A Case Report on Kasabach Merrit Syndrome

Introduction: and importance: Infantile hemangioma, being a benign tumor of the blood vessel, is part of a triad composed of also thrombocytopenia and hypofibrinogenemia as part of Kasabach Merrit Syndrome. Case presentation: We report the case of a 2 months old female Saudi infant referred due to respiratory distress, thrombocytopenia, and enlarging hemangioma on right upper chest, neck, and lower cheek. Diagnosis of kaposiform hemangioendothelioma complicated by Kasabach Merritt thrombocytopenia was done based on the clinical triad of thrombocytopenia, bleeding tendency, and the presence of a vascular tumor. Conclusion: Vincristine and CTA embolization are lines of management that showed to be the most efficient in the improvement of the clinical picture of KMS in our patient.

The Journal of Pediatrics, 1997

Journal of Advanced Pediatrics and Child Health, 2021

Background: Rapidly involuting congenital hemangioma (RICH) is a rare vascular tumor that is present at birth and involutes during the first year of life. Kasabach-Merritt syndrome (KMS) is a complication of some vascular tumors such as kaposiform hemangioendothelioma and tufted angioma associated with thrombocytopenia and coagulopathy. Results: The case of a 2-month-old infant with a diagnosis of RICH with thrombocytopenia and coagulation disorder, successfully treated with surgical excision without complications or recurrence is presented. Conclusion: The association between RICH and KMS is rare. Histopathological study, immunohistochemistry and ultrasound findings are important for the diagnosis. Brief summary: This report covers the rare association between rapidly involuting congenital hemangioma and Kasabach-Merritt syndrome in a 2-months-old female infant.

Journal of Medicine, 2011

The case report deals with a third trimester pregnant female who presented with a mass in the dorsal paraspinal region (D7) resulting in rapid onset paraparesis gradually increasing in severity. The mass was of vascular nature (Haemangioma) on MRI, which on histopathology was confirmed as infiltrating ingiolipoma. Complications in haemangiomas occur and one such being the “Kasabach Merritt syndrome” consisting a triad of thrombocytopenia, haemangioma and coagulopathy, also dubbed as the “Haemangioma Thrombocytopenia Syndrome”. But its presentation in third trimester is definitely an unusual presentation considering the fact that such thrombocytopenic syndromes usually occur with congenital haemangiomas and with other benign vascular lesions in infants. The present case deals with the mass in dorsal spinal area resulting in compressive myelopathy as well as thrombocytopenic coagulopathy. Keyword: Paraspinal; Paraparesis; Haemangioma; MRI; Angiolipoma; Thrombocytopenia; Coagulopathy; ...

Histopathology, 1999

Isolated diffuse hemangiomatosis of the spleen with Kasabach-Merritt-like syndrome Aims: Diffuse haemangiomatosis of the spleen is a rare benign vascular condition occurring as a manifestation of systemic angiomatosis or, less commonly, confined to the spleen. It is sometimes accompanied by severe disturbance of blood coagulation. The goal of this study was to characterize an additional case of isolated diffuse haemangiomatosis of the spleen and to determine the histogenesis of this lesion which remains obscure. Methods and results: We describe a case of isolated diffuse haemangiomatosis of the spleen in which histological and immunohistological findings suggested the possibility of a malformative tumour-like lesion. The pathological cavernous vessels were distributed randomly through the red pulp, without continuity with sinuses. The endothelial cells expressed vimentin, factor VIII related antigen and CD34, but not CD8. Some cells lining the sinus lumen expressed CD68, lysozyme and myeloperoxidase. In addition, trabecular veins presented with intimal thickening. These results allow making a diagnosis between diffuse haemangiomatosis and other tumours/tumour-like lesions of the spleen, especially littoral cell angioma, splenoma and peliosis. Conclusion: If diffuse haemangiomatosis is usually classified as a benign proliferation of endothelial cells, we suggest that diffuse haemangiomatosis, when confined to the spleen, could be a tumour-like vascular lesion. In this hypothesis, the aetiology may be hamartomatous or malformative as is suspected in arterio-venous haemangioma of the lower extremities. The histogenesis is still questionable and no definitive proof in favour of one or the other hypothesis has been reported.

Journal of Vascular and Interventional Radiology, 2012

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor and has a high mortality in newborns when associated with Kasabach-Merritt syndrome (KMS). In two newborns with KHE and severe KMS refractory to medical treatment, emergency embolization led to clinical improvement in the acute neonatal setting by reducing tumor volume, increasing the platelet count, and improving other clotting parameters. Systemic vincristine treatment was added for further tumor control. Both patients remained symptom-free at long-term follow-up. ABBREVIATIONS APTT ϭ activated partial thromboplastin time, KHE ϭ kaposiform hemangioendothelioma, KMS ϭ Kasabach-Merritt syndrome, PT ϭ prothrombin time

Archives De Pediatrie, 2019

Journal of Perinatal Medicine, 1999

Egyptian Pediatric Association Gazette

Background Infantile haemangiomas (IH) represent a common benign vascular tumour affecting the paediatric population. Infantile haemangiomas are characterised by a natural history differentiating it from other vascular anomalies. After a transient proliferative phase in early infancy, the tumour passes through a plateau phase before going into spontaneous involution. In this report, we tried to share our experience over the last 5 years in managing cases presenting with IH at a specialised vascular anomaly clinic. Main body of abstract This report included cases of IH who were attending the vascular anomaly clinic during the period 2015 through 2019. Data of all patients attending the clinic were retrospectively examined. Files of 103 cases with IH were available for review. The diagnosis of IH was usually straight forward owing to the typical history and characteristic findings at clinical examination. A significant female predominance was noticed. Generally, IH were more common in...

Pediatric Dermatology, 2009

Infantile hemangiomas (IHs) are the most common benign vascular tumors of childhood. IH of "segmental" morphology, are clusters of hemangiomas with a configuration involving a broad anatomic territory of skin. They are the least common of all types and generally larger than regular hemangiomas, morphologically characterized as plaque-like lesions. Head and neck segmental hemangiomas have a higher risk of causing life-threatening complications and of having associated structural anomalies, i.e., PHACES syndrome (Posterior fossa malformations, hemangiomas, arterial anomalies, coartation of the aorta and other cardiac defects, eye abnormalities and sternal clefting or supra abdominal raphe). We present a patient with a segmental IH over the right anterior neck complicated by ulceration and life threatening arterial bleeding. Although segmental hemangiomas of head and neck have high incidence of ulceration, fortunately life threatening bleeding events are rare with only 7 previously recorded cases. We recommend that large, neck IH be followed closely for evidence of ulceration and that MRI/MRA be performed to adequately assess their vascular supply. Direct extension of the ulceration into arterial vessels is a possibility and can lead to severe bleeding. Life-threatening bleeding is an unusual complication of IH and may represents a surgical emergency. In such cases we recommend a multidisciplinary approach to their treatment.