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Kasabach Merritt Syndrome

Kasabach Merrit Syndrome

Kasabach Merritt syndrome is a rare disease consisting of a vascular tumor (kaposiform hemangioendothelioma, tufted or congenital angioma), thrombocytopenia and consumptive coagulopathy (hypofibrinogenemia). When microangiopathic hemolytic anemia is also present, the condition is called Kasabach Merritt phenomenon. This pathology has a lethal potential, due to its natural evolution to disseminated intravascular coagulopathy.


Presentation

Patients with Kasabach Merritt syndrome may present for the evaluation of cutaneous lesions [1], for symptoms caused by visceral vascular tumors or for milder dermal manifestations of the disease [2], like petechiae, bruising [3] or bleeding. With hepatic angiomas, patients can have hepatomegaly and jaundice. If the vascular tumor is large, a great amount of blood circulates within it, causing symptomatic high output heart failure [4]. Large lesions may also cause compression of the neighboring structures, with various signs, depending on their location. Death can be due to cerebral bleeding from an intracranial pathology [5], or secondary to disseminated intravascular coagulation, multiorgan failure, shock, and sepsis. Hemangiomas may be located anywhere: the skin, retroperitoneal organs, mediastinum, pelvis, neck, limbs, musculoskeletal, visceral organs. Symptoms may become worse as the child grows older and subsequently, the vascular tumor grows larger. However, patients may remain asymptomatic until they become adults or may present during the first weeks of life.

When no cutaneous lesions are present, the diagnosis is easily missed unless a thorough examination that suggests liver or spleen enlargement, for instance, is performed [6]. Still, clinical examination may be uninformative in some patients, like those with intraosseous disease [7] [8], therefore the diagnosis relies on a high level of suspicion, laboratory and imaging data.

