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Thrombotic microangiopathy, abbreviated TMA, is a pathology that results in thrombosis in capillaries and arterioles, due to an endothelial injury.^[1] It may be seen in association with thrombocytopenia, anemia, purpura and renal failure.

The classic TMAs are hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. Other conditions with TMA include disseminated intravascular coagulation, scleroderma renal crisis, malignant hypertension, antiphospholipid antibody syndrome, and drug toxicities, e.g. calcineurin inhibitor toxicity.^[1]

Etiology[edit]

The etiology is dependent on the specific TMA.

Bacterial toxins are the primary cause of one category of thrombotic microangiopathy known as HUS or hemolytic uremic syndrome. Another major category is TTP or thrombotic thrombocytopenic purpura. It is caused by autoimmune or hereditary dysfunctions that activate the coagulation cascade or the complement system.

Some sources group TTP and HUS together,^[2] while other sources express skepticism about their common pathophysiology.^[3]

Presentation[edit]

The clinical presentation typically includes: fever, microangiopathic hemolytic anemia (see schistocytes in a blood smear), renal failure, thrombocytopenia, neurological manifestations.

Treatment[edit]

The treatment depends on the underlying condition.

References[edit]

^ ^a ^b Benz, K.; Amann, K. (May 2010). "Thrombotic microangiopathy: new insights.". Current Opinion in Nephrology and Hypertension 19 (3): 242–7. doi:10.1097/MNH.0b013e3283378f25. PMID 20186056.
^ http://www.merckmanuals.com/professional/sec12/ch143/ch143g.html
^ http://www.emedicine.com/emerg/topic579.htm

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1. 成人における血栓性血小板減少性紫斑病-溶血性尿毒症症候群の原因 causes of thrombotic thrombocytopenic purpura hemolytic uremic syndrome in adults
2. 成人における血栓性血小板減少性紫斑病-溶血性尿毒症症候群の診断 diagnosis of thrombotic thrombocytopenic purpura hemolytic uremic syndrome in adults
3. 成人における血栓性血小板減少性紫斑病-溶血性尿毒症症候群の治療 treatment of thrombotic thrombocytopenic purpura hemolytic uremic syndrome in adults
4. 抗リン脂質抗体症候群と腎臓 antiphospholipid syndrome and the kidney
5. 造血細胞移植後の腎臓病 kidney disease following hematopoietic cell transplantation

English Journal

Long-term outcomes of thrombotic microangiopathy treated with plasma exchange: A systematic review.

Thejeel B1,2, Garg AX2,3,4, Clark WF2,4, Liu AR2, Iansavichus AV2, Hildebrand AM5.
American journal of hematology.Am J Hematol.2016 Jun;91(6):623-30. doi: 10.1002/ajh.24339. Epub 2016 Apr 6.
With the adoption of plasma exchange as standard treatment for thrombotic microangiopathy (TMA), more patients are surviving and long-term outcomes have greater relevance. We conducted a systematic review to synthesize and evaluate the quality of evidence on long-term outcomes of TMA among adults tr
PMID 26910131

Long-term outcomes of health-related quality of life following diverse thrombotic microangiopathy syndromes.

Page EE1,2, Jiang Y2, Terrell DR1, Vesely SK1, George JN1,2.
American journal of hematology.Am J Hematol.2016 Jun;91(4):E278-9. doi: 10.1002/ajh.24315.
PMID 26822687

Hemophagocytic Syndrome With Histiocytic Glomerulopathy and Intraglomerular Hemophagocytosis.

