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镰状细胞β-珠蛋白生成障碍性贫血伴脾包膜下血肿

Sickle Cell Beta-Plus Thalassemia with Subcapsular Hematoma of the Spleen.

作者信息

Dahal Suyash, Dahal Sumit, Ghimire Dipesh K C, Ur Rahman Ebad, Sharma Eliza

机构信息

KIST Medical College and Teaching Hospital, Lalitpur, Nepal.

Interfaith Medical Center, Brooklyn, NY, USA.

出版信息

Case Rep Hematol. 2017;2017:3819457. doi: 10.1155/2017/3819457. Epub 2017 Dec 14.

Abstract

While splenic complications like hypersplenism, sequestration crisis, and infarction are commonly reported in sickle cell variants like sickle cell beta-plus thalassemia, splenic rupture with hematoma is rare. We present a case of a 32-year-old young male who presented with dull left upper quadrant pain who was found to have multiple subcapsular splenic lacerations and hematoma on abdominal imaging. Hemoglobin electrophoresis confirmed sickle cell beta-plus thalassemia in the patient. There was no history of trauma, and rest of the workup for possible cause of spontaneous rupture of spleen was negative. With the patient refusing splenectomy, he was managed conservatively. Clinicians need to be aware of this rare complication of sickle cell variants.

摘要

虽然像脾功能亢进、脾滞留危象和梗死等脾脏并发症在镰状细胞β-珠蛋白生成障碍性贫血等镰状细胞变体中普遍有报道,但脾破裂伴血肿却很罕见。我们报告了一例32岁的年轻男性病例,该患者因左上腹隐痛就诊,腹部影像学检查发现脾脏有多处包膜下裂伤和血肿。血红蛋白电泳确诊该患者为镰状细胞β-珠蛋白生成障碍性贫血。患者无外伤史,对脾脏自发性破裂可能原因的其余检查结果均为阴性。由于患者拒绝脾切除术,故对其进行保守治疗。临床医生需要了解镰状细胞变体的这种罕见并发症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d2d3/5745719/cc875a3746b6/CRIHEM2017-3819457.001.jpg

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