Splenic kaposiform hemangioendothelioma presenting as insidious consumptive coagulopathy

2021 ◽  
Author(s):  
Marti Goldenberg ◽  
Matthew Shiel ◽  
Subramanian Subramanian ◽  
Ramasubramanian Kalpatthi ◽  
Miguel Reyes‐Múgica ◽  
...  
Keyword(s):  
Kaposiform Hemangioendothelioma ◽  
Consumptive Coagulopathy

scholarly journals Metastatic Angiosarcoma with Kasabach-Merritt Syndrome Responsive to Gemcitabine and Vinorelbine after Failure of Liposomal Doxorubicin and Paclitaxel: A Case Report

2016 ◽  
Vol 9 (1) ◽  
pp. 177-181 ◽  
Author(s):  
William L. Read ◽  
Felicia Williams
Keyword(s):  
Case Report ◽  
Disease Progression ◽  
Liposomal Doxorubicin ◽  
Vascular Tumors ◽  
Kaposiform Hemangioendothelioma ◽  
Consumptive Coagulopathy

Kasabach-Merritt syndrome (KMS) describes a consumptive coagulopathy associated with certain vascular tumors. It is thought that platelets are destroyed as they circulate through the aberrant endothelial surfaces associated with these tumors. Most published literature describes infants with kaposiform hemangioendothelioma, but a similar syndrome can complicate angiosarcoma in adults. This report describes a man with metastatic angiosarcoma arising in the scalp in whom disease progression was complicated by profound thrombocytopenia consistent with KMS. His disease and associated KMS had progressed previously through paclitaxel and then through liposomal doxorubicin. It did not respond to paclitaxel and bevacizumab, but responded almost completely to chemotherapy with gemcitabine and vinorelbine. Six months later, progression through ongoing chemotherapy then responded to chemotherapy with cyclophosphamide and sirolimus.

scholarly journals Consumptive coagulopathy is associated with a disturbed host response in patients with sepsis

2021 ◽  
Author(s):  
Lonneke A. Vught ◽  
Fabrice Uhel ◽  
Chao Ding ◽  
Cees van‘t Veer ◽  
Brendon P. Scicluna ◽  
...  
Keyword(s):  
Host Response ◽  
Consumptive Coagulopathy

scholarly journals Kaposiform Hemangioendothelioma: clinicopathological characteristics of 8 cases of a rare vascular tumor and review of literature

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Qurratulain Chundriger ◽  
Muhammad Usman Tariq ◽  
Jamshid Abdul-Ghafar ◽  
Arsalan Ahmed ◽  
Nasir Ud Din
Keyword(s):  
Pediatric Population ◽  
Adult Population ◽  
Correct Diagnosis ◽  
Kaposi Sarcoma ◽  
Minor Component ◽  
Vascular Tumor ◽  
Giant Cells ◽  
Clinicopathological Characteristics ◽  
Multinucleated Giant Cells ◽  
Kaposiform Hemangioendothelioma

Abstract Background Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumor of intermediate malignant potential which shows locally aggressive growth but only rarely metastasizes. It is mostly considered to be a tumor of pediatric population but its occurrence in the adults is not uncommon as once considered. Histologically, KHE can mimic other soft tissue neoplasms of different behaviors (e.g. Kaposi Sarcoma, hemangioma) and establishing the correct diagnosis is important for appropriate treatment. Herein, we describe the clinicopathological features of 8 cases of KHE which will be helpful in making their diagnosis. Methods We reviewed pathology reports, microscopy glass slides and obtained follow up information about 8 cases of KHE which were diagnosed at our institution from January 2008 till June 2020. Immunohistochemical stain for HHV8 was also performed. Results Age ranged from 7 months to 25 years. Seven patients were less than 20 years of age and one patient was 25 years old. Equal gender distribution was observed. Extremities were the most common sites of involvement, followed by head and neck, pancreas and ischiorectal region. 2 cases were resection specimen and all others were incisional biopsies. The largest tumor size was 5.5 cm in one of the resections. The incisional/fragmented tissues were all less than 5 cm in aggregate. Most cases showed predominance of nodular growth and a minor component of spindle cell population along with lymphangiomatosis like vascular channels, with evidence of microthrombi in 2 cases. Few multinucleated giant cells were observed in 2 cases. None of the cases exhibited significant nuclear atypia or mitotic activity. One of the cases arising in dermis showed underlying bone involvement. HHV8 was negative in 7/7 cases. Conclusions KHE can also involve adult population and it should always be considered in the differential diagnoses of a vascular lesion. Presence of multinucleated giant cells is a rare finding. Knowledge about histological features and potential mimics is helpful in avoiding misdiagnosis.

scholarly journals Potential factors influencing the development of thrombocytopenia and consumptive coagulopathy after genetically modified pig liver xenotransplantation

2012 ◽  
Vol 25 (8) ◽  
pp. 882-896 ◽  
Author(s):  
Burcin Ekser ◽  
Chih C. Lin ◽  
Cassandra Long ◽  
Gabriel J. Echeverri ◽  
Hidetaka Hara ◽  
...  
Keyword(s):  
Genetically Modified ◽  
Pig Liver ◽  
Consumptive Coagulopathy ◽  
Factors Influencing ◽  
Potential Factors
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