Impact of age and tumor size on the development of the Kasabach–Merritt phenomenon in patients with kaposiform hemangioendothelioma: a retrospective cohort study

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 Three hundred thirty-eight 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.

Refractory kaposiform lymphangiomatosis relieved by splenectomy

“…In the previous studies, there have been many hypotheses regarding the occurrence of KMP, mostly related to lesion sizes, platelet activation, and high expression of podoplanin ( 35 , 36 ). Our previous research revealed that the larger the lesion in KHE patients, the higher the risk of developing KMP ( 37 ). The spleen lesions in all three patients were diffuse.…”