Introduction: Kasabach-Merritt syndrome is a benign condition characterized by hemangiomatosis, severely disseminated intravascular consumption coagulopathy, and thrombocytopenia. The mortality rate increases from 12% to 30% in hemorrhagic cases. In general, the symptoms primarily manifest in the gastrointestinal tract, the skin, and the subcutaneous tissue. There is no publication about pulmonary manifestation of angiomatosis in combination with vascular malformation and hemoptysis. This is the first description of a Kasabach-Merritt syndrome-like condition in the lung.
Case Presentation: We present the case of a 29-year-old German woman with angiomatosis and associated pulmonary vascular malformation in her lower left lobe with a Kasabach-Merritt syndrome like condition. It was detected after hemoptysis. We also present our case observations and management.
Conclusion: In a case of angiomatosis with an associated pulmonary lobar vascular malformation, lobectomy can be safely performed to prevent life-threatening bleeding.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4436809 | PMC |
http://dx.doi.org/10.1186/s13256-015-0566-z | DOI Listing |
Neoreviews
January 2025
Vascular Anomalies Center, Division of Pediatric Surgery, Children's Hospital Colorado, Aurora, Colorado.
Vascular anomalies are broadly classified into 2 categories: vascular tumors and vascular malformations. Vascular anomalies frequently present as cutaneous lesions in infants. This review summarizes vascular anomalies that most commonly present as dermatologic lesions in the neonatal period, with a focus on the clinical findings, pathophysiology and histology, relevant radiographic findings, and management of common vascular anomalies such as infantile hemangiomas, congenital hemangiomas, and Kaposiform hemangioendothelioma, along with vascular malformations, including capillary, lymphatic, venous, and arteriovenous malformations.
View Article and Find Full Text PDFJ Med Case Rep
December 2024
Shifa International Hospital/Shifa Tameer e Millat University, Islamabad, Pakistan.
Background: Angiosarcoma is a rapidly proliferating vascular tumor that originates in endothelial cells of vessels. Rarely, it can be associated with consumptive coagulopathy due to disseminated intravascular coagulation eventually leading to thrombocytopenia and microangiopathic hemolytic anemia. This specific manifestation is termed Kasabach-Merritt syndrome.
View Article and Find Full Text PDFJ Med Case Rep
December 2024
Department of Surgery, Aga Khan University, Karachi, Pakistan.
Background: Kaposiform hemangioendothelioma is a rare vascular tumor primarily occurring in infants and children. The most common sites for kaposiform hemangioendothelioma are extremities, with very few cases of abdominal kaposiform hemangioendothelioma reported in neonates. Making a diagnosis of Kaposiform hemangioendothelioma can be challenging when the patient presents with generalized symptoms such as bilious vomiting and constipation that can be attributed to other more common causes of intestinal obstruction.
View Article and Find Full Text PDFFront Pediatr
November 2024
[This corrects the article DOI: 10.3389/fped.2021.
View Article and Find Full Text PDFHematology Am Soc Hematol Educ Program
December 2024
Pediatric Hematology-Oncology, University of Arkansas for Medical Sciences, Little Rock, AR.
The role of the hematologist in the management of vascular anomalies is evolving. Several vascular tumors and malformations are associated with complex coagulation derangements. Kaposiform hemangioendothelioma or tufted angiomas may present with a consumptive coagulopathy known as the Kasabach-Merritt phenomenon (KMP).
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!