Kasabach-Merritt Syndrome in an Adult: A Comment

Gözdaşoĝlu, Sevgi

doi:10.4274/tjh.galenos.2018.2018.0356

Cited by 3 publications

(3 citation statements)

References 6 publications

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“…KMP has been thought to be caused by platelets trapped in the tumor by proliferating vascular endothelial cells, which are often characterized by thrombocytopenia and consumptive coagulation dysfunction, as well as tumors with ill-defined borders. [1,[7][8][9][10] The lesions in KMP patients usually appear in the form of purpuric masses on the surface, along with edema. [11,12] However, lesions can also involve intrathoracic or retroperitoneal structures, which are often associated with higher mortality due to their delayed diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…Kasabach-Merritt phenomenon (KMP) is a coagulopathy usually caused by kaposiform hemangioendothelioma (KHE) and characterized by thrombocytopenia, microangiopathic hemolytic anemia, and a rapidly enlarging vascular tumor in either infants or young children. [1][2][3] KMP usually presents as an enlarging, purpuric lesion, involving the trunk, extremities, face, and retroperitoneum. [4] Despite the discovery of new therapies, there is no consensus treatment for it, and KMP is still associated with high morbidity and mortality.…”

Section: Introductionmentioning

confidence: 99%

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Combination therapy for pediatric patients with Kasabach–Merritt phenomenon: A single-center retrospective study

Zhang

Luo

et al. 2022

Medicine

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This study aimed to in the management of Kasabach–Merritt phenomenon (KMP), a severe thrombocytopenic coagulopathy that occurs in the presence of an enlarging vascular tumor. Here, we retrospectively evaluated 12 patients with KMP in Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, from 2017 to 2021. 12 patients, including 7 females and 5 males, were identified. Tumors were located in the leg (n = 4), neck (n = 1), face (n = 3), chest wall (n = 1), back (n = 2), and retroperitoneum (n = 1). A plaque-like lesion with ecchymosis was the most common cutaneous manifestation. All the patients underwent embolization therapy. Nine patients received steroid treatment and 7 patients were administered with sirolimus. The mean duration of treatment was 1.6 months. All the patients reported in this study were alive when discharged. Embolization combined with steroid and sirolimus appears effective in patients with KMP, as well as in those who experienced disease recurrence. However, a long-term follow-up of the children cured of KMP will be necessary to monitor its recurrence and improve the outcome.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Combination therapy for pediatric patients with Kasabach–Merritt phenomenon: A single-center retrospective study

Zhang

Luo

et al. 2022

Medicine

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“…One such complication is Kasabach-Merritt syndrome, characterized by thrombocytopenia, microangiopathic hemolytic anemia, and consumptive coagulopathy in the presence of a rapidly enlarging vascular tumor. (4,5) Another threat is a coagulopathy limited to the vascular anomaly (LIC). It is characterized by elevated D-dimers and fibrin degradation products, low levels of fibrinogen, FV, FVIII, FXIII, and antithrombin, and sometimes mild to moderate thrombocytopenia.…”

Section: Introductionmentioning

confidence: 99%

Hematologic complications in vascular malformations: A case study of 2 patients

Matus,

Bator,

Machaj

et al. 2024

J Educ Health Sport

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Vascular malformations are congenital vascular anomalies resulting from a disruption in the vasculogenesis process. (1) They are congenital changes that enlarge as the child develops and do not undergo spontaneous involution. They can be classified based on the type of vessels involved (capillary, arterial, venous, lymphatic, mixed) and the nature of the vascular flow (low-flow and high-flow). The clinical presentation of vascular malformations is highly diverse, and despite being congenital, they can remain asymptomatic for a long time, complicating the diagnostic process. (1,3) The presence of malformations carries the risk of health-threatening complications. One such complication is Kasabach-Merritt syndrome, characterized by thrombocytopenia, microangiopathic hemolytic anemia, and consumptive coagulopathy in the presence of a rapidly enlarging vascular tumor. (4,5) Another threat is a coagulopathy limited to the vascular anomaly (LIC). It is characterized by elevated D-dimers and fibrin degradation products, low levels of fibrinogen, FV, FVIII, FXIII, and antithrombin, and sometimes mild to moderate thrombocytopenia. (6,7) Early implementation of anticoagulant therapy allows for the avoidance of health-threatening conditions and the development of disseminated intravascular coagulation syndrome (DIC). (8)

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