Kasabach-Merritt Syndrome Research Articles

Treatment of the Kasabach-Merritt syndrome with pegylated recombinant human megakaryocyte growth and development factor in mice: elevated platelet counts, prolonged survival, and tumor growth inhibition.

Kasabach-Merritt Syndrome (KMS) is seen in children with large vascular tumors. KMS is characterized by very low platelet counts and a consumption of coagulation factors causing life-threatening complications. It has been proposed that thrombopenia in these patients is caused by intratumoral trapping of platelets. The truncated form of the cMpl-receptor ligand thrombopoietin, pegylated human megakaryocyte growth and development factor (Peg-rHuMGDF), is an agent that stimulates platelet production. We hypothesized that stimulation of the platelet production would prevent the life-threatening complications of patients with KMS owing to low platelet counts. In a mouse model of KMS, with tumors derived from a hemangioendothelioma cell line, we studied the effect of Peg-rHuMGDF. Treatment with Peg-rHuMGDF (10 microg/kg/day intraperitoneally) increased platelet counts by 7-8-fold compared with control tumor-bearing mice after 11 d of treatment (p < 0.001, n = 8). Survival was significantly increased, with 50% of treated animals alive at 1 mo versus 0% in untreated controls. Interestingly, we also observed an inhibition of tumor growth by 75% (p < 0.001, n = 8). Hematoxylin and eosin staining showed fresh fibrin clots in the treated tumors, suggesting that higher platelet counts caused intravascular thrombosis of tumor vessels. We conclude that increased platelet production in this model of KMS resulted in an antivascular tumor effect via platelet trapping. Further, we propose that thrombopoietin may be of critical value in preventing life-threatening complications from KMS.

Spontaneous spinal epidural haematoma in association with Kasabach-Merritt syndrome

The authors report a case of spontaneous spinal epidural haematoma causing paraplegia secondary to thrombocytopaenia and consumptive coagulopathy from Kasabach-Merritt syndrome. The patient had long-standing multiple subcutaneous and visceral haemangiomas from childhood and developed lumbago and subsequent paraplegia. Evacuation of a spinal epidural haematoma was performed, however, the patient's neurological symptoms did not improve significantly. This case describes the potential risk of bleeding in the spinal column in patients with Kasabach-Merritt syndrome. Prompt diagnosis and management are crucial for an optimal outcome.

Debulking of a gastrointestinal hemangioma alleviates coagulopathy associated with Kasabach-Merritt syndrome

Kasabach-Merritt syndrome is a congenital proliferative vascular malformation associated with a bleeding tendency caused by profound thrombocytopenia. The congenital hemangiomas of Kasabach-Merritt syndrome are generally quite large in size, and thrombocytopenia results from platelet trapping and destruction within the lesions, as well as from a local consumption coagulopathy. Kasabach-Merritt syndrome presents a difficult management challenge in which surgery remains the most effective treatment. Complete resection of the hemangioma is curative but unfortunately is not always technically possible. In this case report we discuss the surgical management of a young man with Kasabach-Merritt syndrome resulting from a large colorectal hemangioma.

Debulking of a gastrointestinal hemangioma alleviates coagulopathy associated with Kasabach-Merritt syndrome

Kasabach-Merritt syndrome is a congenital proliferative vascular malformation associated with a bleeding tendency caused by profound thrombocytopenia. The congenital hemangiomas of Kasabach-Merritt syndrome are generally quite large in size, and thrombocytopenia results from platelet trapping and destruction within the lesions, as well as from a local consumption coagulopathy. Kasabach-Merritt syndrome presents a difficult management challenge in which surgery remains the most effective treatment.

Diffuse neonatal haemangiomatosis: new views on diagnostic criteria and prognosis

Diffuse neonatal haemangiomatosis (DNH) is a rare and life‐threatening congenital disorder. An extensive retrospective analysis of the literature was performed to evaluate the clinical features, therapies and prognostic factors of DNH. Reports on 68 patients with DNH were obtained. The skin, liver, lungs, brain and intestine were the organs most commonly involved. Congestive heart failure (CHF) was the primary cause of death. The mortality rate was 77.4% in untreated patients and 27% in treated patients. CHF, Kasabach‐Merritt syndrome (KMS) and the involvement of five or more organs were important risk factors in DNH. The measurement of cardiac output might give more insight into the potential prognostic value of total blood‐volume loss through shunting in the haemangiomas. Reports on 64 patients with neonatal haemangiomatosis limited to only the skin and liver were also obtained. The clinical features and outcome of patients with only cutaneous and hepatic haemangiomas were similar to those of patients with DNH. The inclusion criteria for DNH should be expanded to include similar patients with only cutaneous and hepatic haemangiomas.

