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a pattern of symptoms indicative of some disease
a complex of concurrent things; "every word has a syndrome of meanings"

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(疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/09/09 03:46:45」(JST)

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Kasabach–Merritt syndrome (KMS), also known as Hemangioma with thrombocytopenia^[1] is a rare disease, usually of infants, in which a vascular tumor leads to decreased platelet counts and sometimes other bleeding problems,^[2] which can be life-threatening.^[3] It is also known as hemangioma thrombocytopenia syndrome. It is named after Haig Haigouni Kasabach and Katharine Krom Merritt, the two pediatricians who first described the condition in 1940.^[4]^[5]

Pathophysiology

KMS is usually caused by a hemangioendothelioma or other vascular tumor, often present at birth.^[6]^[7] Although these tumors are relatively common, it is rare for them to cause KMS.

When these tumors are large or are growing rapidly, sometimes they can trap platelets, causing severe thrombocytopenia. The combination of vascular tumor and consumptive thrombocytopenia defines KMS. Tumors can be found in the trunk, upper and lower extremities, retroperitoneum, and in the cervical and facial areas.^[2]

This consumptive coagulopathy also uses up clotting factors, such as fibrinogen which may worsen bleeding. The coagulopathy can progress to disseminated intravascular coagulation and even death.^[2]

Hemolytic anemia secondary to microangiopathic destruction (physical damage) of the RBCs can be expressed as mild, moderate, or severe.^[8]

Diagnostic workup

The diagnostic workup^[8] is directed by the presenting signs and symptoms, and can involve:

blood counts, clotting studies, and other laboratory testing
imaging tests (ultrasound, CT scan, MRI, sometimes angiography, and rarely nuclear medicine scans)
biopsy of the tumor.

Patients uniformly show severe thrombocytopenia, low fibrinogen levels, high fibrin degradation products (due to fibrinolysis), and microangiopathic hemolysis.^[2]

Management

Management of KMS, particularly in severe cases, can be complex and require the joint effort of multiple subspecialists. This is a rare disease with no consensus treatment guidelines or large randomized controlled trials to guide therapy.

Supportive care

Patient with KMS can be extremely ill and may need intensive care. They are at risk of bleeding complications including intracranial hemorrhage. The thrombocytopenia and coagulopathy are managed with platelet transfusions and fresh frozen plasma, although caution is needed due to the risk of fluid overload and heart failure from multiple transfusions. The possibility of disseminated intravascular coagulation, a dangerous and difficult-to-manage condition, is concerning. Anticoagulant and antiplatelet medications can be used after careful assessment of the risks and benefits.^[8]

Definitive treatment

Generally, treatment of the underlying vascular tumor results in resolution of KMS. If complete surgical resection is feasible, it provides a good opportunity for cure (although it can be dangerous to operate on a vascular tumor in a patient prone to bleeding, even with appropriate surgical subspecialists involved).^[8]

If surgery is not possible, various other techniques ^[2] can be used to control the tumor:

embolization (by interventional radiology) can limit the tumor's blood supply
external compression bandages can have similar effects
certain medications, including:
- corticosteroids
- alpha-interferon
- chemotherapy (e.g. vincristine)
radiation therapy has been used, often successfully, but now is avoided whenever possible due to the risk of long-term adverse effects (e.g. risk for future cancer).

Outcomes

KMS has a mortality rate of about 30%. For patients that survive the acute disease, supportive care may be required through a gradual recovery.

Furthermore, patients may need care from a dermatologist or plastic surgeon for residual cosmetic lesions. On long-term followup, most patients have skin discoloration and/or mild disfiguration from the dormant tumor.^[9]

