Hundal Jasmin, Bowers David, Gadela Naga Vaishnavi, Jaiswal Abhishek
Department of Internal Medicine, University of Connecticut, Farmington, Connecticut, United States of America.
Department of Cardiology, Hartford Hospital Heart and Vascular Institute, Hartford, Connecticut, United States of America.
Indian J Crit Care Med. 2022 Aug;26(8):970-973. doi: 10.5005/jp-journals-10071-24284.
Acute respiratory distress syndrome (ARDS) is an uncommon complication of hemophagocytic lymphohistiocytosis (HLH). Non-specific findings that mimic other diseases make timely diagnosis and treatment challenging. We present a rare case of severe ARDS and multiorgan failure from secondary HLH due to peripheral T-cell lymphoma.
A middle-aged female presented with dry cough and fever for three days. On presentation, the patient was febrile to 105°F and hypoxic to 88% on room air. Chest X-ray showed bilateral interstitial infiltrates. Laboratory investigations showed lymphopenia and elevated inflammatory markers. The viral panel, including coronavirus disease-2019 (COVID-19), influenza, and respiratory syncytial virus (RSV), was negative. Her respiratory status progressively worsened, requiring invasive mechanical ventilation for ARDS. Despite lung-protective ventilation, prone positioning, and the use of paralytic agents, the patient continued to remain hypoxic, necessitating extracorporeal membrane oxygenation (ECMO) support. The patient was started on antibiotics and high-dose steroid. Thereafter, she developed a leukemoid reaction, and the ferritin level started rising; raising suspicion for lymphophagocytosis. During this time, she also developed acute liver and kidney failure and required multiple vasopressors and renal replacement therapy. Eventually, a diagnosis of mature peripheral T-cell lymphoma was established. Subsequently, her respiratory status and multiorgan failure significantly improved, and ECMO was explanted after 2 weeks. She was started on etoposide and steroid, and eventually discharged after 6 weeks.
This is the first case describing a successful implementation of ECMO in an adult diagnosed with ARDS secondary to mature peripheral T-cell lymphoma; allowing for recovery of respiratory status, which was compromised during the initial cytokine storm and provided time to establish the diagnosis and initiate appropriate treatment of secondary HLH mature due to peripheral T-cell lymphoma, and in the end, prevented a fatality. We believe that ECMO may be appropriately instituted in rapidly deteriorating patients with an unknown illness refractory to conventional therapy, to allow for end-organ recovery, to reach a diagnosis, and to administer appropriate therapy.
Hundal J, Bowers D, Gadela NV, Jaiswal A. Rare Case of Refractory Hypoxia and Severe Multiorgan Failure from Secondary Lymphohistiocytosis Successfully Bridged to Treatment with Extracorporeal Membrane Oxygenation Support. Indian J Crit Care Med 2022;26(8):970-973.
This is a case report and does not contain any images or patient identifying information.
急性呼吸窘迫综合征（ARDS）是噬血细胞性淋巴组织细胞增生症（HLH）的一种罕见并发症。一些非特异性表现与其他疾病相似，这使得及时诊断和治疗具有挑战性。我们报告一例罕见的因外周T细胞淋巴瘤继发HLH导致严重ARDS和多器官功能衰竭的病例。
一名中年女性出现干咳和发热3天。就诊时，患者体温高达105°F（40.6°C），在室内空气中氧饱和度低至88%。胸部X线显示双侧间质性浸润。实验室检查显示淋巴细胞减少和炎症标志物升高。包括2019冠状病毒病（COVID-19）、流感和呼吸道合胞病毒（RSV）在内的病毒检测均为阴性。她的呼吸状况逐渐恶化，因ARDS需要有创机械通气。尽管采用了肺保护性通气、俯卧位通气和使用麻痹剂，患者仍持续低氧，需要体外膜肺氧合（ECMO）支持。患者开始使用抗生素和大剂量类固醇。此后，她出现类白血病反应，铁蛋白水平开始升高；这增加了对噬血细胞现象的怀疑。在此期间，她还出现了急性肝肾功能衰竭，需要多种血管活性药物和肾脏替代治疗。最终，确诊为成熟外周T细胞淋巴瘤。随后，她的呼吸状况和多器官功能衰竭明显改善，2周后撤除了ECMO。她开始使用依托泊苷和类固醇，最终在6周后出院。
这是首例描述在一名诊断为继发于成熟外周T细胞淋巴瘤的ARDS成人患者中成功实施ECMO的病例；使呼吸状况得以恢复，其在最初的细胞因子风暴期间受到损害，并为确立诊断和启动针对外周T细胞淋巴瘤继发HLH成熟型的适当治疗争取了时间，最终避免了死亡。我们认为，对于常规治疗无效且病情迅速恶化的不明病因患者，可适当采用ECMO，以促进终末器官恢复、明确诊断并给予适当治疗。
Hundal J, Bowers D, Gadela NV, Jaiswal A. 罕见病例：继发于淋巴细胞组织细胞增生症的难治性低氧和严重多器官功能衰竭经体外膜肺氧合支持成功过渡到治疗。《印度重症监护医学杂志》2022；26(8):970 - 973。
这是一篇病例报告，不包含任何图像或患者识别信息。
