Chronic defibrination syndrome due to a giant hemangioma associated with microangiopathic hemolytic anemia

Inceman, S; Tangün, Y

doi:10.1016/0002-9343(69)90102-8

Cited by 71 publications

(25 citation statements)

References 25 publications

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“…15 In the present case the palpable petecheal lesions located on the lower extremities were compatible with microembolic lesions.…”

supporting

confidence: 64%

Consumption coagulopathy and microangiopathic hemolytic anemia with an axillo-femoral graft.

Myers¹,

Hild²

1977

Circulation

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SUMMARY Consumption coagulopathy and microangiopathic hemolytic anemia occurred as a complication of insertion of an axillofemoral, preclotted dacron graft. Treatment with heparin followed by dipyridamole and aspirin normalized the hematologic and CONSUMPTION COAGULOPATHY is a recognized complication of a wide variety of diseases."2 Microangiopathic hemolytic anemia (MAHA) has been frequently associated with consumption coagulopathy3`as well as other entities.

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“…15 In the present case the palpable petecheal lesions located on the lower extremities were compatible with microembolic lesions.…”

supporting

confidence: 64%

Consumption coagulopathy and microangiopathic hemolytic anemia with an axillo-femoral graft.

Myers¹,

Hild²

1977

Circulation

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“…[10][11] PE has been described in case reports of 8 adult patients with KTS, ranging in age from 21 to 63 years (Table 1). [15][16][17][18][19][20][21][22] Although no case reports of PE among children with KTS exist in the English-language literature, there are 5 prior reports documenting children (age range: 4 weeks to 18 years) with Proteus syndrome [10][11][12] and/or undiagnosed complex vascular malformations 23 complicated by PE; 4 conditions were fatal (Table 1). All 5 patients experienced cardiopulmonary arrests, 2 of which occurred postoperatively.…”

Section: E598mentioning

confidence: 99%

Pulmonary Thromboembolism Associated With Klippel-Trenaunay Syndrome

Huiras¹,

Barnes

Eichenfield

et al. 2005

Pediatrics

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ABSTRACT. Klippel-Trenaunay syndrome (KTS) is a rare congenital anomaly characterized by unilateral limb overgrowth, venous varicosities, and capillary malformations (port wine stains) of the affected limb or limbs. Large venous malformations such as those observed in KTS are rare, and many physicians are unfamiliar with the potential complications, which include hypercoagulability, thrombosis, and pulmonary embolism (PE). As a result, patients may suffer from delayed diagnosis of a potentially life-threatening thromboembolic event. We present 2 cases of children with KTS complicated by PE, and we review the English-language literature regarding pathophysiologic features, interventions, and outcomes of PE in the setting of KTS among both pediatric and adult patients, with emphasis on issues relevant to pediatricians. I n 1900, French physicians Klippel and Trenaunay described a patient with massive hemihypertrophy of the lower extremity associated with a large, confluent, red-blue plaque and venous varicosities. 1 The term Klippel-Trenaunay syndrome (KTS) has since been used to describe rare, congenital, vascular malformations identified by the classic clinical triad of venous varicosities, ipsilateral cutaneous capillary malformations, and bony/soft-tissue overgrowth of the affected limb or limbs. As our understanding of vascular lesions evolves, it is clear that the constellation of findings reported by Klippel and Trenaunay represents complex, mixed, vascular malformations, often with venous, capillary, and lymphatic components, with various clinical consequences, including hypercoagulability and the complications that ensue. We describe 2 children with KTS of the lower extremity who suffered from pulmonary embolism (PE). CASE REPORTS Patient 1A 14-year-old boy with a combined capillary-venous-lymphatic malformation of the right lower extremity who had been diagnosed as having KTS at birth presented to an outside institution after 2 episodes of syncope. During these episodes, he was unresponsive for several minutes and was observed to have circumoral cyanosis and tonic posturing. Two weeks earlier, the child had been treated for cellulitis, with fever, pain, and warmth of his affected extremity; blood cultures were positive for Staphylococcus aureus, and the patient was treated with a course of orally administered cephalexin. After the second episode of syncope, the patient was transferred to Children's Hospital of Wisconsin (Milwaukee, WI) for evaluation.In the physical examination, the child was pale and lethargic, with a heart rate of 119 beats per minute, a respiratory rate of 28 breaths per minute, and oxygen saturation ranging from 93% to 97% with room air. A cardiac examination revealed a new grade II/VI systolic murmur. The lungs were clear. The right lower extremity was enlarged, with 2ϩ edema at the ankle to mid-shin level, which was its usual size, according to the boy's family. There was an extensive red-purple patch extending from the right buttock to the lateral ankle (Fig 1). There were hundred...

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“…There was no such evidence suggesting bleeding diasthesis which may sometimes be seen to be of kasabachMerritt variety as by Inceman et al in 1969.…”

Section: Discussionmentioning

confidence: 92%