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abnormal deposit of hemosiderin; often a symptom of thalassemia or hemochromatosis (同)haemosiderosis

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/07/21 21:19:29」(JST)

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Hemosiderosis (AmE) or haemosiderosis (BrE) is a form of iron overload disorder resulting in the accumulation of hemosiderin.

Types include:

Transfusion hemosiderosis
Idiopathic pulmonary hemosiderosis

Hemosiderin deposition in the lungs is often seen after diffuse alveolar hemorrhage, which occurs in diseases such as Goodpasture's syndrome, Wegener's granulomatosis, and idiopathic pulmonary hemosiderosis. Mitral stenosis can also lead to pulmonary hemosiderosis. Hemosiderin collects throughout the body in hemochromatosis. Hemosiderin deposition in the liver is a common feature of hemochromatosis and is the cause of liver failure in the disease. Deposition in the pancreas leads to diabetes and in the skin leads to hyperpigmentation. Hemosiderin deposition in the brain is seen after bleeds from any source, including chronic subdural hemorrhage, Cerebral arteriovenous malformations, cavernous hemangiomata. Hemosiderin collects in the skin and is slowly removed after bruising; hemosiderin may remain in some conditions such as stasis dermatitis. Hemosiderin in the kidneys have been associated with marked hemolysis and a rare blood disorder called paroxysmal nocturnal hemoglobinuria.

Hemosiderin may deposit in diseases associated with iron overload. These diseases are typically diseases in which chronic blood loss requires frequent blood transfusions, such as sickle cell anemia and thalassemia, though beta thalassemia minor has been associated with hemosiderin deposits in the liver in those with non-alcoholic fatty liver disease independent of any transfusions.^[1]^[2]

Treatment

Treatment for hemosiderin focuses on limiting the effects of the underlying disease leading to continued deposition. In hemochromatosis, this entails frequent phlebotomy granulomatosis, immune suppression is required. Limiting blood transfusions and institution of iron chelation therapy when iron overload is detected are important when managing sickle-cell anemia and other chronic hemolytic anemias.

Diagnosis

There are several methods available for diagnosing and monitoring hemosiderosis including:

Serum ferritin
Liver biopsy
MRI

Serum ferritin is a low cost, readily available, and minimally invasive method for assessing body iron stores. However, the major problem with using it as an indicator of hemosiderosis is that it can be elevated in a range of other medical conditions unrelated to iron levels including infection, inflammation, fever, liver disease, renal disease and cancer.

While liver biopsies provide a direct measure of liver iron concentration, the small sample size relative to the size of the liver can lead to sampling errors given the heterogeneity of iron concentration within the liver. Furthermore, the invasive nature of liver biopsy and the associated risks of complications (which can range from pain, haemorrhage, gallbladder perforation and other morbidities through to death in approx 1 in 10,000 cases) prevent it being used as a regular monitoring tool.

MRI is emerging as an alternative method for measuring liver iron loading due to the fact that it is non-invasive, safer and generally cheaper to perform than liver biopsy; does not suffer from problems with sampling variability; and can be used more frequently than performing liver biopsies.^[3]

References

^ Valenti, Luca; Canavesi, Elena; Galmozzi, Enrico; Dongiovanni, Paola; Rametta, Raffaela; Maggioni, Paolo; Maggioni, Marco; Fracanzani, Anna Ludovica; Fargion, Silvia (2010). "Beta-globin mutations are associated with parenchymal siderosis and fibrosis in patients with non-alcoholic fatty liver disease". Journal of Hepatology 53 (5): 927–33. doi:10.1016/j.jhep.2010.05.023. PMID 20739079.
^ Stickel, Felix; Hampe, Jochen (2010). "Dissecting the evolutionary genetics of iron overload in non-alcoholic fatty liver disease". Journal of Hepatology 53 (5): 793–4. doi:10.1016/j.jhep.2010.06.010. PMID 20739088.
^ St. Pierre, T. G.; Clark, PR; Chua-Anusorn, W; Fleming, AJ; Jeffrey, GP; Olynyk, JK; Pootrakul, P; Robins, E; Lindeman, R (2005). "Noninvasive measurement and imaging of liver iron concentrations using proton magnetic resonance". Blood 105 (2): 855–61. doi:10.1182/blood-2004-01-0177. PMID 15256427.

External links

FerriScan - MRI-based test to measure iron overload

UpToDate Contents

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1. 特発性肺ヘモジデローシス idiopathic pulmonary hemosiderosis
2. 遺伝性ヘモクロマトーシスの臨床症状および診断 clinical manifestations and diagnosis of hereditary hemochromatosis
3. サラセミアおよびその他の鉄過剰状態に対するキレート療法 chelation therapy for thalassemia and other iron overload states
4. 成人におけるセリアック病の病因、疫学、および臨床症状 pathogenesis epidemiology and clinical manifestations of celiac disease in adults
5. 成人における鉄欠乏性貧血の原因および診断 causes and diagnosis of iron deficiency anemia in the adult

English Journal

Extracorporeal life support for a 5-week-old infant with idiopathic pulmonary hemosiderosis.

