Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe the case of a 31-week fetus who died in utero with an invasive retroperitoneal kaposiform hemangioendothelioma. This rare vascular neoplasm usually presents as a localized violaceous skin lesion in infants and behaves in a benign fashion; however, kaposiform hemangioendothelioma may present as an invasive neoplasm of the chest or abdominal cavity, where it can lead to the Kasabach-Merritt syndrome, which consists of thrombocytopenia, consumptive coagulopathy, and microangiopathic anemia in association with a vascular anomaly. The case we describe is unique in that the tumor presented in utero and led to intrauterine nonimmune fetal hydrops. Kaposiform hemangioendothelioma has been described in utero; however, to our knowledge, intrauterine fetal death as a direct consequence has not been reported previously in the literature.
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PMID:Kaposiform hemangioendothelioma associated with nonimmune fetal hydrops. 1516 30

Kaposiform hemangioendothelioma is a rare, aggressive vascular proliferation in children that is clinically and histologically distinct from hemangioma of infancy. It is often complicated with Kasabach-Merritt syndrome. The authors describe a 2-month-old girl who developed a rapidly enlarging deep subcutaneous solid tumor in the left axilla. The tumor size was about 10x7 cm and there was no skin change over the mass. The results of laboratory tests were significant for a moderate anemia (7.0 g/dL) and a profound thrombocytopenia (3x10/L). The preoperative radiologic findings were suspicious for a soft tissue sarcoma with scapular erosion. Anemia and thrombocytopenia were corrected by preoperative corticosteroid therapy and blood component transfusion. The tumor was excised totally and the pathologic findings were consistent with Kaposiform hemangioendothelioma. Serial blood examinations showed that the hemoglobin and platelet count returned to the normal range after surgery.
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PMID:A case of axillary kaposiform hemangioendothelioma resembles a soft tissue sarcoma. 1700 73

We describe an infant presenting with fetal pericardial effusion requiring in utero pericardiocentesis. Important postnatal clinical features included recurrent pericardial effusion, progressive stridor, thrombocytopenia, anemia, and a mediastinal mass surrounding the heart and coronary arteries. Investigations and management consisted of repeat pericardiocentesis, platelet and red blood cell transfusions, laryngoscopy, creation of a pericardial window, and biopsy of the mediastinal mass. Diagnosis was made of Kaposiform hemangioendothelioma surrounding the base of the heart, trachea, and esophagus as well as Kasabach-Merritt phenomenon. The infant responded well to treatment with vincristine and prednisone.
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PMID:Kaposiform hemangioendothelioma presenting antenatally with a pericardial effusion. 1901 75

Kaposiform hemangioendothelioma is a very infrequent, locally aggressive vascular neoplasm, characterized by fascicular spindle cell proliferation. It occurs almost exclusively in infants and adolescents, and is often associated with Kasabach-Merritt phenomenon. The tumor is predominantly located subcutaneously or in the deep soft tissue of the extremities and trunk, peritoneum, or retroperitoneum. However, this tumor can sometimes be located on the head and neck. We report a case of kaposiform hemangioendothelioma of the maxillary sinus in a 4-month-old female infant presenting with cheek swelling, thrombocytopenia, anemia, and disseminated intravascular coagulation. Sinus computed tomography presented an enhancing, bone-destructing tumor. Magnetic resonance images showed an uncommon appearance as mostly low signal intensity on T2-weighted images.
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PMID:Kaposiform hemangioendothelioma arising from the maxillary sinus: a case report. 2022 56

Kaposiform hemangioendothelioma is an aggressive endothelial-derived spindle cell neoplasm that occurs nearly exclusively during childhood and teenage years. The lesion grows rapidly and is often associated with Kasabach-Merritt syndrome.In this study a 24 days old male neonate who presented with an ill-defined deeply situated violaceous mass on his left arm is described. He had also anemia and life-threatening thrombocytopenia. Despite hospitalization in intensive care unit (ICU) and transfusion of platelets and packed red blood cells as well as medical managements such as oral prednisolone, intravenous (IV) methylprednisolone and interferon alpha, thrombocytopenia persisted, so surgical resection was considered. The histopathological findings were distinctive and characteristic of kaposiform hemangioendothelioma. Following surgery, the infant did not have any complications and was discharged from the hospital in good condition.
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PMID:Kaposiform hemangioendothelioma: report of a case unresponsive to usual medical treatments. 2177 13

Kaposiform hemangioendothelioma (KH) is a rare vascular tumor of intermediate malignancy that occurs mainly in the childhood. Adult patients with KH are rare. Imaging findings of KH have rarely been reported before. We present magnetic resonance imaging (MRI), computed tomography (CT), and fluorine-18-fluorodeoxyglucose (F-FDG) positron emission tomography (PET)/CT findings in an adult patient with KH associated with lymphangiomatosis involving mesentery and ileum.A 22-year-old female complained of a 9-month history of intermittent melena, weakness, and palpitation. Laboratory tests revealed anemia and hypoproteinemia. Fecal occult blood test was positive. Abdominal enhanced MRI and CT showed a large abdominal mass involving mesentery and ileum. On enhanced MRI, there were many hypervascular nodules in the mass. On FDG PET/CT, the mass and the nodules showed slight FDG uptake. Small bowel capsule endoscopy showed numerous grape-shaped red nodules in the luminal wall of the involved ileum. The patient underwent resection of the abdominal mass and a segment of the ileum invaded by the abdominal mass. KH arising within lymphangiomatosis involving mesentery and ileum was confirmed by pathology. After surgery, the patient's symptoms improved.This is the first case of KH associated with lymphangiomatosis involving mesentery and ileum. In this case, the lymphangiomatosis overshadowed the small tumor nodules resulting in unusual imaging findings. Familiarity with these imaging findings is helpful for diagnosis and differential diagnosis of KH.
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PMID:Abdominal Kaposiform Hemangioendothelioma Associated With Lymphangiomatosis Involving Mesentery and Ileum: A Case Report of MRI, CT, and 18F-FDG PET/CT Findings. 2687 48

Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor that usually manifests during early childhood. Typically the lesion presents with skin, soft tissue and bone involvement and is characterized histologically by ill-defined nodularity and the presence of spindle cells with resemblance to Kaposi's sarcoma. We report a rare neonatal case of a splenic kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon that was diagnosed with radiographic imaging. Because of the rapid onset of thrombocytopenia and anemia, the patient required urgent splenectomy with subsequent resolution of the blood dyscrasias.
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PMID:Neonatal kaposiform hemangioendothelioma of the spleen associated with Kasabach-Merritt phenomenon. 2734 10