References

^ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology (10th ed.). Saunders. p. 597. ISBN 0-7216-2921-0.
^ ^a ^b ^c ^d ^e Hall G (2001). "Kasabach–Merritt syndrome: pathogenesis and management". Br J Haematol 112 (4): 851–62. doi:10.1046/j.1365-2141.2001.02453.x. PMID 11298580.
^ Shim W (1968). "Hemangiomas of infancy complicated by thrombocytopenia". Am J Surg 116 (6): 896–906. doi:10.1016/0002-9610(68)90462-5. PMID 4881491.
^ Kasabach HH, Merritt KK (1940). "Capillary hemangioma with extensive purpura: report of a case". Am J Dis Child 59: 1063. doi:10.1001/archpedi.1940.01990160135009.
^ Kasabach–Merritt syndrome at Who Named It?
^ Enjolras O, Wassef M, Mazoyer E, Frieden I, Rieu P, Drouet L, Taïeb A, Stalder J, Escande J (1997). "Infants with Kasabach–Merritt syndrome do not have "true" hemangiomas". J Pediatr 130 (4): 631–40. doi:10.1016/S0022-3476(97)70249-X. PMID 9108863.
^ el-Dessouky M, Azmy A, Raine P, Young D (1988). "Kasabach–Merritt syndrome". J Pediatr Surg 23 (2): 109–11. doi:10.1016/S0022-3468(88)80135-0. PMID 3278084.
^ ^a ^b ^c ^d Kasabach-Merritt Syndrome at eMedicine
^ Enjolras O, Mulliken J, Wassef M, Frieden I, Rieu P, Burrows P, Salhi A, Léauté-Labrèze C, Kozakewich H (2000). "Residual lesions after Kasabach–Merritt phenomenon in 41 patients". J Am Acad Dermatol 42 (2 Pt 1): 225–35. doi:10.1016/S0190-9622(00)90130-0. PMID 10642677.

UpToDate Contents

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1. 房状血管腫、カポジ肉腫様血管腫、およびKasabach‐Merritt現象 tufted angioma kaposiform hemangioendothelioma and the kasabach merritt phenomenon
2. 幼児血管腫の疫学、病因、臨床的特徴、および合併症 epidemiology pathogenesis clinical features and complications of infantile hemangiomas
3. 新生児血小板減少症 neonatal thrombocytopenia
4. 肝血管腫 hepatic hemangioma
5. 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

English Journal

Variable response to propranolol treatment of kaposiform hemangioendothelioma, tufted angioma, and Kasabach-Merritt phenomenon.

Chiu YE, Drolet BA, Blei F, Carcao M, Fangusaro J, Kelly ME, Krol A, Lofgren S, Mancini AJ, Metry DW, Recht M, Silverman RA, Tom WL, Pope E.SourceDepartment of Dermatology, Division of Pediatric Dermatology, Medical College of Wisconsin, Milwaukee, Wisconsin, USA. ychiu@mcw.edu
Pediatric blood & cancer.Pediatr Blood Cancer.2012 Nov;59(5):934-8. doi: 10.1002/pbc.24103. Epub 2012 Feb 2.
Propranolol is a non-selective beta-adrenergic antagonist successfully used in a case of kaposiform hemangioendothelioma (KHE) associated with Kasabach-Merritt phenomenon (KMP). We report 11 patients treated with propranolol for KHE and the related variant tufted angioma (TA), six of whom also had K
PMID 22648868

Kaposiform hemangioendothelioma in the uterine cervix of a 5-year girl.

Zhang H, Luo J, Feng X.SourceDepartment of Pathology, Beijing Fu Xing Hospital, the Capital Medical University, Beijing, China. zhk0484@sina.com.cn
Fetal and pediatric pathology.Fetal Pediatr Pathol.2012 Oct;31(5):273-7. doi: 10.3109/15513815.2012.659382. Epub 2012 Mar 20.
The occurrence of the vascular tumors in the female genital tract, particularly in the uterine cervix, is extremely rare. This kind of tumor has mainly been reported as either cavernous hemangiomas or capillary hemangiomas. To date, no Kaposiform hemangioendothelioma has been reported in this site.
PMID 22432747

Japanese Journal

新生児期にKasabach-Merritt症候群を呈した頸部血管腫の1例

福田篤久,竜田恭介,高橋由紀子,生野猛
日本小児外科学会雑誌 47(2), 246-250, 2011-04-20
… 検目的にて当科紹介入院となった.入院時,左下顎部に小児手拳大の腫瘤を認め,血液所見では血小板数1.6×10^4/μlと減少していた.腫瘤生検の結果,腫瘤はcapillary hemangiomaと判明した.血管腫に合併したKasabach-Merritt症候群(KMS)と診断し,血小板輸血に加えステロイド治療,放射線治療を行ったが効果なく,129生日に70?80%の腫瘍部分摘出術を施行した.術後,血小板減少は著明に改善した.2年経過した現在,残存血管 …
NAID 110008607768

