微小血管障害性溶血性貧血 microangiopathic hemolytic anemia, MHA
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出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2013/10/25 05:44:00」(JST)
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Microangiopathic hemolytic anemia |
Classification and external resources |
ICD-10 |
D59.4 |
ICD-9 |
283.19 |
DiseasesDB |
29721 |
In medicine (hematology) microangiopathic hemolytic anemia (MAHA) is a microangiopathic subgroup of hemolytic anemia (loss of red blood cells through destruction) caused by factors in the small blood vessels. It is identified by the finding of anemia and schistocytes on microscopy of the blood film.
Presentation[edit]
In diseases such as hemolytic uremic syndrome, disseminated intravascular coagulation, thrombotic thrombocytopenic purpura, and malignant hypertension, the endothelial layer of small vessels is damaged with resulting fibrin deposition and platelet aggregation. As red blood cells travel through these damaged vessels, they are fragmented resulting in intravascular hemolysis. The resulting schistocytes (red cell fragments) are also increasingly targeted for destruction by the reticuloendothelial system in the spleen, due to their narrow passage through obstructed vessel lumina. It is seen in systemic lupus erythematosus because the immune complex aggregates with platelets, which creates intravascular thrombi.
Automated analysers (the machines that perform routine full blood counts in most hospitals) are generally programmed to flag blood films that display red blood cell fragments or schistocytes.
Pathophysiology[edit]
In all causes, the mechanism of MAHA is the formation of a fibrin mesh due to increased activation of the system of coagulation. The red blood cells are physically cut by these protein networks, and the fragments are identical to the schistocytes seen on light microscopy.
References[edit]
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This article does not cite any references or sources. Please help improve this article by adding citations to reliable sources. Unsourced material may be challenged and removed. (November 2011) |
Pathology: hematology, hematologic diseases of RBCs and megakaryocytes / MEP (D50-69,74, 280-287)
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Red
blood cells |
↑ |
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↓ |
Anemia |
Nutritional |
- Micro-: Iron deficiency anemia
- Macro-: Megaloblastic anemia
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Hemolytic
(mostly Normo-) |
Hereditary |
- enzymopathy: G6PD
- glycolysis
- hemoglobinopathy: Thalassemia
- Sickle-cell disease/trait
- HPFH
- membrane: Hereditary spherocytosis
- Minkowski-Chauffard syndrome
- Hereditary elliptocytosis
- Southeast Asian ovalocytosis
- Hereditary stomatocytosis
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Acquired |
- Drug-induced autoimmune
- Drug-induced nonautoimmune
- Hemolytic disease of the newborn
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Aplastic
(mostly Normo-) |
- Hereditary: Fanconi anemia
- Diamond–Blackfan anemia
- Acquired: PRCA
- Sideroblastic anemia
- Myelophthisic
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Blood tests |
- MCV
- Normocytic
- Microcytic
- Macrocytic
- MCHC
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Other |
- Methemoglobinemia
- Sulfhemoglobinemia
- Reticulocytopenia
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Coagulation/
coagulopathy |
↑ |
Hyper-
coagulability |
- primary: Antithrombin III deficiency
- Protein C deficiency/Activated protein C resistance/Protein S deficiency/Factor V Leiden
- Prothrombin G20210A
- Sticky platelet syndrome
- acquired:Thrombocytosis
- DIC
- Congenital afibrinogenemia
- Purpura fulminans
- autoimmune
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↓ |
Hypo-
coagulability |
Thrombocytopenia |
- Thrombocytopenic purpura: ITP
- TM
- Heparin-induced thrombocytopenia
- May-Hegglin anomaly
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Platelet function |
- adhesion
- aggregation
- Glanzmann's thrombasthenia
- platelet storage pool deficiency
- Hermansky–Pudlak syndrome
- Gray platelet syndrome
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Clotting factor |
- Hemophilia
- von Willebrand disease
- Hypoprothrombinemia/II
- XIII
- Dysfibrinogenemia
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cell/phys (coag, heme, immu, gran), csfs
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rbmg/mogr/tumr/hist, sysi/epon, btst
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drug (B1/2/3+5+6), btst, trns
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UpToDate Contents
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English Journal
- Preparation of 1,3-bis(allyloxy)benzene under a new multi-site phase-transfer catalyst combined with ultrasonication--a kinetic study.
- Selvaraj V, Rajendran V.SourceSri Chandrasekharendra Saraswathi Viswa Maha Vidyalaya, Deemed University, Enathur, Kanchipuram, Tamil Nadu 631 561, India.
- Ultrasonics sonochemistry.Ultrason Sonochem.2013 Sep;20(5):1236-44. doi: 10.1016/j.ultsonch.2013.02.011. Epub 2013 Mar 14.
- In the present work, kinetics of synthesis of 1,3-bis(allyloxy)benzene was successfully carried out by O-allylation of resorcinol with allyl bromide using aqueous potassium hydroxide and catalyzed by a new multi-site phase-transfer catalyst viz., 1,3,5,7-tetrabenzylhexamethylenetetraammonium tetrach
- PMID 23545104
- Experimental infection of Carrion crows (Corvus corone) with two European West Nile virus (WNV) strains.
