microangiopathic hemolytic anemia

微小血管障害性溶血性貧血 MHA

WordNet

1. relating to or involving or causing hemolysis; "hemolytic anemia" (同)haemolytic
2. a deficiency of red blood cells (同)anaemia
3. a lack of vitality (同)anaemia
4. genus of terrestrial or lithophytic ferns having pinnatifid fronds; chiefly of tropical America (同)genus Anemia

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/07/31 15:31:01」(JST)

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"MAHA" redirects here. For the governing body formerly known as Manitoba Amateur Hockey Association, see Hockey Manitoba.

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

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 (helmet cells) 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

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

UpToDate Contents

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• 1. 溶血性尿毒症症候群（HUS）、血栓性血小板減少性紫斑病（TTP）、またはその他の血栓性微小血管障害症（TMA）が疑われる患者に対するアプローチ approach to the patient with suspected ttp hus or other thrombotic microangiopathy tma
• 2. 機械的損傷による外因性非免疫性溶血性貧血：断片化溶血および脾機能亢進 extrinsic nonimmune hemolytic anemia due to mechanical damage fragmentation hemolysis and hypersplenism
• 3. 後天性TTP：臨床症状および診断 acquired ttp clinical manifestations and diagnosis
• 4. 血栓性微小血管障害症 drug induced thrombotic microangiopathy
• 5. 成人における全身性エリテマトーデスの血液学的異常 hematologic manifestations of systemic lupus erythematosus in adults

English Journal

• Venous thromboembolism related to warm autoimmune hemolytic anemia: A case-control study.

• Lecouffe-Desprets M1, Néel A2, Graveleau J1, Leux C3, Perrin F4, Visomblain B5, Artifoni M4, Masseau A1, Connault J1, Pottier P4, Agard C4, Hamidou M4.
• Autoimmunity reviews.Autoimmun Rev.2015 Nov;14(11):1023-8. doi: 10.1016/j.autrev.2015.07.001. Epub 2015 Jul 8.
• BACKGROUND: The risk of venous thromboembolism (VTE) during warm autoimmune hemolytic anemia (wAIHA) is apparent in several published series. Unlike proximate disorders (autoimmune thrombocytopenia, non-immune hemolytic diseases) little is known about the presentation and risk factors for VTE in thi
• PMID 26162301

• Paroxysmal cold hemoglobinuria due to an IgA donath-landsteiner antibody.

• Whipple NS1, Moreau DA2, Moulds JM3, Hankins JS1, Wang WC1, Nottage KA1.
• Pediatric blood & cancer.Pediatr Blood Cancer.2015 Nov;62(11):2044-6. doi: 10.1002/pbc.25591. Epub 2015 Jun 5.
• Paroxysmal cold hemoglobinuria (PCH) is an autoimmune hemolytic anemia (AIHA) characterized by the presence of a Donath-Landsteiner (D-L) antibody. PCH occurs most commonly in young children and is associated with acute, often self-limited hemolytic anemia. The D-L antibody is classically a biphasic
• PMID 26053459

• Novel Gardos channel mutations linked to dehydrated hereditary stomatocytosis (xerocytosis).

• Andolfo I1,2, Russo R1,2, Manna F1,2, Shmukler BE3,4, Gambale A1,2, Vitiello G2,5, De Rosa G1,2, Brugnara C6, Alper SL3, Snyder LM7,8, Iolascon A1,2.
• American journal of hematology.Am J Hematol.2015 Oct;90(10):921-6. doi: 10.1002/ajh.24117.
• Dehydrated hereditary stomatocytosis (DHSt) is an autosomal dominant congenital hemolytic anemia with moderate splenomegaly and often compensated hemolysis. Affected red cells are characterized by a nonspecific cation leak of the red cell membrane, reflected in elevated sodium content, decreased pot
• PMID 26178367

Japanese Journal

• Clinical features of severe acquired ADAMTS13 deficiency in thrombotic thrombocytopenic purpura : the Korean TTP registry experience

