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

  A hemogram revealed anemia with severe thrombocytopenia and prolongation of bleeding and clotting time and the presence of increased fibrin degradation products. [e-ijd.org]

  Doppler examination showed elevated peak systolic velocity in the middle cerebral artery, indicating acute fetal anemia. [ncbi.nlm.nih.gov]

  Hemolytic anemia Increased hemolysis, Haemolytic anaemia A type of anemia caused by premature destruction of red blood cells (hemolysis). [rarediseases.oscar.ncsu.edu]

• 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]

• Collapse

  Several local complications may need to be treated in time as hemangiomas may sometimes develop collapse, necrosis or hemorrhage, while vascular tumor growth in particular parts of the body, such as the upper eyelid, could lead to ametropia, amblyopia [spandidos-publications.com]

• Tachypnea

  At admission, she had tachycardia, tachypnea, grunting, prolonged capillary refill time, hypoxia (SpO2 85% on oxygen by prongs) and feeble pulses. [indianpediatrics.net]

• Abdominal Pain

  After surgery, blood coagulopathy was improved immediately Full size image Discussion Most patients with cavernous hemangioma are asymptomatic but a minority present with abdominal pain or abdominal distension. [surgicalcasereports.springeropen.com]

  The most common symptoms are abdominal pain and right upper quadrant discomfort. Consumption coagulopathy is rare but can occur as a symptom of hemangioma, and this phenomenon is known as Kasabach-Merritt syndrome (KMS). [synapse.koreamed.org]

  Presenting symptoms included abdominal distension in 3 patients and abdominal pain in 2 patients. The mean length of time from the diagnosis of hemangioma was 15.4 months (3–38 months). [journals.lww.com]

• Liver Mass

  CT revealed a huge mass without enhancement, with a diameter of 13 cm, and clear boundaries in the anterior and medial sections of the liver. [surgicalcasereports.springeropen.com]

• Tachycardia

  ( 2 Files ) Erkrankung : Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) ICD 10: I47 (Paroxysmal tachycardia), I47.2 (Ventricular tachycardia), ORPHA3286 Synonyme : Catecholamine-induced polymorphic ventricular tachycardia, bidirectional [orphananesthesia.eu]

  At admission, she had tachycardia, tachypnea, grunting, prolonged capillary refill time, hypoxia (SpO2 85% on oxygen by prongs) and feeble pulses. [indianpediatrics.net]

  […] tufted angioma (TA) - Blue or reddish-brown discoloration and skin induration Petechiae and bruising Painful, tender lesions Bleeding from thrombocytopenia and coagulopathy (locally and, at times, distantly [disseminated intravascular coagulation (DIC)]) Tachycardia [emedicine.medscape.com]

• Hypotension

  For 4 days, attempts were made to dress the exposed tissues, but the child began to exhibit signs of sepsis with hypotension and ischemia of the remaining tissues. [scielo.br]

  The child then began to develop sepsis with hypotension and ischemia of remnant tissues. This case presented a therapeutic challenge, which is the subject of this article. [jvascbras.org]

  […] which acts by stopping the growth and hastening involution.[ 2 ] It is associated with some severe systemic complications and side effects and infants need to be closely monitored.[ 5 ] The most common reported adverse effects of propranolol include hypotension [ncbi.nlm.nih.gov]

  […] hemangiomas which acts by stopping the growth and hastening involution. [2] It is associated with some severe systemic complications and side effects and infants need to be closely monitored. [5] The most common reported adverse effects of propranolol include hypotension [jiaps.com]

• Petechiae

  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]

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

  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]

• Phenylketonuria

  Phenylketonuria in a child from the Middle East. Am J Dis Child. 1963 Jan; 105 :102–103. [ PubMed ] [ Google Scholar ] Woods WR, Tulumello TN. Management of oral hemangioma. Review of the literature and report of a case. [ncbi.nlm.nih.gov]

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.

• Decreased Platelet Count

  platelet counts and sometimes other bleeding problems,[1] which can be life-threatening.[2] It is also known as hemangioma thrombocytopenia syndrome. [bionity.com]

  It is a rare disease of infancy in which the thrombocytopenia (decreased platelet count) results from the hemangioma trapping and activating platelets, which in turn promotes further growth of the vascular tumor. [nicklauschildrens.org]

  A rare disease, usually of infants, in which a vascular tumor leads to decreased platelet counts and sometimes other bleeding problems. Can be life-threatening. [standardofcare.com]

  Kasabach–Merritt syndrome, 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. [en.wikipedia.org]

  […] to view Discussion The main diagnostic features of KMS are giant hemangioma and decreased platelet count and consumptive coagulopathy. [jcnonweb.com]

• Hepatocellular Carcinoma

  carcinomas, and (3) metastatic infiltration of the bile ducts, particularly in colon adenocarcinoma [4]. [surgicalcasereports.springeropen.com]

Anticoagulant and antiplatelet medications can be used after careful assessment of the risks and benefits.[5] Definitive treatment Generally, treatment of the underlying vascular tumor results in resolution of KMS. [bionity.com]

The objectives of treatment of KMS are to prevent bleeding from consumptive coagulopathy and induce vascular tumor regression. [e-ijd.org]

Historically steroids are typically first-line treatment. [statpearls.com]

Various treatment modalities have been tried with limited success. [ijaweb.org]

Hence, it can be used as one of the lifesaving treatment modality in patients with refractory KMS. Conclusions Vascular tumors in infancy may present with KMS and require aggressive treatment. [jcnonweb.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]

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]

KHE has an unpredictable course and with visceral infiltration are considered extensive and unresectable lesions, so they have a poor prognosis 4. [scielo.org.pe]

[…] 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]

Primary angiosarcoma of the breast is a rare aggressive tumour of unknown etiology. [ncbi.nlm.nih.gov]

[…] 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]

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]

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.[5] KHE and TA are classified as having intermediate malignant potential as they are locally aggressive but are not known to metastasize.[6][7][8] 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]

Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology ICD-9 287.39 OMIM 141000 DiseasesDB 30701 Kasabach-Merritt syndrome Microchapters Home Overview Historical Perspective Pathophysiology [wikidoc.org]

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]

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]

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]

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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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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.