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Query: UMLS:C0012739 (disseminated intravascular coagulation)
 8,673 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors describe the total excision of a huge occipital hemangioendothelioma in an infant with thrombocytopenia, hypofibrinogenemia, and anemia; treatment was followed by normalization of platelet count, blood cell count, and level of fibrinogen. Histological and clincial studies support the concept the disseminated intravascular coagulation was related to the thrombocytopenia and coagulation deficits found in this case.
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PMID:Giant occipital hemangioendothelioma with thrombocytopenia, anemia, and hypofibrinogenemia. Case report. 114 85

An infant with a large occipital hemangioendothelioma with thrombocytopenia, anemia, and hypofibrinogenemia--Kasabach-Merritt syndrome--was reported. The case, a male neonate is unique, for this is the first report with this syndrome in whom the large hemangioma was noted at birth on the midocciput simulating the occipital encephalomeningocele. With the development of thrombocytopenia of 84,000 per mm3, hypofibrinogenemia of 92 mg/dl, anemia (erythrocyte 193 X 10(4) per mm3, hemoglobin 5.9 g/dl, hematocrit 16 vol%), hepato-splenomegalia, enlargement and bluish discoloration of the tumor noted on the 21/2 months of life, total excision was intended prior to the expected occurence of the systemic purpura. The patient received fresh whole blood transfusion immediately prior to surgery, and the total excision was successfully performed. Excessive bleeding was not encountered. Abrupt rise in the platelet count, red blood cell count, hemoglobin and hematocrit to normal range was noted at the first postoperative day; he was discharged on the 17th day after surgery. Nineteen months' follow-up showed normal hematologic findings with good somatic and mental development. The specimen weighing 250 g. revealed benigh hemangioendothelioma. Silver impregnation demonstrated lobular aggregates of small vascular channels. Papillary projection of interstitial cells into the lumen, reaction of the endothelium of the vessels, newly formed thrombus, ishemic necrosis and hemorrhage, hyaline degeneration of interstitial tissue were noted. These findings suggested the disseminated intravascular coagulation within the tumor followed by fibrinolysis accounts for loss of blood corpuscles, platelet, fibrinogen and clotting factors, which leads ultimately to the consumption coagulopathy and diffuse bleeding.
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PMID:[Giant occipital hemangioendothelioma with thrombocytopenia, anemia and hypofibrinogenemia treated by total excision (author's transl)]. 123 5

This report describes four infants with hepatic hemangioendotheliomas and cardiac failure who had extensive portal venous and systemic collateral arterial supply complicating hepatic arterial embolization. One patient with diffuse hepatic hemangioendothelioma and extensive portal vein supply but minimal systemic collateral arteries showed no improvement after technically successful hepatic artery embolization and died with disseminated intravascular coagulation and sepsis. A second infant with extensive portal vein and collateral supply died without undergoing embolization. Two patients had portal vein-hepatic vein fistulas as well as an extensive systemic arterial supply. Both infants tolerated staged hepatic and collateral artery embolization, although one patient died of congestive heart failure, probably related to pulmonary hemangiomas. The authors conclude that angiographic study of the potential collateral vessels and portal venous circulation should be performed before embolization. Patients with shunting from the portal vein to the hepatic vein and minimal systemic arterial collateral circulation should not undergo hepatic artery embolization.
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PMID:Infantile hepatic hemangioendotheliomas: significance of portal venous and systemic collateral arterial supply. 162 83

Hemangioendothelioma is seldom seen in adults. Its severe evolution is due, not so much to the exceptional transformation into hemangiosarcoma, but mainly to haemorrhagic complications by rupture or consumption coagulopathy and to severe cardiac insuffficiency secondary to arteriovenous shunts. The case reported here concerns a 64 year-old woman presenting pain in the left hypochondrium and splenomegaly. A splenectomy was performed and the histological findings were compatible with the diagnosis of hepato-splenoganglionic hemangioendothelioma. The evolution was unfavorable. The patient died a few months later in a picture of haemorrhagic syndrome and cardiac insufficiency. Histological findings on autopsy specimens indicated a cavernous hemangioma. The treatment of these diffuse hemangiomas is a difficult one. Hepatic artery ligation has been advocated in certain desperate situations. Nevertheless, because of a collateral circulation, recurrences are frequent.
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PMID:[Lymph node-hepatosplenic hemangioma in an adult with consumption coagulopathy and fatal cardiac insufficiency]. 343 34