Santoriello D1, Hogan J2, D'Agati VD3.
American journal of kidney diseases : the official journal of the National Kidney Foundation.Am J Kidney Dis.2016 Jun;67(6):978-83. doi: 10.1053/j.ajkd.2015.11.017. Epub 2016 Jan 8.
Hemophagocytic syndrome (HPS), a rare and life-threatening disease, is characterized by hyperactivation of the immune system that causes hypercytokinemia and potential multiorgan failure. Acute kidney injury is the most common kidney manifestation of HPS and is generally considered a poor prognostic
PMID 26774467

Japanese Journal

広範微小肺腫瘍塞栓によって呼吸不全を来たした肝細胞癌患者の一例

田中紘介,中舎晃男,宮崎将之,高尾信一郎,樋口野日斗,田中正剛,田中ゆき,加藤正樹,古藤和浩,髙栁涼一,相島慎一,Tanaka Kosuke,Nakasya Akio,Miyazaki Masanori,Takao Shin'ichiro,Higuchi Nobito,Tanaka Masatake,Tanaka Yuki,Kato Masaki,Koto Kazuhiro,Takayanagi Ryoichi,Aishima Shin'ichi,タナカコウスケ,ナカシャアキオ,ミヤザキマサノリ,タカオシンイチロウ,ヒグチノビト,タナカマサタケ,タナカユキ,カトウマサキ,コトウカズヒロ,タカヤナギリョウイチ,アイシマシンイチ
福岡医学雑誌 102, 298-302, 2011-10-25
… It is well known that some patients with adenocarcinoma including HCC can develop respiratory failure owing to pulmonary tumor thrombotic microangiopathy (PTTM). …
NAID 120003646789

症例剖検にて明らかとなった腫瘍塞栓性肺微小血管障害(PTTM)の1例

榎本善成,原英彦,新倉寛輝 [他]
呼吸と循環 59(10), 1043-1048, 2011-10
NAID 40019001702

Related Pictures

★リンクテーブル★

リンク元	「フォンウィルブランド因子」「血栓性微小血管症」「TMA」
関連記事	「thrombotic」

「フォンウィルブランド因子」

Thrombotic microangiopathy
	Classification and external resources
	; Micrograph of thrombotic microangiopathy with the characteristic onion-skin layering seen in older lesions. PAS stain.
ICD-10	M31.1
ICD-9	446.6

　　[★]

英: von Willebrand factor (BPT), vWF, VWF
同: フォンビルブラント因子、フォンウィルブランド因子、von Willebrand因子、vW因子？
関: 血液凝固因子

概念

血小板がコラーゲン等に結合するのに必須の補助因子

構造

ジスルフィド結合によりマルチマーを形成しており、分子量は500～20,000kDa以上に分布している。

産生組織

血管内皮、巨核球

存在部位

血漿中にあり循環。血漿、血小板、α顆粒、内皮下組織などに存在(SAN.236)

半減期

24時間

機能

コラーゲンと血小板を結合させる

血管内皮細胞が脱落しその下の細胞外マトリクスが露出されると、血漿中のフォンウィルブランド因子が細胞マトリックスに結合し、これを足場に血小板のGpIb/GpIX複合体を介して血小板が結合する

第VIII因子と結合し担体として機能。

臨床関連

フォンウィルブランド因子の機能異常

vWFのレセプター異常

ベルナール・スリエ症候群 Bernard-Soulier syndrome

「血栓性微小血管症」

　　[★]

英: thrombotic microangiopathy, TMA
同: 血栓性微小血管障害症、血栓性微小血管障害
関: [[]]

「TMA」

　　[★]

「thrombotic」

　　[★]

adj.

血栓性の、血栓の

関: thrombus n.血栓; thrombi n.(pl.)血栓

[pmid20186056-1] Benz, K.; Amann, K. (May 2010). "Thrombotic microangiopathy: new insights.". Current Opinion in Nephrology and Hypertension 19 (3): 242–7. doi:10.1097/MNH.0b013e3283378f25. PMID 20186056.

[2] ttp://www.merckmanuals.com/professional/sec12/ch143/ch143g.html

[3] ttp://www.emedicine.com/emerg/topic579.htm

匿名

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案内

案内

thrombotic microangiopathy