Abid Qureshi, Kinjal Kasbawala, Monica T Santos, Christina Cuoccio, Sumeet Bahl, Ahmar A Butt, Phillip Xiao, Romulo Genato, Luca Milone
{"title":"成人镰状细胞病患者脾功能亢进的罕见表现:罕见病例报告","authors":"Abid Qureshi, Kinjal Kasbawala, Monica T Santos, Christina Cuoccio, Sumeet Bahl, Ahmar A Butt, Phillip Xiao, Romulo Genato, Luca Milone","doi":"10.12659/AJCR.944693","DOIUrl":null,"url":null,"abstract":"<p><p>BACKGROUND Hypersplenism, the rapid and premature destruction of blood cells, encompasses a triad of splenomegaly, cytopenias (anemia, leukopenia, or thrombocytopenia), and compensatory bone marrow proliferation. Secondary hypersplenism results from non-intrinsic splenic diseases, such as hemoglobinopathies. Sickle cell disease consists of a group of genotypes, where hemoglobin sickle C disease (HbSC) is the inheritance of hemoglobin S with hemoglobin C. Most homozygous genotypes undergo complete auto-splenectomy by age 6 years, whereas those with HbSC disease rarely do. We report a rare case of hypersplenism and massive splenomegaly in an adult with sickle cell disease, the HbSC genotype, requiring splenectomy. CASE REPORT A 41-year-old woman with known splenomegaly initially presented to the general surgery clinic for management of abdominal pain. She was found to have anemia, indicating cytopenia likely from hypersplenism. Consequently, she underwent splenic artery embolization, followed by an exploratory laparotomy and splenectomy, with an unremarkable postoperative course. CONCLUSIONS Acute splenic sequestration crisis can result from hypersplenism, a potentially fatal complication of sickle hemoglobinemia. The continuous cycle of sickled cell entrapment and stasis causes numerous splenic infarctions, forming splenic parenchymal scar tissue which reduces the spleen's size and functionality - the process of auto-splenectomy. Adults rarely experience these crises past adolescence, which are secondary to the scarring and atrophy from premature auto-splenectomy. Our patient's spleen measured 21.1 cm, larger than the average adult's spleen. In our case, adjunctive preoperative splenic artery embolization likely contributed to decreased intraoperative blood loss during splenectomy, mitigating the need for perioperative transfusions.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0000,"publicationDate":"2024-09-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Uncommon Presentation of Hypersplenism in Adult Sickle Cell Disease Patients: A Rare Case Report.\",\"authors\":\"Abid Qureshi, Kinjal Kasbawala, Monica T Santos, Christina Cuoccio, Sumeet Bahl, Ahmar A Butt, Phillip Xiao, Romulo Genato, Luca Milone\",\"doi\":\"10.12659/AJCR.944693\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>BACKGROUND Hypersplenism, the rapid and premature destruction of blood cells, encompasses a triad of splenomegaly, cytopenias (anemia, leukopenia, or thrombocytopenia), and compensatory bone marrow proliferation. Secondary hypersplenism results from non-intrinsic splenic diseases, such as hemoglobinopathies. Sickle cell disease consists of a group of genotypes, where hemoglobin sickle C disease (HbSC) is the inheritance of hemoglobin S with hemoglobin C. Most homozygous genotypes undergo complete auto-splenectomy by age 6 years, whereas those with HbSC disease rarely do. We report a rare case of hypersplenism and massive splenomegaly in an adult with sickle cell disease, the HbSC genotype, requiring splenectomy. CASE REPORT A 41-year-old woman with known splenomegaly initially presented to the general surgery clinic for management of abdominal pain. She was found to have anemia, indicating cytopenia likely from hypersplenism. Consequently, she underwent splenic artery embolization, followed by an exploratory laparotomy and splenectomy, with an unremarkable postoperative course. CONCLUSIONS Acute splenic sequestration crisis can result from hypersplenism, a potentially fatal complication of sickle hemoglobinemia. The continuous cycle of sickled cell entrapment and stasis causes numerous splenic infarctions, forming splenic parenchymal scar tissue which reduces the spleen's size and functionality - the process of auto-splenectomy. Adults rarely experience these crises past adolescence, which are secondary to the scarring and atrophy from premature auto-splenectomy. Our patient's spleen measured 21.1 cm, larger than the average adult's spleen. In our case, adjunctive preoperative splenic artery embolization likely contributed to decreased intraoperative blood loss during splenectomy, mitigating the need for perioperative transfusions.