Two cases of hemangioma with Kasabach-Merritt phenomenon diagnosed via ultrasound.
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ABSTRACT:
SUBMITTER: Wang Y
PROVIDER: S-EPMC10498253 | biostudies-literature | 2023 Sep
REPOSITORIES: biostudies-literature
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Two cases of hemangioma with Kasabach-Merritt phenomenon diagnosed via ultrasound.
Wang Yanzhao Y Guo Wenjing W Ma Bin B Li Tiangang T Yan Xide X Wang Yixuan Y Yan Zhiheng Z
Quantitative imaging in medicine and surgery 20230808 9
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Project description:Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of infancy that is commonly associated with a life-threatening thrombocytopenic condition, Kasabach-Merritt phenomenon (KMP). Platelet CLEC-2, tumor podoplanin interaction is considered the key mechanism of platelet clearance in these patients. Here, we aimed to assess platelet functionality in such patients. Three groups of 6 to 9 children were enrolled: group A with KHE/KMP without hematologic response (HR) to therapy; group B with KHE/KMP with HR; and group C with healthy children. Platelet functionality was assessed by continuous and end point flow cytometry, low-angle light scattering analysis (LaSca), fluorescent microscopy of blood smears, and ex vivo thrombi formation. Platelet integrin activation in response to a combination of CRP (GPVI agonist) and TRAP-6 (PAR1 agonist), as well as calcium mobilization and integrin activation in response to CRP or rhodocytin (CLEC-2 agonist) alone, were significantly diminished in groups A and B. At the same time, platelet responses to ADP with or without TRAP-6 were unaltered. Thrombi formation from collagen in parallel plate flow chambers was also noticeably decreased in groups A and B. In silico analysis of these results predicted diminished amounts of CLEC-2 on the platelet surface of patients, which was further confirmed by immunofluorescence microscopy and flow cytometry. In addition, we also noted a decrease in GPVI levels on platelets from group A. In KHE/KMP, platelet responses induced by CLEC-2 or GPVI activation are impaired because of the diminished number of receptors on the platelet surface. This impairment correlates with the severity of the disease and resolves as the patient recovers.
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Project description:Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm associated with the Kasabach-Merritt phenomenon (KMP), which is a consumptive coagulopathy with associated potentially life-threatening thrombocytopenia. There are no standardized treatment protocols for the management of KHE with KMP. Moreover, there are limited reports regarding the treatment of cases of rebound. Herein, we describe a rare case of rebound of KHE/KMP, during systemic sirolimus treatment, successfully treated with embolization and vincristine infusion combined with microwave ablation.
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Project description:Abstract Introduction The Kasabach–Merritt phenomenon (KMP) is a severe complication of kaposiform hemangioendothelioma (KHE). The risk factors for KMP need further investigation. Methods The medical records of patients with KHE were reviewed. Univariate and multivariate logistic regression models were used for the risk factors for KMP, and the area under the receiver operator characteristic (ROC) curve was used to assess the predictive power of risk factors. Results A total of 338 patients with KHE were enrolled. The incidence of KMP was 45.9%. Age of onset (P < 0.001, odds ratio [OR] 0.939; 95% confidence interval [CI] 0.914–0.966), lesion size (P < 0.001, OR 1.944; 95% CI 1.646–2.296), mixed type (P = 0.030, OR 2.428; 95% CI 1.092–5.397), deep type (P = 0.010, OR 4.006; 95% CI 1.389–11.556), and mediastinal or retroperitoneal lesion location (P = 0.019, OR 11.864; 95% CI 1.497–94.003) were correlated with KMP occurrence through multivariate logistic regression. ROC curve analysis revealed that the optimal cutoffs were 4.75 months for the age of onset (P < 0.001, OR 7.206, 95% CI 4.073–12.749) and a lesion diameter of 5.35 cm (P < 0.001, OR 11.817, 95% CI 7.084–19.714). Bounded by a lesion size of 5.35 cm, we found significant differences in tumor morphology, age of onset, treatments, and hematological parameters. Using an onset age of 4.75 months as a cutoff, we found significant differences in tumor morphology, lesion size, hematological parameters, and prognosis. Conclusion For KHE patients with an onset age <4.75 months and/or lesion diameter >5.35 cm, clinicians should be wary of the occurrence of KMP. Active management is recommended to improve the prognosis.
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Project description:Aim Kaposiform hemangiondothelioma (KHE) is a rare vascular tumor, commonly associated with Kasaback-Merritt phenomenon characterized by thrombocytopenia and consumptive coagulopathy. We report a case of pancreatic KHE presenting with neonatal duodenal obstruction and Kasaback-Merritt phenomenon. Case Report A full term male baby presented with bile stained vomiting on Day 3 of life. Contrast study and computed tomography scan showed duodenal obstruction by a 5 cm extrinsic hypervascular mass. Platelet count was 23 x 109/L. Laparotomy confirmed a vascular tumor arising from the pancreatic head compressing on the duodenum. Whipple operation was performed. Results Intestinal obstruction and thrombocytopenia resolved after surgery. There was no post-operative complications. Histology confirmed KHE. The boy was tolerating hydrolyzed milk formula and was thriving at 5 months follow up. Conclusion We reported a case of pancreatic KHE presented with neonatal intestinal obstruction and Kasaback-Merritt phenomenon. High index of suspicion is necessary for diagnosis. To our knowledge, this is the youngest patient who underwent Whipple operation.
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