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转移性直肠神经内分泌癌伴治疗抵抗性免疫性血小板减少症:病例报告及文献复习。

Metastatic rectal neuroendocrine carcinoma presenting with treatment-refractory immune thrombocytopenia: A case report and literature review.

机构信息

Internal Medicine, Howard University Hospital, Washington, DC.

出版信息

Medicine (Baltimore). 2022 Jul 22;101(29):e29616. doi: 10.1097/MD.0000000000029616.

DOI:10.1097/MD.0000000000029616
PMID:35866784
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9302362/
Abstract

RATIONALE

Colorectal neuroendocrine cancer (CRNEC) accounts for < 1% of all colorectal cancers. It presents in advanced stages and carries a risk of poor survival. CRNEC may be diagnosed incidentally on imaging or routine endoscopy. When symptomatic, it may mimic colorectal adenocarcinoma. Syncope and immune thrombocytopenic purpura (ITP) have not previously been reported as clinical presentations.

PATIENT CONCERNS

A 57-year-old man with human immunodeficiency virus infection on treatment, seizure and stroke without residue presented for the evaluation of fall and syncope.

DIAGNOSIS

Physical examination revealed tachycardia, swelling, and ecchymosis of his proximal left lower extremity. Laboratory tests showed a new isolated thrombocytopenia of 26,000/mm3. Computed tomography for a trauma survey showed an incidental left posterior rectal wall mass. After hospital admission, his platelet count dropped to 14,000/mm3. A peripheral blood smear revealed low platelet count, no schistocytes or immature cells. ITP at a high risk for bleeding was diagnosed and treated with standard medical therapy but remained refractory. Bone marrow biopsy showed metastatic neuroendocrine carcinoma, likely from the rectum.

INTERVENTIONS

Patient received courses of high-dose dexamethasone and intravenous immunoglobulin. He also receive eleven units of platelet transfusion. A course of rituximab was administered. The platelet count response was suboptimal or short lived with drop to a nadir of 4000/mm3. However, after initiation of Eltrombopag, the thrombocytopenia resolved. Patient was started on etoposide, carboplatin, Atezolizumab. His hospital stay was complicated by neutropenia and sepsis, which was successfully treated.

OUTCOMES

He was discharged to subacute rehab in stable condition. About 4 months later, he was readmitted for severe thrombocytopenia, septic shock, and acute respiratory failure. Despite appropriate treatment, the patient deteriorated and expired.

CONCLUSION

CRNEC is a rare aggressive disease with dismal outcome that lacks standardized treatment. Metastasis to the bone marrow is uncommon and concomitant ITP has not been reported. We report a rare case of rectal neuroendocrine carcinoma metastatic to bone marrow associated with refractory ITP and review the relevant literature.

摘要

背景

结直肠神经内分泌癌(CRNEC)占所有结直肠癌的比例<1%。它表现为晚期,生存风险高。CRNEC 可能在影像学或常规内镜检查中偶然诊断出来。出现症状时,可能与结直肠腺癌相似。晕厥和免疫性血小板减少性紫癜(ITP)以前并未被报道为临床表现。

病例介绍

一名 57 岁男性,人免疫缺陷病毒感染治疗中,有癫痫发作和中风病史,无后遗症,因跌倒和晕厥就诊。

体格检查发现其左侧下肢近端肿胀、瘀斑。实验室检查发现血小板计数新出现孤立性血小板减少至 26,000/mm3。创伤检查的计算机断层扫描显示左后直肠壁有偶然发现的肿块。住院后,血小板计数降至 14,000/mm3。外周血涂片显示血小板计数低,无裂体细胞或不成熟细胞。诊断为高出血风险的 ITP,并给予标准的药物治疗,但仍未缓解。骨髓活检显示转移性神经内分泌癌,可能来自直肠。

干预措施

患者接受了高剂量地塞米松和静脉注射免疫球蛋白的治疗。他还接受了 11 单位的血小板输注。给予利妥昔单抗治疗。血小板计数反应不理想或短暂,降至最低值 4000/mm3。然而,在开始使用艾曲泊帕后,血小板减少症得到了缓解。患者开始接受依托泊苷、卡铂、阿特珠单抗治疗。他的住院期间还并发了中性粒细胞减少和败血症,经治疗后得到了成功控制。

结果

他稳定出院至亚急性康复中心。大约 4 个月后,他因严重血小板减少症、感染性休克和急性呼吸衰竭再次入院。尽管进行了适当的治疗,患者病情仍恶化并死亡。

结论

CRNEC 是一种罕见的侵袭性疾病,预后极差,缺乏标准化治疗。骨髓转移并不常见,同时伴有 ITP 也尚未有报道。我们报告了一例罕见的直肠神经内分泌癌骨髓转移合并难治性 ITP 的病例,并复习了相关文献。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3e3c/9302362/0284fadc353f/medi-101-e29616-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3e3c/9302362/9d618ae6057a/medi-101-e29616-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3e3c/9302362/7b75c92a8fa6/medi-101-e29616-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3e3c/9302362/18a8b4c94595/medi-101-e29616-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3e3c/9302362/16b09fcd58d5/medi-101-e29616-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3e3c/9302362/0284fadc353f/medi-101-e29616-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3e3c/9302362/9d618ae6057a/medi-101-e29616-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3e3c/9302362/7b75c92a8fa6/medi-101-e29616-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3e3c/9302362/18a8b4c94595/medi-101-e29616-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3e3c/9302362/16b09fcd58d5/medi-101-e29616-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3e3c/9302362/0284fadc353f/medi-101-e29616-g005.jpg

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