2 were mildly elevated. Neither LIC nor SF changed over the interval of follow-up extending to 3 years in 2 patients (aged 11.5y and 14.5 y) who returned annually for LIC measurements. The ratio of SF/LIC ranged from 0.5 to 1.4 (median: 0.9) in the patients with AML. This compares to ratios of 1.2 (0.6–2.6) in regularly transfused sickle cell disease patients (n=45), 0.87 (0.23–2.7) in transfusion dependent thalassemia patients and 0.32 (0.05–0.57) in transfusion independent thalassemia patients. These preliminary observations suggest that children with acute leukemias who undergo bone marrow transplantation develop significant transfusion related iron accumulation. Additional investigation should be undertaken to determine if AML patients would benefit from iron reduction therapy by phlebotomy after BMT.

scholarly journals Evidence for complement-mediated bone marrow necrosis in a young adult with sickle cell disease

2021 ◽  
Vol 86 ◽  
pp. 102508
Author(s):  
Melissa Azul ◽  
Surbhi Shah ◽  
Sarah Williams ◽  
Gregory M. Vercellotti ◽  
Alexander A. Boucher
Keyword(s):  
Bone Marrow ◽  
Young Adult ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Bone Marrow Necrosis

scholarly journals Serum ferritin and total units transfused for assessing iron overload in adults with sickle cell disease

2012 ◽  
Vol 157 (5) ◽  
pp. 645-647 ◽  
Author(s):  
Emma Drasar ◽  
Nisha Vasavda ◽  
Norris Igbineweka ◽  
Moji Awogbade ◽  
Marlene Allman ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Serum Ferritin ◽  
Cell Disease

scholarly journals Pulmonary, Gonadal, and Central Nervous System Status after Bone Marrow Transplantation for Sickle Cell Disease

2010 ◽  
Vol 16 (2) ◽  
pp. 263-272 ◽  
Author(s):  
Mark C. Walters ◽  
Karen Hardy ◽  
Sandie Edwards ◽  
Thomas Adamkiewicz ◽  
James Barkovich ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Bone Marrow ◽  
Nervous System ◽  
Bone Marrow Transplantation ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Marrow Transplantation ◽  
Cell Disease

Haemoglobinopathies

2013 ◽  
pp. 1466-1470
Author(s):  
David Rees
Keyword(s):  
Bone Marrow ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Lupus Erythematosus ◽  
Single Gene ◽  
Globin Genes ◽  
Globin Chain ◽  
Cell Disease ◽  
Chain Synthesis ◽  
Marrow Expansion

Inherited abnormalities of the globin genes are the commonest single-gene disorders in the world and fall into two main groups: thalassaemias and sickle cell disease. Thalassaemias are due to quantitative defects in globin chain synthesis which cause variable anaemia and ineffective erythropoiesis. Thalassaemia was initially thought to be a disease of the bones due to uncontrolled bone marrow expansion causing bony distortion, although this is now unusual with appropriate blood transfusions. Osteopenia, often severe, is a feature of most patients with thalassaemia major and intermedia, caused by bone marrow expansion, iron overload, endocrinopathy, and iron chelation. Treatment with bisphosphonates is generally recommended. Other rheumatological manifestations include arthropathy associated with the use of the iron chelator deferiprone. Sickle cell disease involves a group of conditions caused by polymerization of the abnormal -globin chain, resulting in abnormal erythrocytes which cause vaso-occlusion, vasculopathy, and ischaemic tissue damage. The characteristic symptom is acute bone pain caused by vaso-occlusion; typical episodes require treatment with opiate analgesia and resolve spontaneously by 5 days with no lasting bone damage. The frequency of acute episodes varies widely between patients. The incidence of osteomyelitis is increased, particularly with salmonella, although it is much rarer than acute vaso-occlusion. Avascular necrosis can affect the hips, and less commonly the shoulders and knees. Coincidental rheumatological disease sometimes complicates the condition, particularly systemic lupus erythematosus (SLE) which is more prevalent in populations at increased risk of sickle cell disease.

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