• JANG Moon Ju,CHONG So Young,KIM In-Ho,KIM Jee-Hyun,JUNG Chul-Won,KIM Ja Young,PARK Ji-Chan,LEE Sun Min,KIM Yeo-Kyeoung,LEE Ji-Eun,JANG Sung-Su,KIM Jin-Seok,JO Deog-Yeon,ZANG Dae-Young,LEE Young-Yiul,YHIM Ho-Young,OH Doyeun
• International journal of hematology 93(2), 163-169, 2011-02-01
• NAID 10029533023

• 播種性骨髄癌腫症を呈した前立腺癌の2例

• 加藤 琢磨,山本 議仁,松岡 祐貴,桑田 善弘,田岡 輝久,香月 奈穂美,串田 吉生,筧 善行
• 日本泌尿器科學會雜誌 102(1), 28-33, 2011-01-20
• … 播種性骨髄癌腫症は固形癌のびまん性骨転移によって播種性血管内凝固症候群(disseminated intravascular coagulation;DIC),白赤芽球症(leukoerythroblastosis),最小血管障害性溶血性貧血(microangiopathic hemolytic anemia;MHA)を呈し,急激に予後不良な経過をたどる病態である.本症を併発した前立腺癌の2例を経験した.前立腺癌による播種性骨髄癌腫症の報告は稀であり,若干の文献的考察を加え報告する.症例 …
• NAID 110008440745

Related Links

• Microangiopathic hemolytic anemia - Wikipedia, the free encyclopedia

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 ...

• Microangiopathic hemolytic anemia - Pathology Student

29 Jul 2009 ... We've also talked about immune-related hemolytic anemia (warm and cold), which is an acquired hemolytic anemia. The last main type of hemolytic anemia on our list is microangiopathic hemolytic anemia, or MAHA for short, ...

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★リンクテーブル★

リンク元	「血栓性血小板減少性紫斑病」「微小血管障害性溶血性貧血」「MHA」「MAHA」
関連記事	「microangiopathic」「hemolytic」

「血栓性血小板減少性紫斑病」

　　[★]

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

• 先天性：VWF-CPの遺伝子異常
• 後天性

• 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

• vWF切断酵素(ADAMTS13)活性の低下

• 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

• 発熱：組織崩壊、溶血、発熱中枢障害による

TTPの5徴候

let's memorize: The Fr.(=The franch)

• 血小板減少 thrombocytopenia
• 細血管障害性溶血性貧血（microangiopathic hemolytic anemia MAHA）
• 動揺する精神神経症状 encephalopathy
• 発熱 fever
• 腎障害 renal dysfunction

(⇔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

• 血小板減少：膠原病(SLEなど)、抗リン脂質抗体症候群、特発性血小板減少症 ITP、肝硬変、骨髄異形成症候群 MDS、播種性血管内凝固症候群(DIC)

治療

• 血漿交換療法

• 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

• Q book p.290 14

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

• 難病情報センター

http://www.nanbyou.or.jp/sikkan/026_i.htm

「微小血管障害性溶血性貧血」

　　[★]

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]

微小血管障害性溶血性貧血 : 約 14,800 件
微小血管症性溶血性貧血 : 約 10,700 件
細血管障害性溶血性貧血 : 約 6,930 件

• 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

• 血栓性血小板減少性紫斑病(TTP)
• 溶血性尿毒症症候群(HUS)
• 播種性血管内凝固症候群(DIC)
• 癌など

診断

• 塗抹標本による種々の奇形赤血球の検出(分裂赤血球など)

「MHA」

　　[★]

• 微小血管障害性溶血性貧血、微小血管症性溶血性貧血 microangiopathic hemolytic anemia
• メチル馬尿酸 methylhippuric acid

「MAHA」

　　[★] 微小血管障害性溶血性貧血 microangiopathic hemolytic anemia, MHA

「microangiopathic」

　　[★]

• adj.

• 微小血管症の、微小血管障害性の、細小血管症の

HELLP syndrome refers to a syndrome characterized by hemolysis with a microangiopathic blood smear, elevated liver enzymes, and a low platelet count.(uptodate)

関

microangiopathy

「hemolytic」

　　[★]

• adj.

• 溶血性の、溶血の

関

haemolysis、haemolytic、hemolysis、hemolyze