120:
spontaneous bleeding can also occur. The tumors are not hemangiomas. They usually present in young infants, less than three months of age, but have also been reported in the toddler age group. These tumors occur in the extremities, chest, neck, abdomen and pelvis. They infiltrate across tissue and can be aggravated by interventions, infection and trauma. When the tumors associated with KMP are internal such as in the chest or abdomen, they can cause significant illness and can be life-threatening due to bleeding. Internal lesions can take a longer time to diagnose.
67:
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48:
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Initially a vascular lesion is usually noted on the skin which can be firm and hard (indurated). Areas of tiny red dots (petechiae) can appear around the lesion or on other parts of the body. If the vascular lesion is internal, these petechiae and bruising can be seen on the skin. Bruising and
848:
832:
177:. The combination of vascular tumor and consumptive thrombocytopenia defines Kasabach–Merritt syndrome. Tumors can be found in the trunk, upper and lower extremities, retroperitoneum, and in the cervical and facial areas.
273:
Management of
Kasabach–Merritt syndrome, 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
392:
Kasabach–Merritt syndrome 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
326:
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).
778:
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".
590:
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".
405:
for head & neck/airway involvement. On long-term followup, most patients have skin discoloration and/or mild disfiguration from the dormant tumor.
971:
381:
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).
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or other vascular tumor, often present at birth. Although these tumors are relatively common, they only rarely cause
Kasabach–Merritt syndrome.
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193:
443:
863:
262:
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Larsen, EC; Zinkham, WH; Eggleston, JC; Zitelli, BJ (June 1987). "Kasabach-Merritt syndrome: therapeutic considerations".
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Generally, treatment of the underlying vascular tumor results in resolution of
Kasabach–Merritt syndrome. If complete
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347:
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66:
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Kasabach HH, Merritt KK (1940). "Capillary hemangioma with extensive purpura: report of a case".
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and antiplatelet medications can be used after careful assessment of the risks and benefits.
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The diagnostic workup is directed by the presenting signs and symptoms, and can involve:
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destruction (physical damage) of the RBCs can be expressed as mild, moderate, or severe.
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If surgery is not possible, various other techniques can be used to control the tumor:
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294:. The thrombocytopenia and coagulopathy are managed with platelet transfusions and
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el-Dessouky M, Azmy A, Raine P, Young D (1988). "Kasabach–Merritt syndrome".
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When these tumors are large or are growing rapidly, sometimes they can trap
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532:
934:
511:
Shim W (1968). "Hemangiomas of infancy complicated by thrombocytopenia".
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Hall G (2001). "Kasabach–Merritt syndrome: pathogenesis and management".
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Patient with
Kasabach–Merritt syndrome can be extremely ill and may need
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111:, the two pediatricians who first described the condition in 1940.
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310:, a dangerous and difficult-to-manage condition, is concerning.
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which may worsen bleeding. The coagulopathy can progress to
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blood counts, clotting studies, and other laboratory testing
290:. They are at risk of bleeding complications including
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of the tumor is contraindicated due to risk of bleeding.
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434:
James, William; Berger, Timothy; Elston, Dirk (2005).
103:, which can be life-threatening. It is also known as
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Patients uniformly show severe thrombocytopenia, low
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37:
32:
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162:Kasabach–Merritt syndrome is usually caused by a
306:from multiple transfusions. The possibility of
298:, although caution is needed due to the risk of
107:. It is named after Haig Haigouni Kasabach and
95:, in which a vascular tumor leads to decreased
8:
739:"Kasabach - Merritt syndrome: A case report"
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463:
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65:
46:
29:
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438:(10th ed.). Saunders. p. 597.