Anemia
  • Doppler examination showed elevated peak systolic velocity in the middle cerebral artery, indicating acute fetal anemia.[ncbi.nlm.nih.gov]
  • Excision biopsy of the spleen led to resolution of anemia, thrombocytopenia, and coagulopathy. The diagnosis of SH was confirmed by histopathology.[ncbi.nlm.nih.gov]
  • Laboratory examinations revealed thrombocytopenia anemia and elevated fibrin degradation products. There were no signs of sepsis. Blood transfusion and steroid therapy were executed.[ncbi.nlm.nih.gov]
  • Abstract We report a 55-year-old white woman with an angiosarcoma of the left breast, anemia and thromobocytopenia due to a consumptive coagulopathy (Kasabach-Merritt syndrome).[ncbi.nlm.nih.gov]
  • Werner Cambridge University Press, 18 Aug 2005 - Medical - 452 pages Neonatal hematology : a historical overview -- Disorders of the fetomaternal unit -- Erythropoiesis, red cells, and the approach to anemia -- Anemia of prematurity and indications for[books.google.com]
Ecchymosis
  • However, despite hematologic correction, the ecchymosis and petechiae worsened, and a mass became evident in the right posterior triangle of the patient's neck.[ncbi.nlm.nih.gov]
  • We report a two- and- half month old Saudi female infant who presented with epistaxis, conjunctival haemorrage and bilateral periorbital ecchymosis.[ncbi.nlm.nih.gov]
  • We report the case of a one-month male infant who presented with a large cutaneous tumor in his right axilla with ecchymosis, thrombocytopenia, and chronic consumption coagulopathy.[ncbi.nlm.nih.gov]
  • They deserve to be widely known because they mandate rapid medical management and because they are one of the only differential diagnoses of ecchymosis, especially in children.[ncbi.nlm.nih.gov]
  • The patient was a 60-year-old woman who complained of hematochezia, ecchymosis, and abdominal distension. She refused all surgical management and was therefore treated with systemic glucocorticoids and beta-blockers.[ncbi.nlm.nih.gov]
Prolonged Immobilization
  • A number of events such as sclerotherapy, surgery, bone fracture, prolonged immobilization and pregnancy or menstruation triggered conversion of the LIC to disseminated intravascular coagulation (DIC), with bleeding related to factor consumption and multiorgan[ncbi.nlm.nih.gov]
Hemoptysis
  • It was detected after hemoptysis. We also present our case observations and management.[ncbi.nlm.nih.gov]
Cardiomegaly
  • Acute enlargement of the hemangiomas and the appearance of new retroperitoneal hemangiomas were detected at 27 weeks, along with skin edema and cardiomegaly.[ncbi.nlm.nih.gov]
Petechiae
  • Abstract Kasabach-Merritt syndrome (KMS) consists of large and rapidly growing vascular tumors associated with thrombocytopenia, generalized petechiae, and bleeding.[ncbi.nlm.nih.gov]
  • However, despite hematologic correction, the ecchymosis and petechiae worsened, and a mass became evident in the right posterior triangle of the patient's neck.[ncbi.nlm.nih.gov]
  • O/e: Visible cutaneous giant hemangioma or multiple smaller hemangiomas, usually on the extremities, Petechiae, bruising, and frank bleeding, Hepatomegaly, enlarged abdomen is seen.[dailyrounds.org]
  • Patients with Kasabach Merritt syndrome may present for the evaluation of cutaneous lesions, for symptoms caused by visceral vascular tumors or for milder dermal manifestations of the disease, like petechiae, bruising or bleeding.[symptoma.com]
  • Upon evaluation, she was noted to have scattered petechiae and a purplish right forehead scalp swelling that she reported had been growing over the previous several months and was initially thought to be a hematoma. She otherwise appeared healthy.[pagepress.org]
Skin Lesion
  • However, the platelet count remained low and the skin lesion enlarged gradually. Trans-feeding-arterial embolotherapy with a 5 ml pure ethanol (1 ml/kg) injection was performed at four months of age.[ncbi.nlm.nih.gov]
  • Skin Lesions: A deep vascular malformation, characterized by soft compressible deep-tissue usually red- purple or blue swelling or tumor. 2. Variants of CH a.[web.sapmed.ac.jp]
Skin Edema
  • Acute enlargement of the hemangiomas and the appearance of new retroperitoneal hemangiomas were detected at 27 weeks, along with skin edema and cardiomegaly.[ncbi.nlm.nih.gov]
Flank Pain
  • CASE: A 23-year-old woman, gravida 2, para 0101, with a prenatal diagnosis of Gorham syndrome from her previous pregnancy was admitted at 32 weeks' gestation with severe back and flank pain.[ncbi.nlm.nih.gov]

Workup

Clinical examination may reveal pallor, reddish-brown, indurated lesions [9], classical capillary hemangiomas or tufted angiomas. Patients may be tachycardic due to the anemia, heart failure or shock. Lesion ulceration and infection are rarely seen.

In Kasabach Merritt syndrome, blood workup should include complete blood count, peripheral smear, fibrinogen, fibrin degradation products, D-dimers, prothrombin time and activated partial thromboplastin time. The laboratory personnel should look for Burr cells and schistocytes. Disseminated intravascular coagulation is accompanied by prolonged prothrombin time and activated partial thromboplastin time, low fibrinogen level, elevated fibrin degradation product and D-dimer levels. Intravascular coagulation may also have a chronic, low-grade character.

It is important to determine the extent of cutaneous lesions and the existence of other involved sites, therefore radiography, ultrasonography- especially Doppler flow, computed tomography, Indium or Chromium radionuclide scintigraphy [10] magnetic resonance imaging scans and angiographic scans should be performed. Angiography can be followed by embolization. When the nature of the tumor is uncertain, histologic findings are important to evaluate its nature [11], but biopsies are usually not performed due to the critical state of the patient unless surgical procedures are performed with curative intent. Most frequently, kaposiform haemangioendothelioma and tufted angiomas are found [12]. Both contain dilated capillary vessels with endothelial lesions, microthrombi, lymphlike vessels and hemosiderin deposits, and both may be found in the same patient.