症例放射線治療が著効したKasabach-Merritt症候群の1例

村木宏一郎,淡河恵津世,鈴木弦 [他]
臨床放射線 56(3), 405-408, 2011-03
NAID 40018750220

Related Pictures

★リンクテーブル★

リンク元	「カサバッハ・メリット症候群」
関連記事	「syndrome」

「カサバッハ・メリット症候群」

Kasabach–Merritt syndrome
Classification and external resources
Specialty	hematology
ICD-10	D69.5 (ILDS D69.507)
ICD-9-CM	287.39
OMIM	141000
DiseasesDB	30701
eMedicine	med/1221 ped/1234

　　[★]

英: Kasabach-Merritt syndrome
同: (国試)Kasabach-Merritt症候群、血管腫血小板減少症候群 thrombocytopenia-hemangioma syndrome, giant hemangioma with purpura、

概念

先天的に血管腫を生じる疾患で、巨大血管腫、血小板減少、全身性紫斑が特徴的な症候群

文献

Kasabach-Merritt 症候群

鬼田智子,七野浩之,陳基明,長野伸彦,梁尚弘,斉藤勉,麦島秀雄
日大醫學雜誌 68(6), 339-342, 2009-12-01
LINKOUT CiNii Article

小児外科疾患に対する標準的治療指針(2)血管腫とリンパ管腫

窪田昭男,川原央好,奥山宏臣 [他]
外科治療 94(3), 309-316, 2006-03
LINKOUT CiNii Article

参考

uptodate

1. [charged] 幼児血管腫の疫学、病因、臨床的特徴、および合併症 - uptodate [1]
2. [charged] 新生児血小板減少症 - uptodate [2]
3. [charged] 幼児および小児における播種性血管内凝固 - uptodate [3]
4. [charged] 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 - uptodate [4]

国試

104A034

「syndrome」

　　[★]

n.

症候群

[Andrews_p597-1] James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology (10th ed.). Saunders. p. 597. ISBN 0-7216-2921-0.

[Hall2001-2] Hall G (2001). "Kasabach–Merritt syndrome: pathogenesis and management". Br J Haematol 112 (4): 851–62. doi:10.1046/j.1365-2141.2001.02453.x. PMID 11298580.

[Shim1968-3] Shim W (1968). "Hemangiomas of infancy complicated by thrombocytopenia". Am J Surg 116 (6): 896–906. doi:10.1016/0002-9610(68)90462-5. PMID 4881491.

[4] Kasabach HH, Merritt KK (1940). "Capillary hemangioma with extensive purpura: report of a case". Am J Dis Child 59: 1063. doi:10.1001/archpedi.1940.01990160135009.

[5] Kasabach–Merritt syndrome at Who Named It?

[Enjolras1997-6] Enjolras O, Wassef M, Mazoyer E, Frieden I, Rieu P, Drouet L, Taïeb A, Stalder J, Escande J (1997). "Infants with Kasabach–Merritt syndrome do not have "true" hemangiomas". J Pediatr 130 (4): 631–40. doi:10.1016/S0022-3476(97)70249-X. PMID 9108863.

[el-Dessouky1988-7] -Dessouky M, Azmy A, Raine P, Young D (1988). "Kasabach–Merritt syndrome". J Pediatr Surg 23 (2): 109–11. doi:10.1016/S0022-3468(88)80135-0. PMID 3278084.

[EMedicine956136-8] Kasabach-Merritt Syndrome at eMedicine

[Enjolras2000-9] Enjolras O, Mulliken J, Wassef M, Frieden I, Rieu P, Burrows P, Salhi A, Léauté-Labrèze C, Kozakewich H (2000). "Residual lesions after Kasabach–Merritt phenomenon in 41 patients". J Am Acad Dermatol 42 (2 Pt 1): 225–35. doi:10.1016/S0190-9622(00)90130-0. PMID 10642677.

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Kasabach-Merritt syndrome