- Dridi M, Vangeluwe D, Lecollinet S, van den Berg T, Lambrecht B.SourceOperational Direction of Viral Diseases, Veterinary and Agrochemical Research Center (CODA-CERVA-VAR), 99 Groeselenberg, 1180 Brussels, Belgium. Electronic address: maha.dridi@coda-cerva.be.
- Veterinary microbiology.Vet Microbiol.2013 Jul 26;165(1-2):160-6. doi: 10.1016/j.vetmic.2012.12.043. Epub 2013 Feb 4.
- West Nile virus (WNV) has become a wide-spread arbovirus in Europe and the Mediterranean Basin countries. This emerging zoonotic disease disseminated 13 years ago in North America where its impact on animal and public health has been considerable. Although American corvids have been the most reliabl
- PMID 23434187
- Pyrolysis of cassava rhizome in a counter-rotating twin screw reactor unit.
- Sirijanusorn S, Sriprateep K, Pattiya A.SourceBio-Energy and Renewable Resources Research Laboratory, Faculty of Engineering, Mahasarakham University, Kamriang, Kantharawichai District, Maha Sarakham 44150, Thailand.
- Bioresource technology.Bioresour Technol.2013 Jul;139:343-8. doi: 10.1016/j.biortech.2013.04.024. Epub 2013 Apr 13.
- A counter-rotating twin screw reactor unit was investigated for its behaviour in the pyrolysis of cassava rhizome biomass. Several parameters such as pyrolysis temperature in the range of 500-700°C, biomass particle size of <0.6mm, the use of sand as heat transfer medium, nitrogen flow rate of 4
- PMID 23669070
Japanese Journal
- 『大乗五門実相論』について : 敦煌写本中の地論宗系『大集経』注釈書
- 仁和寺蔵『大毘盧遮那成佛経疏』巻第一 寛治七年点 釋文編(2)
Related Links
- App Store で Maha のレビューや、カスタマー評価をチェック。Maha をダウンロードして iPhone、iPad、iPod touch でお楽しみください。
- Maha is a Twitter client for your iPhone, with gorgeous interface. No unnecessary button on interface. Slide to switch between views.Simple. Lots of colors. Customize your own client. Simple and easy error handling. Never miss any tweet.
Related Pictures
★リンクテーブル★
[★]
- 英
- thrombotic thrombocytopenic purpura, TTP
- 同
- モシュコビッツ症候群 モシュコウィッツ病 Moschkowitz's syndrome Moschcowitz disease
- 関
- 溶血性尿毒症症候群 hemolytic uremic syndrome HUS、血小板減少、難病
- 同
- TTP
- ICU.594やuptodate([1] [2])も参考になった
病型
- 特発性:VWF-CP に対する自己抗体産生
- 二次性:自己免疫疾患、薬物(チクロピジン)、妊娠などによる自己抗体の出現
病因
- 1. 先天的な酵素活性の低下
- 2. vWF切断酵素に対する自己抗体
病態
- IMD.985 YN.G-83
- vWF切断酵素(ADAMTS13)活性の低下 → unusually large vWFマルチマーが分解されない → 血小板凝固促進
- 1. 血小板消費 → 出血傾向
- 2. 微小血管血小板血栓
- 2-1. 循環障害 → 腎臓(腎不全)、脳の障害(精神症状)
- 2-2. 狭窄血管の中への赤血球循環 → 破砕赤血球(MAHA) → 溶血性貧血
TTPの5徴候
- let's memorize: The Fr.(=The franch)
(⇔溶血性尿毒症症候群 HUS は血小板減少、細血管障害性溶血性貧血、腎障害。精神症状と発熱は特徴的ではない)
鑑別診断
治療
- 奏功するはっきりとしたメカニズムは不明だが、異常なvWF重合体、血小板活性化因子 PAF、あるいは免疫複合体除去、ULvWF重合体やプロスタサイクリンの置換(血漿交換で血漿内に入れる)によるものと考えられている(WCH.1559)
- 血漿輸注療法 → 先天性のTTPの場合
- 抗血小板薬
- 副腎皮質ステロイド
- 血小板輸血は原則禁忌:血小板輸血の間に腎機能や神経学的状態(neurologic status)が著明に悪化するため(WCH.1559)
USMLE
予後
- 寛解後した患者の1/3が初期再発(完全寛解後1ヶ月間)、あるいは後期再発(late relapse)(最初のエピソードから10年以内)する。
参考
- http://www.nanbyou.or.jp/sikkan/026_i.htm
[★]
- 英
- microangiopathic hemolytic anemia, MAHA, MHA
- 同
- 細血管障害性溶血性貧血, 微小血管症性溶血性貧血
- 関
- 溶血性貧血
[show details]
- さまざまな基礎疾患を背景に発生する細血管障害(microangiopathy)が原因で赤血球が破砕されて出現する溶血性貧血
原因疾患
診断
[★]
微小血管障害性溶血性貧血
- 同
- MAHA
[★]
[★]
malignancy associated hypercalcemia