動脈塞栓術が奏効したＫａｓａｂａｃｈ‐Ｍｅｒｒｉｔｔ症候群の１症例

動脈塞栓術が奏効したＫａｓａｂａｃｈ‐Ｍｅｒｒｉｔｔ症候群の１症例

Treatment of Kasabach-Merritt syndrome

Treatment of Kasabach-Merritt syndrome

Kaposiform hemangioendothelioma associated with Kasabach-Merritt syndrome

OBJECTIVE: To describe a case of kaposiform hemangioendothelioma, the only malignant tumor of vascular origin specific of childhood.METHODS: We report a case of a 40-days-old girl who presented with a giant hemangioma of the face. Rapid enlargement of the tumor lead to laryngeal compression with severe respiratory distress. She had also a consumptive thrombocytopenic coagulopathy (Kasabach-Merritt syndrome).RESULTS: She was admitted to the pediatric intensive care unit of the Hospital das Clínicas da Universidade Estadual de Campinas and mechanical ventilation was introduced. A ten day course of dexamethasone was only partially effective. We then started alpha-2a interferon at a dose of 1.8 million units/m(2)/day by subcutaneous route, but she died four days after the onset of this therapy. The microscopic features showed a kaposiform hemangioendothelioma.CONCLUSION: We discuss this unusual fatal evolution of a rapidly growing hemangioma and its hematological complications.

Kaposiform hemangioendothelioma: A locally aggressive vascular tumor

Kaposiform hemangioendothelioma is a locally aggressive, endothelial-derived spindle cell neoplasm that occurs exclusively in infants and adolescents. Lesions are characterized by rapid growth and extension, and are often associated with Kasabach-Merritt syndrome and lymphangiomatosis. Clinically nonspecific, they can appear as tender rapidly expanding red plaques, nodules, grouped papules, or telangiectasias. The histology is distinctive, however, as it combines features of tufted angioma, progressive lymphangioma, and Kaposi's sarcoma in a characteristic pattern. We describe a patient with kaposiform hemangioendothelioma currently controlled with systemic prednisone. (J Am Acad Dermatol 1998;38:799-802.)

Letter to the editor: “Spindle cell (kaposiform) hemangioendothelioma with Kasabach-Merritt syndrome in an infant: Successful treatment with α-2A interferon (Deb et al., Med. Pediatr. Oncol. 28:358–361)”

Letter to the editor: “Spindle cell (kaposiform) hemangioendothelioma with Kasabach-Merritt syndrome in an infant: Successful treatment with α-2A interferon (Deb et al., Med. Pediatr. Oncol. 28:358–361)”

Syndrome de Kasabach-Merritt néonatal guéri par exérèse chirurgicale complète de l'angiome

Kasabach-Merritt syndrome is an infrequent combination of a giant hemangioma and severe thrombocytopenia. This syndrome may be life-threatening. There are various but no definitive methods of treatment. We describe here a neonatal case cured by total excision of the tumor. A boy was admitted at birth with a giant hemangioma of the back with thrombocytopenia. Tumor compression, corticosteroids, acetylsalicylic acid and ticlopidine were ineffective. Total surgical excision was performed on day 51, resulting in complete recovery. Total surgical excision of a localized hemangioma in a non-functional area is probably the most appropriate and justified treatment for the Kasabach-Merritt syndrome.

Kaposiform Hemangioendothelioma

<h3>Background:</h3> Kaposiform hemangioendothelioma is a rare, aggressive vascular proliferation in children that is clinically and histologically distinct from hemangioma of infancy. It often manifests later than infantile hemangioma, and complication by Kasabach-Merritt syndrome is common. <h3>Observations:</h3> We examined 3 children with kaposiform hemangioendothelioma, all of whom were initially misdiagnosed as having infantile hemangioma. All 3 children developed Kasabach-Merritt syndrome, in association with a rapidly growing cutaneous vascular mass. Treatment with systemic corticosteroids, interferon alfa, vincristine, and radiation therapy appeared to halt progression of the disease. None had evidence of human herpesvirus 8 infection. <h3>Conclusions:</h3> Cutaneous kaposiform hemangioendothelioma may appear in early infancy but often appears months to years later. It is frequently complicated by Kasabach-Merritt syndrome, has no known association with Kaposi sarcoma related to human immunodeficiency virus infection, and demonstrates aggressive local behavior with invasion but not distant metastasis. Awareness of this entity is important to prevent a mistaken diagnosis of common hemangioma of infancy. Arch Dermatol. 1997;133:1573-1578

Prenatal diagnosis of Klippel–Trénaunay–Weber syndrome as a cause forin utero heart failure and severe postnatal sequelae

Ultrasound examination led to prenatal diagnosis of Klippel-Trénaunay-Weber syndrome complicated by early fetal congestive heart failure. The postnatal course was complicated by the Kasabach-Merritt syndrome of thrombocytopenia due to platelet consumption within the haemangioma. There was need for neonatal cardiopulmonary resuscitation and limb amputation.

