Thomas Binu Prathap, Fouzia N A, Raveendran Sreekanth, Pallapati Samuel R, Abraham Aby, Srivastava Alok
Paul Brand Centre for Hand Surgery & Peripheral Nerve Surgery, Christian Medical College & Hospital, Vellore, Tamil Nadu, India.
Department of Hematology, Christian Medical College & Hospital, Vellore, Tamil Nadu, India.
J Hand Surg Am. 2018 May;43(5):486.e1-486.e9. doi: 10.1016/j.jhsa.2017.10.035. Epub 2017 Dec 18.
Hemophilic cysts and pseudotumors (HCPTs) of the hand are rare and are secondary to bleeding disorders such as hemophilia A and B. This is a report of our experience in the management of this rare condition.
Seven male patients with hemophilia A presenting with progressive swelling of the hand were treated between 2004 and 2013 at a tertiary referral hospital. All patients had clotting factor replacement based on our previously reported protocol. The age of the patients ranged from 3 to 49 years (median age, 19 years).
Four patients had soft tissue hemophilic cysts and 3 had bony hemophilic pseudotumors. Two patients had traumatic pseudoaneurysm of the ulnar artery in addition to the cysts. The soft tissue cysts required surgical excision in 3 patients under factor cover as per the protocol. The bony lesions were initially managed nonsurgically by factor replacement, but 2 patients failed to respond and required amputation of the fingers. The ulnar artery aneurysm was excised and artery ligated in 1 patient and the artery was vein grafted owing to poor hand perfusion in 1.
Based on our observations in the management of HCPTs of the hand and the existing literature, we conclude that the soft tissue cysts require surgical excision along with factor replacement and distal bony lesions smaller than 3 cm respond to factor replacement. Larger bony lesions require surgical treatment. Treatment of hemophilic cysts and pseudotumors should be undertaken only in centers with a major hematology backup.
TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic V.
手部血友病性囊肿和假肿瘤（HCPTs）较为罕见，继发于诸如甲型和乙型血友病等出血性疾病。本文报告了我们在处理这种罕见病症方面的经验。
2004年至2013年期间，一家三级转诊医院对7例患有甲型血友病且手部出现进行性肿胀的男性患者进行了治疗。所有患者均根据我们先前报告的方案进行凝血因子替代治疗。患者年龄在3岁至49岁之间（中位年龄19岁）。
4例患者患有软组织血友病性囊肿，3例患有骨血友病性假肿瘤。2例患者除囊肿外还伴有尺动脉创伤性假性动脉瘤。根据方案，3例软组织囊肿患者在凝血因子覆盖下接受了手术切除。骨病变最初通过凝血因子替代进行非手术治疗，但2例患者治疗无效，需要截指。1例患者切除了尺动脉动脉瘤并结扎了动脉，另1例因手部血供不良进行了动脉静脉移植。
基于我们对手部HCPTs的治疗观察及现有文献，我们得出结论，软组织囊肿需要手术切除并进行凝血因子替代治疗，小于3 cm的远端骨病变对凝血因子替代治疗有反应。较大的骨病变需要手术治疗。血友病性囊肿和假肿瘤的治疗应仅在有大型血液学支持的中心进行。
研究类型/证据水平：治疗性研究V。
