METHODS: Here, we present a case of a 39-year-old man with a rapidly enlarging 25-cm GHH arising from the entire left hemiliver. Laboratory findings suggested pancytopenia but normal liver and renal functions. He was diagnosed with Kasabach-Merritt syndrome (KMS). After three units of aphaeretic platelet transfusion, the patient underwent left hepatectomy. Postoperative recovery was uneventful, and his regular follow-up revealed no recurrence even after two years.
UNASSIGNED: HH predominantly affects females, but males can also be affected, as seen in this case. With observation, it can grow over time, particularly in patients under 50 years of age. Surgical management should be considered when HH causes symptoms or is larger than 10 cm. The evolving understanding of GHH and the critical role of surgery are important, particularly when they complicate haematological or coagulation profiles and lead to thrombocytopenia.
CONCLUSIONS: Our case report highlights the significance of surgical intervention in GHH, and a disease-free outcome can be expected for patients with this condition in the future. To our knowledge, this is the first such case report from Bangladesh.
方法:这里,我们介绍了一例39岁男子的病例,该男子的GHH迅速扩大了25厘米,由整个左半侧引起。实验室检查结果提示全血细胞减少,但肝肾功能正常。他被诊断出患有Kasabach-Merritt综合征(KMS)。输注三个单位的失语症血小板后,患者接受了左肝切除术。术后恢复顺利,他的定期随访显示,即使两年后也没有复发。
■HH主要影响女性,但是男性也会受到影响,正如在这个案例中看到的。有了观察,它可以随着时间的推移而增长,特别是50岁以下的患者。当HH引起症状或大于10cm时,应考虑手术治疗。对GHH的不断发展的理解和手术的关键作用非常重要,特别是当它们使血液学或凝血功能复杂化并导致血小板减少时。
结论:我们的病例报告强调了GHH手术干预的重要性,并且可以预期将来患有这种疾病的患者的无病结局。据我们所知,这是孟加拉国的首例此类病例报告。