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一例严重血小板减少、全身性水肿、发热、肾功能不全或网状纤维组织增生以及尽管存在低钙血症但伴有心肌和骨骼肌钙化的器官肿大综合征:病例报告

A severe case of thrombocytopenia, anasarca, fever, renal insufficiency or reticulin fibrosis, and organomegaly syndrome with myocardial and skeletal muscle calcification despite hypocalcemia: a case report.

作者信息

Minomo Shogo, Fujiwara Yu, Sakashita Shota, Takamura Akito, Nagata Kaoru

机构信息

Department of General Internal Medicine, Musashino Red Cross Hospital, 1-26-1, Kyonancho, Musashino-shi, Tokyo, 1808610, Japan.

Department of Medicine, Icahn School of Medicine at Mount Sinai, Mount Sinai Beth Israel, 281 First Avenue, New York, NY, 10003, USA.

出版信息

J Med Case Rep. 2021 Jan 6;15(1):3. doi: 10.1186/s13256-020-02588-2.

Abstract

BACKGROUND

TAFRO (thrombocytopenia, anasarca, fever, renal insufficiency or reticulin fibrosis, and organomegaly) syndrome is a recently recognized disease with a variety of presentations of variable severity. In acute settings, this disease also involves organ dysfunction because of the associated systemic inflammation. However, cases of TAFRO syndrome with myocardial and/or skeletal muscle calcification have never been reported.

CASE PRESENTATION

A 24-year-old healthy young Asian man was admitted with intermittent epigastric pain and fever for 2 weeks. Computed tomography revealed pleural effusion, ascites and systemic lymphadenopathy. Laboratory tests showed thrombocytopenia, elevated C-reactive protein, hypoalbuminemia, anemia and renal dysfunction. Based on these findings and bone marrow biopsy, we diagnosed his disease as TAFRO syndrome and commenced hemodialysis for the renal dysfunction. However, he developed refractory hypocalcemia with unstable vital signs, for which we administered calcium gluconate hydrate. Thereafter, myocardial and skeletal muscle calcification was revealed radiologically, with the myocardial calcification causing sick sinus syndrome. He was treated with tocilizumab and finally discharged in an ambulatory condition after prolonged hospitalization, with residual calcific lesions.

CONCLUSION

This is the first report of a patient with TAFRO syndrome and the complication of organ calcification. The etiology of calcification in this case is not clear. Systemic inflammation with possible hypercytokinemia might have been involved in the unexpected complication of systemic calcification. It is important to carefully handle the general management of TAFRO syndrome because of the possibility of various complications.

摘要

背景

TAFRO(血小板减少、全身性水肿、发热、肾功能不全或网状纤维增生以及器官肿大)综合征是一种最近才被认识的疾病,有多种不同严重程度的表现形式。在急性情况下,这种疾病还会因相关的全身炎症而导致器官功能障碍。然而,从未有过TAFRO综合征合并心肌和/或骨骼肌钙化的病例报道。

病例介绍

一名24岁健康的亚洲年轻男性因间歇性上腹部疼痛和发热2周入院。计算机断层扫描显示有胸腔积液、腹水和全身淋巴结肿大。实验室检查显示血小板减少、C反应蛋白升高、低白蛋白血症、贫血和肾功能不全。基于这些发现以及骨髓活检结果,我们将他的疾病诊断为TAFRO综合征,并因肾功能不全开始进行血液透析。然而,他出现了难治性低钙血症且生命体征不稳定,为此我们给予了水合葡萄糖酸钙治疗。此后,影像学检查发现了心肌和骨骼肌钙化,心肌钙化导致了病态窦房结综合征。他接受了托珠单抗治疗,经过长时间住院后最终以非卧床状态出院,仍留有钙化病灶。

结论

这是首例关于TAFRO综合征合并器官钙化并发症的病例报告。该病例中钙化的病因尚不清楚。全身性炎症伴可能的高细胞因子血症可能与全身性钙化这一意外并发症有关。由于可能出现各种并发症,因此谨慎处理TAFRO综合征的综合管理非常重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/475e/7786502/fe8082d48ceb/13256_2020_2588_Fig1_HTML.jpg

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