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308:disseminated intravascular coagulation
194:disseminated intravascular coagulation
7:
560:10.1001/archpedi.1940.01990160135009
401:for residual cosmetic lesions or an
343:) can limit the tumor's blood supply
105:hemangioma thrombocytopenia syndrome
41:Hemangioma-thrombocytopenia syndrome
25:
482:10.1046/j.1365-2141.2001.02453.x
353:certain medications, including:
126:
85:hemangioma with thrombocytopenia
972:Dermal and subcutaneous growths
743:Sudanese Journal of Paediatrics
1:
792:10.1016/S0190-9622(00)90130-0
649:10.1016/S0022-3468(88)80135-0
604:10.1016/S0022-3476(97)70249-X
525:10.1016/0002-9610(68)90462-5
415:List of cutaneous conditions
276:randomized controlled trials
255:fibrin degradation products
99:counts and sometimes other
988:
263:microangiopathic hemolysis
678:Kasabach-Merritt Syndrome
574:Kasabach–Merritt syndrome
77:Kasabach–Merritt syndrome
45:
33:Kasabach–Merritt syndrome
18:Kasabach-Merritt syndrome
350:can have similar effects
341:interventional radiology
182:consumptive coagulopathy
292:intracranial hemorrhage
135:This section is empty.
109:Katharine Krom Merritt
708:10.1542/peds.79.6.971
348:compression bandages
318:Definitive treatment
164:hemangioendothelioma
296:fresh frozen plasma
912:External resources
786:(2 Pt 1): 225–35.
780:J Am Acad Dermatol
737:Osman, NM (2013).
324:surgical resection
278:to guide therapy.
115:Signs and symptoms
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445:978-0-7216-2921-6
379:radiation therapy
173:, causing severe
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101:bleeding problems
83:), also known as
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27:Medical condition
16:(Redirected from
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403:otolaryngologist
362:alpha-interferon
237:nuclear medicine
202:microangiopathic
198:Hemolytic anemia
196:and even death.
186:clotting factors
175:thrombocytopenia
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137:You can help by
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399:plastic surgeon
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357:corticosteroids
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282:Supportive care
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219:imaging tests (
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158:Pathophysiology
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23:
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15:
12:
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5:
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967:Rare syndromes
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962:Coagulopathies
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819:Classification
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811:External links
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637:J Pediatr Surg
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548:Am J Dis Child
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519:(6): 896–906.
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598:(4): 631–40.
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579:Who Named It?
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470:Br J Haematol
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312:Anticoagulant
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146:November 2021
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749:(1): 49–52.
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367:chemotherapy
337:embolization
329:
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259:fibrinolysis
248:
231:, sometimes
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143:
139:adding to it
134:
118:
104:
89:rare disease
84:
80:
76:
75:
554:(5): 1063.
371:vincristine
233:angiography
38:Other names
956:Categories
886:DiseasesDB
696:Pediatrics
613:2066/26075
421:References
269:Management
251:fibrinogen
221:ultrasound
190:fibrinogen
188:, such as
61:Hematology
921:eMedicine
897:SNOMED CT
840:: D69.5 (
683:eMedicine
592:J Pediatr
513:Am J Surg
388:Prognosis
346:external
208:Diagnosis
171:platelets
56:Specialty
935:Orphanet
929:ped/1234
926:med/1221
902:86635005
844:D69.507)
800:10642677
765:27493358
724:19678178
498:28677474
490:11298580
409:See also
257:(due to
97:platelet
880:D059885
756:4949964
716:3108848
657:3278084
622:9108863
533:4881491
261:), and
225:CT scan
93:infants
87:, is a
869:141000
858:287.39
798:
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655:
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488:
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369:(e.g.
243:Biopsy
239:scans)
63:
891:30701
720:S2CID
494:S2CID
180:This
940:2330
875:MeSH
864:OMIM
853:9-CM
842:ILDS
796:PMID
761:PMID
712:PMID
653:PMID
618:PMID
529:PMID
486:PMID
440:ISBN
339:(by
302:and
849:ICD
833:ICD
788:doi
751:PMC
704:doi
681:at
645:doi
608:hdl
600:doi
596:130
577:at
556:doi
521:doi
517:116
478:doi
474:112
397:or
229:MRI
141:.
81:KMS
958::
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900::
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794:.
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665:^
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144:(
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