The case report of a newborn infant with a splenic hemangioendothelioma with the life-threatening complications of thrombocytopenia, anemia, and disseminated intravascular coagulation is presented together with a review of the literature. Removal of the tumor via splenectomy, despite the known risk of subsequent overwhelming sepsis due to encapsulated organisms in the young child, is the treatment of choice for splenic hemangiomas complicated by thrombocytopenia. The use of enhanced CT with delayed filling is a diagnostic tool in the workup of suspected hemangiomatous lesions.
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PMID:Splenic hemangioma with thrombocytopenia in a newborn. 358 49

A 4-month-old boy with benign hemangioma of the porta hepatis is described. Obstructive jaundice and consumption coagulopathy developed, which were treated by percutaneous transhepatic drainage (PTHD), without resection of the tumor or bypass surgery. Because of tumor regression, the patient has remained free of symptoms even after the PTHD tube was removed. Because juvenile hemangioma is a benign tumor and occasional spontaneous regression is known to occur (as in our case and other reports), it is suggested that complete resection or bypass surgery is not necessary for juvenile hemangioendothelioma, even with obstructive jaundice, if bile drainage is adequately maintained.
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PMID:Successful management of infantile hepatic hilar hemangioendothelioma with obstructive jaundice and consumption coagulopathy. 878 96

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 alpha-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.
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PMID:Spindle cell (Kaposiform) hemangioendothelioma with Kasabach-Merritt syndrome in an infant: successful treatment with alpha-2A interferon. 912 1

The term haemangioendothelioma has been used in the past for a number of vascular lesions, which vary not only by their morphological features, but more importantly, also by their biological behavior. In the recent WHO-classification of mesenchymal tumours haemangioendotheliomas have been defined as vascular tumours of "intermediate" or "borderline" malignancy, and spindle cell haemangioendothelioma (SHE), epithelioid haemangioendothelioma (EHE), and rare malignant endovascular papillary angioendothelioma (Dabska's tumour) were included in this category. To this list might be added the more recently delineated kaposiform (KHE), retiform (RHE), polymorphous (PHE), and composite haemangioendothelioma (CHE). Although very popular, the concept of "borderline" or "intermediate" malignancy encompasses a wide variety of clinical situations, prognosis, and biological behavior. Therefore uncritical use of the term haemangioendothelioma represents a potential source of confusion to patients and oncologists, and it should not be used without further clarification. SHE was originally described as low-grade angiosarcoma, however, the study of large series with expanded follow-up information clarified that these lesions are often multicentric in one anatomic region, whereas true recurrences are rather rare, and systemic metastases and tumour progression do not occur. Therefore redesignation of these lesions as spindle cell haemangioma has been proposed. EHE of skin and soft tissues represents a distinctive vascular neoplasm characterized by nests and cords of epithelioid endothelial tumour cells with characteristic cytoplasmic vacuoles, which are set in a myxohyaline matrix. The reported rates of systemic metastases (20-30%), and tumour related death of patients (13-17%) in EHE, and the occurrence of multicentric EHE argue against the classification of EHE as a low-grade or "borderline" malignant neoplasm; EHE should be better regarded as a clearly malignant vascular tumour (G2). Although it seems that KHE is associated with a high mortality rate, the deaths are almost always related to locally invasive effects or as result of bleeding and consumption coagulopathy. So far no metastasizing case of KHE has been reported, and it seems that the prognosis in KHE is mainly related to size, anatomical site and depth of the lesion. KHE should be classified as a locally aggressive, non-metastasizing vascular tumour. The remaining entities (RHE, Dabska's tumour, PHE, and CHE) are characterized by an infiltrative growth, a high rate of (often repeated) local recurrences, and a definitive risk of metastases. Therefore these lesions fulfil criteria for low-grade malignant vascular neoplasms.
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PMID:[Hemangioendotheliomas--evolution of a concept of a heterogeneous group of vascular neoplasms]. 1009 22

