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.
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. 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 . 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 , 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 . Still, clinical examination may be uninformative in some patients, like those with intraosseous disease  , therefore the diagnosis relies on a high level of suspicion, laboratory and imaging data.
Entire Body System
Hemolytic anemia Increased hemolysis, Haemolytic anaemia A type of anemia caused by premature destruction of red blood cells (hemolysis). [rarediseases.oscar.ncsu.edu]
anemia and consumption coagulopathy as a result of platelet and red blood cell trapping and activation of clotting system within the vasculature of hemangioma. [ajol.info]
Doppler examination showed elevated peak systolic velocity in the middle cerebral artery, indicating acute fetal anemia. [ncbi.nlm.nih.gov]
At a glance It is characterized by an association of vascular tumors with thrombocytopenia, hypofibrinogenemia, anemia, and coagulopathy. [accessanesthesiology.mhmedical.com]
Extensive bilateral periorbital ecchymosis was documented with bilateral subconjunctival haemorrhage with no discharge (Figure 1B). She did not have purpuric rash, bruises or ecchymosis on her skin or mucus membranes. [ncbi.nlm.nih.gov]
- Intravenous Administration
Intravenous administration of interleukin-2 (350,000 U daily) was initiated, but the tumour enlarged rapidly (Fig. 2). In accordance with the tumour growth, sudden thrombocytopaenia (72,000/mm3) was observed. [medicaljournals.se]
In sclerosing embolization, attention must be paid to prevent the regurgitation of the embolization agent, which may cause ectopic embolism and other severe unexpected complications. [frontiersin.org]
The infant was readmitted to our hospital at 90-days-old with repeatedly regurgitating milk associated with cough for 10 days, as well as reduced activity and poor response for 2 days. [spandidos-publications.com]
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]
Some of the signs and symptoms include: Visible cutaneous giant hemangioma or multiple smaller hemangiomas, usually on the extremities Enlarged abdomen Hepatomegaly Petechiae, bruising, and bleeding Painful lesions Anemia What are the treatment options [nicklauschildrens.org]
Kasabach-Merritt syndrome (KMS) consists of large and rapidly growing vascular tumors associated with thrombocytopenia, generalized petechiae, and bleeding. [ncbi.nlm.nih.gov]
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 spontaneous bleeding can also occur. [en.wikipedia.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]
Both lesions may be associated with overlying hypertrichosis or hyperhidrosis. [statpearls.com]
Clinical examination may reveal pallor, reddish-brown, indurated lesions , 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  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 , 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 . Both contain dilated capillary vessels with endothelial lesions, microthrombi, lymphlike vessels and hemosiderin deposits, and both may be found in the same patient.
Successful treatment of kasabach-merritt syndrome with vincristine and surgery: a case report and review of literature. Cases J, 2008;1;9. Jiang, RS, Hu, R. [bloodresearch.or.kr]
might also determine responsiveness to treatment. [jmedicalcasereports.biomedcentral.com]
Our patient was refractory to medical treatment with prednisone and vincristine, requiring surgical treatment, without complications or tumor recurrence up to 6 months after the intervention. [heighpubs.org]
Treatment Options We treat KHE and Kasabach-Merritt Syndrome with steroids to stop the tumors from forming new blood vessels (angiogenesis). We usually prescribe a pill. If pills don’t work, we try intravenous treatment. [mountsinai.org]
Historically steroids are typically first-line treatment. [statpearls.com]
Treatment and prognosis Various treatments are used, with varying degrees of success, in Kasabach-Merritt syndrome, including systemic corticosteroids, radiation therapy, compression, embolization and pharmacotherapy (e.g. interferon, platelet aggregation [radiopaedia.org]
What is the Prognosis of Kasabach-Merritt Syndrome? (Outcomes/Resolutions) The prognosis of Kasabach-Merritt Syndrome depends upon the severity of the signs and symptoms, the numbers, sizes, and locations of the vascular tumors. [dovemed.com]
141000 DiseasesDB 30701 Kasabach-Merritt syndrome Microchapters Home Overview Historical Perspective Pathophysiology Causes Differentiating Kasabach-Merritt syndrome from other Diseases Epidemiology and Demographics Natural History, Complications and Prognosis [wikidoc.org]
The therapeutic management should be early, multidisciplinary adapting therapeutic benefits to risks in order to improve the prognosis. [ijias.issr-journals.org]
Primary angiosarcoma of the breast is a rare aggressive tumour of unknown etiology. [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]
[…] rubber bleb nevus syndrome Hepatic hemangioma Other conditions to consider: Classic hemangiomas of infancy Port-wine stain Arteriovenous malformation Neuroblastoma Leukemia cutis Angiosarcoma Infantile fibrosarcoma Consumptive coagulopathy of other etiology [visualdx.com]
[…] blood CF cerebrospinal fluid CI chemically induced CH chemistry CL classification CO complications CN congenital DI diagnosis DG diagnostic imaging DH diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology EH ethnology ET etiology [decs.bvs.br]
Alabama at Birmingham, Department of Pathology ICD-9 287.39 OMIM 141000 DiseasesDB 30701 Kasabach-Merritt syndrome Microchapters Home Overview Historical Perspective Pathophysiology Causes Differentiating Kasabach-Merritt syndrome from other Diseases Epidemiology [wikidoc.org]
Inglês: BS blood supply BL blood CF cerebrospinal fluid CI chemically induced CH chemistry CL classification CO complications CN congenital DI diagnosis DG diagnostic imaging DH diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology [decs.bvs.br]
[…] a complication of KHE and TA, which are rare, benign vascular tumors that typically present in infancy. KHE and TA are classified as having intermediate malignant potential as they are locally aggressive but are not known to metastasize. Epidemiology [statpearls.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]
Additional recommended knowledge Contents 1 Pathophysiology 2 Diagnostic workup 3 Management 3.1 Supportive care 3.2 Definitive treatment 4 Outcomes 5 References Pathophysiology KMS is usually caused by a hemangioendothelioma or other vascular tumor, [bionity.com]
The clinical presentation, laboratory findings, vascular pathology, and pathophysiology will also be discussed. [ncbi.nlm.nih.gov]
Pathophysiology Ecchymotic tumor develops on a vascular lesion; it usually starts in the neonatal period and grows very quickly; tumoral growth is accompanied by thrombocytopenia and hypofibrinogenemia. [accessanesthesiology.mhmedical.com]
The clinical presentation, laboratory findings, vascular pathology, and pathophysiology will also be discussed. Fingerprint Cite this APA Standard Harvard Vancouver Author BIBTEX RIS [experts.umn.edu]
In a case of a lobar manifestation of KMS, lobectomy can be safely performed to prevent life-threatening bleeding. [jmedicalcasereports.biomedcentral.com]
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]
Hence, emergency surgical excision was done to prevent life-threatening multi-organ hemorrhage. [jcnonweb.com]
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- 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.
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- 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.