</p>\",\"PeriodicalId\":39064,\"journal\":{\"name\":\"American Journal of Case Reports\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":1.0000,\"publicationDate\":\"2024-09-20\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"American Journal of Case Reports\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.12659/AJCR.944693\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"MEDICINE, GENERAL & INTERNAL\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"American Journal of Case Reports","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.12659/AJCR.944693","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
引用次数: 0
摘要
背景 脾功能亢进是指血细胞的快速和过早破坏,包括脾脏肿大、细胞减少症(贫血、白细胞减少或血小板减少)和代偿性骨髓增生三联征。继发性脾功能亢进源于非内在性脾脏疾病,如血红蛋白病。镰状细胞病由一组基因型组成,其中血红蛋白镰状 C 病(HbSC)是血红蛋白 S 与血红蛋白 C 的遗传。大多数同型基因型患者在 6 岁前会接受完全的自身脾切除术,而那些患有 HbSC 病的患者很少这样做。我们报告了一例罕见的 HbSC 基因型镰状细胞病成人脾功能亢进和巨大脾肿大病例,患者需要进行脾切除术。病例报告 一名 41 岁女性,已知脾脏肿大,最初因腹痛到普外科门诊就诊。她被发现患有贫血,表明可能是脾功能亢进引起的全血细胞减少。因此,她接受了脾动脉栓塞术,随后进行了探查性开腹手术和脾切除术,术后情况无异常。结论 脾功能亢进可导致急性脾疝危象,这是镰状血红蛋白血症的一种潜在致命并发症。成年人过了青春期后很少会出现这种危机,这是由于过早的自身脾切除造成的瘢痕和萎缩引起的继发性危机。我们患者的脾脏大小为 21.1 厘米,比一般成年人的脾脏要大。在我们的病例中,术前辅助性脾动脉栓塞可能有助于减少脾切除术中的术中失血,从而减轻围手术期输血的需要。
Uncommon Presentation of Hypersplenism in Adult Sickle Cell Disease Patients: A Rare Case Report.
BACKGROUND Hypersplenism, the rapid and premature destruction of blood cells, encompasses a triad of splenomegaly, cytopenias (anemia, leukopenia, or thrombocytopenia), and compensatory bone marrow proliferation. Secondary hypersplenism results from non-intrinsic splenic diseases, such as hemoglobinopathies. Sickle cell disease consists of a group of genotypes, where hemoglobin sickle C disease (HbSC) is the inheritance of hemoglobin S with hemoglobin C. Most homozygous genotypes undergo complete auto-splenectomy by age 6 years, whereas those with HbSC disease rarely do. We report a rare case of hypersplenism and massive splenomegaly in an adult with sickle cell disease, the HbSC genotype, requiring splenectomy. CASE REPORT A 41-year-old woman with known splenomegaly initially presented to the general surgery clinic for management of abdominal pain. She was found to have anemia, indicating cytopenia likely from hypersplenism. Consequently, she underwent splenic artery embolization, followed by an exploratory laparotomy and splenectomy, with an unremarkable postoperative course. CONCLUSIONS Acute splenic sequestration crisis can result from hypersplenism, a potentially fatal complication of sickle hemoglobinemia. The continuous cycle of sickled cell entrapment and stasis causes numerous splenic infarctions, forming splenic parenchymal scar tissue which reduces the spleen's size and functionality - the process of auto-splenectomy. Adults rarely experience these crises past adolescence, which are secondary to the scarring and atrophy from premature auto-splenectomy. Our patient's spleen measured 21.1 cm, larger than the average adult's spleen. In our case, adjunctive preoperative splenic artery embolization likely contributed to decreased intraoperative blood loss during splenectomy, mitigating the need for perioperative transfusions.
期刊介绍:
American Journal of Case Reports is an international, peer-reviewed scientific journal that publishes single and series case reports in all medical fields. American Journal of Case Reports is issued on a continuous basis as a primary electronic journal. Print copies of a single article or a set of articles can be ordered on demand.