Gutierrez S1, Shaw S, Huseni S, Sachdeva S, Costello JP, Basu S, Nath DS, Klugman D.
European journal of pediatrics.Eur J Pediatr.2014 Dec;173(12):1573-6. doi: 10.1007/s00431-013-2130-4. Epub 2013 Aug 14.
Idiopathic pulmonary hemosiderosis is a rare disease defined by the triad of iron deficiency anemia, hemoptysis, and diffuse pulmonary infiltrates on chest radiograph. Idiopathic pulmonary hemosiderosis is known to cause dyspnea and, in some cases, acute onset of massive pulmonary hemorrhage which i
PMID 23942745

Quantification of liver iron with MRI: State of the art and remaining challenges.

Hernando D1, Levin YS, Sirlin CB, Reeder SB.
Journal of magnetic resonance imaging : JMRI.J Magn Reson Imaging.2014 Nov;40(5):1003-21. doi: 10.1002/jmri.24584. Epub 2014 Mar 3.
Liver iron overload is the histological hallmark of hereditary hemochromatosis and transfusional hemosiderosis, and can also occur in chronic hepatopathies. Iron overload can result in liver damage, with the eventual development of cirrhosis, liver failure, and hepatocellular carcinoma. Assessment o
PMID 24585403

High noon back pain- severe pseudoradicular pain as a lead symptom of superficial siderosis: a case report.

Saft C1, Siglienti I2, Gold R2, Schlamann M3, Hindy NE4, Sure U4, Forsting M3.
Therapeutic advances in neurological disorders.Ther Adv Neurol Disord.2014 Nov;7(6):276-8. doi: 10.1177/1756285614548189.
A superficial siderosis of the central nervous system following a traumatic cervical nerve root avulsion usually leads to gait difficulties and hearing loss, whereas back pain is described only rarely. Here we report on the first case with circadian occurrence of severe back pain as the only symptom
PMID 25371709

Japanese Journal

Microscopic Sclerosing Hemangioma Diagnosed by Histopathological Examination after Lung Cancer Surgery

GOTO Taichiro,MAESHIMA Arafumi,KATO Ryoichi
Annals of thoracic and cardiovascular surgery 17(5), 507-510, 2011-10-01
… This lesion exhibited hemorrhage, hemosiderosis, calcification, and varying degrees of fibrosis, leading to the diagnosis of sclerosing hemangioma. …
NAID 10030235267

Prognosis in Adult Patients with Idiopathic Pulmonary Hemosiderosis

Miwa Seiichi,Imokawa Shiro,Kato Masato,Ide Kyotaro,Uchiyama Hiroshi,Yokomura Koushi,Suda Takafumi,Shirai Masahiro,Hayakawa Hiroshi,Chida Kingo
Internal Medicine 50(17), 1803-1808, 2011
… Background Diffuse alveolar hemorrhage (DAH) of unknown cause has been characterized as idiopathic pulmonary hemosiderosis (IPH). …
NAID 130002063074

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リンク元	「ヘモジデリン沈着症」
拡張検索	「idiopathic pulmonary hemosiderosis」「pulmonary hemosiderosis」

「ヘモジデリン沈着症」

Hemosiderosis
	Classification and external resources
	Image of a kidney viewed under a microscope. The brown areas contain hemosiderin
MeSH	D006486

　　[★]

英: hemosiderosis
ラ: haemosiderosis
同: 血鉄症、ヘモジデローシス
関: ヘモジデリン、ヘモクロマトーシス

[show details]

ヘモシデローシス : 約 86 件
ヘモジデローシス : 約 4,730 件

定義

組織内に染色可能な鉄が存在している状態 (HAR.2429)
病的な状態を強調するときはヘモクロマトーシス

「idiopathic pulmonary hemosiderosis」

　　[★] 特発性肺ヘモジデリン沈着症 IPH

「pulmonary hemosiderosis」

　　[★] 肺ヘモジデリン沈着症

[1] Valenti, Luca; Canavesi, Elena; Galmozzi, Enrico; Dongiovanni, Paola; Rametta, Raffaela; Maggioni, Paolo; Maggioni, Marco; Fracanzani, Anna Ludovica; Fargion, Silvia (2010). "Beta-globin mutations are associated with parenchymal siderosis and fibrosis in patients with non-alcoholic fatty liver disease". Journal of Hepatology 53 (5): 927–33. doi:10.1016/j.jhep.2010.05.023. PMID 20739079.

[2] Stickel, Felix; Hampe, Jochen (2010). "Dissecting the evolutionary genetics of iron overload in non-alcoholic fatty liver disease". Journal of Hepatology 53 (5): 793–4. doi:10.1016/j.jhep.2010.06.010. PMID 20739088.

[pmid15256427-3] St. Pierre, T. G.; Clark, PR; Chua-Anusorn, W; Fleming, AJ; Jeffrey, GP; Olynyk, JK; Pootrakul, P; Robins, E; Lindeman, R (2005). "Noninvasive measurement and imaging of liver iron concentrations using proton magnetic resonance". Blood 105 (2): 855–61. doi:10.1182/blood-2004-01-0177. PMID 15256427.

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案内

案内

hemosiderosis