Burr Cell
  • The laboratory personnel should look for Burr cells and schistocytes.[symptoma.com]

Treatment

  • BACKGROUND: The purpose of the present study was to evalutate the various treatment modalities for Kasabach-Merritt syndrome (KMS) and to identify the most reliable treatment modalities.[ncbi.nlm.nih.gov]
  • The mean platelet count before treatment was 18,000/L (range, 8,000 to 33,000/L).[ncbi.nlm.nih.gov]
  • Two weeks corticosteroid treatment was ineffective, and deterioration of coagulation parameters and compromise of the airway and left vision due to huge hemangioma prompted use of endovascular treatment.[ncbi.nlm.nih.gov]
  • OBJECTIVE AND CONCLUSION: The objective of this article is to provide a comprehensive review of KMS and give an up-to-date summary of treatment options.[ncbi.nlm.nih.gov]
  • The treatment of the two conditions is very different.[ncbi.nlm.nih.gov]

Prognosis

  • Treatment and prognosis Various treatments are used, with varying degrees of success, in Kasabach-Merritt syndrome - systemic corticosteroids, radiation therapy, compression, embolization and/or pharmacotherapy (e.g. interferon, platelet aggregation inhibitors[radiopaedia.org]
  • It has been known that the prognosis was very poor and the splenectomy before rupture could increase survival.[e-crt.org]
  • […] lymphedema after breast surgery (Stewart-Treves syndrome), 5 and most recently a possible association with silicone breast implants has also been described. 6 , 7 Angiosarcomas exhibit a high propensity for metastatic multifocal disease and carry a poor prognosis[pagepress.org]
  • The therapeutic management should be early, multidisciplinary adapting therapeutic benefits to risks in order to improve the prognosis.[ijias.issr-journals.org]
  • Prognosis When KMS is promptly recognized and properly treated, the prognosis is usually excellent because the DIC resolves as the vascular lesion recedes and because KMS does not recur. Therefore, most children do well if they reach age 2 years.[emedicine.medscape.com]

Etiology

  • Author information 1 Department of Radiology, Innsbruck Medical University, Anichstrasse 35, 6020 Innsbruck, Austria. maria.bernathova@uibk.ac.at Abstract Primary angiosarcoma of the breast is a rare aggressive tumour of unknown etiology.[ncbi.nlm.nih.gov]
  • Abstract Since diffuse hepatic hemangiomatosis (DHH) is an extremely rare disease especially in adults, the etiology and natural course of adult-onset DHH has not been well understood.[ncbi.nlm.nih.gov]
  • Etiology Kasabach-Merritt syndrome is usually caused by kaposiform hemangioendothelioma or tufted angioma. Other vascular tumors may be responsible, such as infantile hemangioma. The radiographic features are specific to each vascular tumor.[radiopaedia.org]
  • Indian J Dermatol 2 Clinical observations in mesh suture treatment for infants of Kasabach-Merritt phenomenon Kelei Li,Maozhong Tai,Zhongping Qin,Chunxiao Ge Journal of Paediatrics and Child Health. 2014; : n/a 3 Neonatal thrombocytopenia: Etiology and[e-ijd.org]
  • Etiology: a. A striking predilection for the skin of the head. b. Most patients are in the seventh to eighth decade 2.[web.sapmed.ac.jp]

Epidemiology

  • […] multivariate analysis indicated that in patients with kaposiform hemangioendothelioma, risk factors for the presence of Kasabach-Merritt syndrome (KMS) include patient age under 6 months, a lesion size of over 5.0 cm, and the existence of a mixed lesion. [10] Epidemiology[emedicine.medscape.com]
  • Epidemiology; Epidemic or AIDS-associated. 2. Clinical Lesions: A multi-centric systemic vascular tumor characterized by violaceous nodules and by edema secondary to lymphatic obstruction. 3. Clinical Variants of KS a. Classic KS b.[web.sapmed.ac.jp]
Sex distribution
Age distribution