Outcome of radiation therapy for patients with Kasabach-Merritt syndrome

The efficacy of radiation therapy for Kasabach-Merritt syndrome, which is characterized by a huge hemangioma with consumption coagulopathy, remains controversial. In this study, we retrospectively investigated the treatment outcome of radiation therapy for seven neonates with Kasabach-Merritt syndrome. During the past 25 years we have seen seven children with Kasabach-Merritt syndrome who were treated with radiation therapy. Their ages ranged from 1 day to 5 months, with a median age of 1 month. The hemangioma was located in the extremities in four of seven children. Tumor sizes ranged from 70 cm to more than 150 cm in greatest diameter. Initial platelet counts were all less than 40,000/mm3 except for one patient. In principle, the total dose applied to the hemangioma was 8-10 Gy, with a daily dose of 1 Gy five times a week. Four of seven hemangiomas responded dramatically, with a concomitant rise of the platelet count to radiation therapy. Although the remaining three hemangiomas, all of which were ill circumscribed by widespread overlying shiny, dusky purple skin, became less tense during radiation therapy. Disseminated intravascular coagulopathy was not improved, but they have responded favorably to two or three courses of radiation therapy with an extended radiation field by 1.5 years of age. As a result, all seven patients are now surviving with no evidence of hemangioma or hematological abnormalities. Shortening of the extremity was observed in three patients who received multiple courses of radiation therapy. Radiation therapy appears to be one of the effective treatment options for Kasabach-Merritt syndrome despite the risk of growth delay and malignancy.

Kasabach-Merritt Syndrome Is Not Associated with Typical Hemangiomas

Kasabach-Merritt syndrome, in which platelet trapping leads to life-threatening thrombocytopenia, is considered a complication of hemangioma. This

Radiotherapy in kasabach-merritt syndrome

A male infant was born with a 1 × 3 cm reddish lesion under his umbilicus which enlarged within a month and was associated with consumptive coagulopathy. A diagnosis of Kasabach-Merritt syndrome (KMS) was made. After no response to prednisolone and interferon-α treatment, 8 Gy of radiotherapy (2 Gy. day −1) was applied. The clinical status improved and the lesion decreased by 95%. This case illustrates the potential use of radiotherapy in the management of KMS. Radiotherapy is a relatively simple and inexpensive treatment modality which should be considered for lesions that have failed to respond to medical treatment.

Spindle cell (Kaposiform) hemangioendothelioma with Kasabach‐Merritt syndrome in an infant: Successful treatment with α‐2A interferon

A two-month-old infant developed a vascular tumor of the right flank which, upon biopsy, proved to be a spindle cell hemangioendothelioma. The increased capillary bed characterizing the neoplasm caused a severe thrombocytopenia together with a consumption coagulopathy (Kasabach-Merritt syndrome). The patient, who was dependent on platelet transfusions, improved quickly after interferon α-2a was given at the dosage of 3,000,000 U/m2, with resolution of the Kasabach-Merritt syndrome after three weeks and a 75% decrease of the tumor volume within three months of treatment. Med. Pediatr. Oncol. 28:358–361, 1997. © 1997 Wiley-Liss, Inc.

Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas

Objective: In 1940 Kasabach and Merritt described an infant with a vascular anomaly, extensive purpura, and thrombocytopenia; they called his lesion “capillary hemangioma.” Hemangioma is a benign tumor that grows in infancy and is characterized by proliferation of endothelial cells and regression during childhood. Although Kasabach-Merritt syndrome (KMS) is frequently mentioned as a possible complication of hemangioma, our experience suggests that the anatomic vascular lesion underlying the thrombocytopenia is not a “true,” classic, involuting type of hemangioma of infancy and childhood. Study design: We reviewed the clinical and hemostasis data and the response to treatment in 22 cases of KMS, and we analyzed the biopsy specimens of 15 of them. Results: Clinically none of the 22 patients had classic hemangioma. There was no female preponderance. All patients had severe thrombocytopenia (lowest platelet count = 3000/mm 3) and consumption of fibrinogen. Histologically, none had the typical “capillary,” involuting type of hemangioma of infancy: they exhibited either a tufted angioma or a kaposiform hemangioendothelioma pattern; all specimens also contained numerous abnormal lymphatic-like vessels; lymphatic malformation was the major component in two patients. The infants exhibited a heterogeneous response to a number of therapeutic regimens, as noted in other reports. Severe morbidity was present; three of our patients died, and one had leg amputation. “Residua” were, in fact, residual vascular neoplasia, variable in duration, and not a stable fibrofatty residuum, as in classic involuted hemangioma; only the hematologic phenomenon was “cured” after a period of years. Conclusions: KMS is a distinctive disease of infancy, but the underlying vascular lesion is not a “true,” classic, involuting type of hemangioma of infancy. This is a different vascular tumor with a resemblance pathologically to either tufted angioma or kaposiform hemangioendothelioma in association with lymphatic-like vessels. Whether the underlying lesion in KMS is a single anatomic entity or heterogeneous cannot be definitely concluded from this study. We need a better understanding of the pathogenesis of KMS to improve our therapeutic management. (J Pediatr 1997;130:631-40)

Response to pentoxifylline of the coagulopathy associated with a vascular malformation (Kasabach-Merritt syndrome). 651

Response to pentoxifylline of the coagulopathy associated with a vascular malformation (Kasabach-Merritt syndrome). 651

Non-operative management of a splenic laceration in a patient with the Proteus syndrome.

An adult patient with the Proteus syndrome sustained a grade III splenic laceration after falling off a horse. Clinical features of this rare disorder include subcutaneous and visceral hamartomatous tumours....