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]

  Search Search for a rare disease Kasabach-Merritt syndrome Disease definition Kasabach-Merritt syndrome (KMS), also known as hemangioma-thrombocytopenia syndrome, is a rare disorder characterized by profound thrombocytopenia, microangiopathic hemolytic anemia [orpha.net]

• Ecchymosis

  The medicolegal examination concluded that the appearance of the lesion was only mildly suggestive of an ecchymosis. A second, temporally remote examination confirmed this doubt. [ncbi.nlm.nih.gov]

• Hunting

  [Medline]. de la Hunt MN. Kasabach-Merritt syndrome: dangers of interferon and successful treatment with pentoxifylline. J Pediatr Surg. 2006 Jan. 41(1):e29-31. [Medline]. Haisley-Royster C, Enjolras O, Frieden IJ, et al. [emedicine.medscape.com]

• 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]

• Aspiration

  In such patients, operative management of long bone hemangiomatous lesions, including percutaneous needle aspirations, may be contraindicated, and nonoperative management may be preferable. [ncbi.nlm.nih.gov]

  Biopsy of the scalp lesion was deemed too risky and fine needle aspiration of the liver was nondiagnostic. [pagepress.org]

  Thus, it is possible that KHE and tufted angiomas are the part of the same neoplastic spectrum and histologic continuum. [3], [4] The trigger factors for the development of KMS include surgical intervention, pregnancy, angiography, and needle aspiration [jcnonweb.com]

• Inspiratory Stridor

  However, the mass continued to grow, resulting in inspiratory stridor. Magnetic resonance imaging revealed encroachment into the thecal sac and compression of the spinal cord. The lesion was embolized, and vincristine therapy was commenced. [ncbi.nlm.nih.gov]

• Petechiae

  Kasabach-Merritt syndrome (KMS) consists of large and rapidly growing vascular tumors associated with thrombocytopenia, generalized petechiae, and bleeding. [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]

  If the baby’s blood platelet count is low, we may see multiple tiny red spots on the skin (petechiae). At Mount Sinai, we can usually diagnose KHE visually and with bloodwork. [mountsinai.org]

  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]

  CASE REPORT A 2-month-old male infant presented with a skin lesion on his left pubis 2 weeks after birth. He had no complications during birth. [synapse.koreamed.org]

• 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]

• Motor Disturbances

  Spastic diplegia and other motor disturbances in infants receiving interferon-alpha. Laryngoscope. 2004 Jul. 114(7):1231-6. [Medline]. de la Hunt MN. Kasabach-Merritt syndrome: dangers of interferon and successful treatment with pentoxifylline. [emedicine.medscape.com]

  Michaud AP, Bauman NM, Burke DK, Manaligod JM, Smith RJ: Spastic diplegia and other motor disturbances in Infants receiving interferon-alpha. Laryngoscope. 2004, 114: 1231-1236. 10.1097/00005537-200407000-00017. 9. [bmcpediatr.biomedcentral.com]

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.

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.
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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.