Pathophysiology

  • The clinical presentation, laboratory findings, vascular pathology, and pathophysiology will also be discussed.[ncbi.nlm.nih.gov]
  • This case study discusses the pathophysiology, clinical manifestations, diagnostics, treatment modalities, differential diagnosis, and psychosocial considerations of KMS.[ncbi.nlm.nih.gov]
  • Abstract Clinical studies have provided ample evidence that high (either systemic or local) levels of vascular endothelial growth factor (VEGF) are associated with several pathophysiological disorders, including hemangiomas.[ncbi.nlm.nih.gov]
  • The pathophysiology is believed to be exposure of subendothelial elements or abnormal endothelium within the haemangioma resulting in aggregation and activation of platelets with a secondary consumption of clotting factors ( 2 ) and formation of intralesional[clinicaldermatology.eu]

Prevention

  • Author information 1 Department of Gastroenterology, National Hospital Organization Kanazawa Medical Center, 1-1 Shimoishibikicho, Kanazawa, Ishikawa, 920-8650, Japan. y.s.0831@gmail.com. 2 System Biology, Graduate School of Advanced Preventive Medical[ncbi.nlm.nih.gov]
  • Vincristine chemotherapy after the operation was necessary to prevent the enlargement of the remaining tumor. Two cases which were ineffectively treated by other means were treated in this way.[ncbi.nlm.nih.gov]
  • Vincristine chemotherapy after operation is necessary to prevent the enlargement of the remaining tumor.[ncbi.nlm.nih.gov]
  • CONCLUSION: In a case of angiomatosis with an associated pulmonary lobar vascular malformation, lobectomy can be safely performed to prevent life-threatening bleeding.[ncbi.nlm.nih.gov]
  • Skin biopsy or liver biopsy were avoided to prevent torrential bleed.[journalijar.com]

References

Article

  1. Arunachalam P, Kumar VR, Swathi D. Kasabach-Merritt syndrome with large cutaneous vascular tumors. J Indian Assoc Pediatr Surg. 2012;17(1):33-6
  2. Sidbury R. Update on vascular tumors of infancy. Curr Opin Pediatr. 2010;22:432–437.
  3. Tang JY, Chen J, Pan C, et al. Diffuse cavernous hemangioma of the spleen with Kasabach-Merritt syndrome misdiagnosed as idiopathic thrombocytopenia in a child. World J Pediatr. 2008;4(3):227-230.
  4. Enjolras O, Riche MC, Merland JJ, et al. Management of alarming hemangiomas in infancy: a review of 25 cases. Pediatrics. 1990;85(4):491-498.
  5. Maguiness S, Guenther L. Kasabach-Merritt syndrome. J Cutan Med Surg. 2002;6:335–9.
  6. Von Schweinitz D, Gluer S, Mildenberger H. Liver tumors in neonates and very young infants: diagnostic pitfalls and therapeutic problems. Eur J Ped Surg. 1995; 5:72–76.
  7. Biswal BM, Anand AK, Aggarwal HN, et al. Vertebral haemangioma presenting as Kasabach–Merritt syndrome. Clinical Oncology. 1993;5:187–188.
  8. Carrington PR, Rowley MJ, Fowler M, et al. Kasabach–Merritt syndrome with bone involvement: the pseudomalignant sign of Gorham. J Am Acad Dermatol. 1993; 29:117–119.
  9. Mitsuhashi N, Furuta M, Sakurai H, et al. Outcome of radiation therapy for patients with Kasabach-Merritt syndrome. Int J Radiat Oncol Biol Phys. 1997; 39(2):467-473.
  10. Pampin C, Devillers A, Treguier C, et al. Intratumoral consumption of indium-111-labeled platelets in a child with splenic hemangioma and thrombocytopenia. J Pediatr Hematol Oncol. 2000;22(3):256-258.
  11. Mueller BU, Mulliken JB. The infant with a vascular tumor. Seminars in Perinatology. 1999;23:332–340.
  12. Sarkar M, Mulliken JB, Kozakewich HP, et al. Thrombocytopenic coagulopathy (Kasabach–Merritt phenomenon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma. Plast Reconstruct Surg. 1997;100:1377–1386.

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Last updated: 2018-06-21 21:35