Jitsuiki Kei, Hamada Michika, Ota Soichiro, Muramatsu Ken-Ichi, Yanagawa Youichi
Acute Critical Care Medicine, Shizuoka Hospital, Juntendo University, Izunokuni, JPN.
Cureus. 2022 Jun 2;14(6):e25589. doi: 10.7759/cureus.25589. eCollection 2022 Jun.
A 48-year-old Mongolian man developed bilateral leg edema after suffering from a fever for three months. He lost his appetite, and the edema gradually spread from the legs, becoming systemic. In addition, he had difficulty in moving. He had a history of being diagnosed with numerous venous malformations and Kasabach-Merritt syndrome when he was a child. On arrival, he had numerous venous malformations over pale skin, edema at each extremity, and anemic conjunctiva. Chest roentgen showed bilateral pleural effusion, and cardiac echography findings showed a left ventricular ejection fraction of 30% with diffuse hypokinesis. The results of a blood analysis showed coagulopathy, which was compatible with disseminated intravascular coagulation and pancytopenia. He was diagnosed with blue rubber bleb nevus syndrome with Kasabach-Merritt syndrome and heart failure. Use of diuretics, thiamine, iron, phytonadione, carbazochrome, and tranexamic acid, in addition to intermittent transfusion resulted in the improvement of his Kasabach-Merritt syndrome. Radical management of blue rubber bleb nevus syndrome was deemed impossible by dermatologists due to the large amount of venous malformations. We encountered an extremely rare case of blue rubber bleb nevus syndrome with Kasabach-Merritt and heart failure. Multimodal therapy might help manage Kasabach-Merritt syndrome following improvement in coagulopathy and pancytopenia.
一名48岁的蒙古族男性在发热三个月后出现双侧腿部水肿。他食欲不振，水肿逐渐从腿部蔓延至全身。此外，他行动困难。他小时候曾被诊断出患有多处静脉畸形和卡萨巴彻-梅里特综合征。入院时，他苍白的皮肤上有多处静脉畸形，四肢水肿，结膜贫血。胸部X线显示双侧胸腔积液，心脏超声检查结果显示左心室射血分数为30%，伴有弥漫性运动减弱。血液分析结果显示凝血功能障碍，符合弥散性血管内凝血和全血细胞减少。他被诊断为患有蓝色橡皮疱痣综合征合并卡萨巴彻-梅里特综合征和心力衰竭。除了间歇性输血外，使用利尿剂、硫胺素、铁、维生素K、卡巴克络和氨甲环酸后，他的卡萨巴彻-梅里特综合征有所改善。由于静脉畸形数量众多，皮肤科医生认为无法对蓝色橡皮疱痣综合征进行根治性治疗。我们遇到了一例极为罕见的蓝色橡皮疱痣综合征合并卡萨巴彻-梅里特综合征和心力衰竭的病例。在凝血功能障碍和全血细胞减少有所改善后，多模式治疗可能有助于控制卡萨巴彻-梅里特综合征。