Angiosarcoma associated with fibrous dysplasia is very rare. We have recently experienced two cases of angiosarcoma that secondarily arose from fibrous dysplasia. The first patient, a 55 year-old man, had noticed a deformity in the right upper arm since he was five years old. At the age of 25 years, polyostotic fibrous dysplasia was diagnosed by X-ray examination. The patient complained of swelling and pain around the left shoulder. The diaphysis of the humerus was mostly non-observable due to severe bone destruction by tumor invasion and there was a large soft tissue tumor. Biopsy examination revealed grade II or III hemangioendothelioma with typical histologic findings of fibrous dysplasia. After interscapulothoracic amputation for wide tumor resection, he died of DIC. Autopsy revealed multiple liver metastatic lesions of angiosarcoma. The second patient was a 66-year-old woman. She presented with a pathological fracture in the right tibia, due to an osteolytic lesion with cortical bone swelling and thinning. The histologic diagnosis was fibrous dysplasia without any sarcomatous changes. However, after a 3-time recurrence, angiosarcoma arose from the same lesion. She is now still alive with disease-free period of 6.5 years after amputation.
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PMID:Two cases of secondary angiosarcoma arising from fibrous dysplasia. 1065 Jul 91

Hepatic resection is the main treatment modality for hepatic tumors in childhood. Advances in diagnostic technique, preoperative preparation, surgical technique, and postoperative management increased the success rate. The aim of this study is to report our experience in hepatic lobectomy, which is relatively rare procedure in childhood. Medical records of 25 patients who underwent hepatic lobectomy between January 1977 and June 2002 were reviewed retrospectively. Age, gender, diagnosis, physical examination findings, results of preoperative laboratory investigations, radiological examination, resectability criteria, preoperative biopsies, chemotherapies, radiotherapies, postoperative pathological results, incisions, operation technique, intraoperative transfusions, drains used, antibiotic prophylaxes, and intraoperative and postoperative complications were evaluated for all patients. Out of 25 patients with hepatic tumor seven patients with hepatoblastoma and four patients with hepatocellular carcinoma were given 5.7 +/- 0.3 cycles of chemotherapy before the operation. Right lobectomy (n = 12), left lobectomy (n = 5), extended left lobectomy (n = 4), and extended right lobectomy (n = 3) and right lobectomy with enucleation of two masses from left lobe (n = 1) were performed. Intraoperative blood transfusion of 30.7+/-6.0 ml/kg body weight was necessary. Pathological examination of resected tumors revealed hepatoblastoma (n=11), mesenchymal hamartoma (n = 5), hepatocellular carcinoma (n = 4), hemangioendothelioma (n=1), malignant mesenchymal tumor (n = 1), hemangioma (n = 1), cyst adenoma (n = 1), and metastasis of cellular mesoblastic nephroma (n = 1). Patients were observed in the intensive care unit for 3.4 +/- 0.3 days. Postoperative complications were sepsis (n = 1), disseminated intravascular coagulation (n = 2), fever (n = 3), jaundice (n = 3), intraabdominal abscess (n = 3), ileus (n = 2), and subdiaphragmatic abscess with pleural effusion (n = 1). Hepatic lobectomy is a major operation, which is feasible yielding curative results in children. Safe hepatic resections with acceptable blood loss can be performed by a technique relying on good anatomic dissection and surgical control.
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PMID:Hepatic lobectomies in children: experience of a center in the light of changing management of malignant liver tumors. 1639 9


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