Freire Monique, Carvalho Viviane, Spener Renata, da Silva Christiane Rodrigues, da Silva Neto João Ricardo, Carlos Ferreira Luiz, Nogueira Paulo Afonso
Fundação de Medicina Tropical Dr. Heitor Vieira Dourado, Manaus, Brazil.
Programa de Pós-graduação em Ciências da Saúde, Universidade Federal do Amazonas, Manaus, Brazil.
Clin Pathol. 2022 Aug 25;15:2632010X221118059. doi: 10.1177/2632010X221118059. eCollection 2022 Jan-Dec.
Hemophagocytic lymphohistiocytosis (HLH) is a disorder that occurs due to unsuitable monocyte activation in a variety of infections. In human immunodeficiency virus (HIV) infections, patients with advanced immunossupression associated with opportunistic infections are at increased risk of developing HLH. We describe a clinical case of a 33-year-old male student diagnosed with HIV who was hospitalized for investigation of asthenia and dyspnea, accompanied by adynamia, decreased motor force in the left leg, dysphagia, and dysfluency. His general condition was regular, he was pale, feverish, and had normal cardiac and pulmonary auscultation. Physical examination revealed ulcerated lesions in the perianal region and hepatosplenomegaly without palpable lymph node enlargement. Laboratory parameters showed pancytopenia, a slight increase in liver function accompanied by high lactate dehydrogenase, and hiperferritinemia. The initial diagnosis was disseminated histoplasmosis, thus amphotericin B deoxycholate was empirically prescribed while waiting on myeloculture and blood cultures for fungi and mycobacteria. Other clinical procedures were blood transfusion, resumption of antiretroviral therapy (ART) and secondary prophylaxis. Myeloculture blood cultures of fungi and mycobacteria were negative. Patient evolved well in relation to the initial complaints and showed partial clinical and laboratory improvement. However, 23 days after hospitalization, he developed a febrile episode accompanied by chills and a convulsive crisis. The patient was transferred to the intensive unit care and developed septic shock and respiratory failure. He died 25 days after the onset of the condition. After the postmortem examination, histopathology revealed countless rounded fungal structures compatible with sp., which were observed in the peripancreatic lymph node, liver, and spleen, in addition to hemophagocytosis in the splenic parenchyma. We thus conclude that when the patient met criteria for HLH, such as fever, hepatosplenomegaly, hiperferritinemia, and pancytopenia, the evolution was fast due to the aggressive and rapidly fatal nature of HLH, despite anti-fungal and corticoid treatment. Therefore, this case report reinforces the need to consider hemophagocytic syndrome in patients with HIV and disseminated histoplasmosis, especially where histoplasmosis is highly endemic, in order for the treatment be started early when there is high clinical suspicion.
噬血细胞性淋巴组织细胞增生症（HLH）是一种因多种感染中单核细胞活化异常而发生的疾病。在人类免疫缺陷病毒（HIV）感染中，伴有机会性感染的晚期免疫抑制患者发生HLH的风险增加。我们描述了一例临床病例，一名33岁的男学生被诊断为HIV，因乏力和呼吸困难入院检查，伴有肌无力、左腿肌力下降、吞咽困难和言语不流畅。他的一般情况正常，面色苍白、发热，心肺听诊正常。体格检查发现肛周区域有溃疡病变，肝脾肿大，但未触及淋巴结肿大。实验室检查参数显示全血细胞减少，肝功能轻度升高，伴有高乳酸脱氢酶和高铁蛋白血症。初步诊断为播散性组织胞浆菌病，因此在等待真菌和分枝杆菌的骨髓培养及血培养结果期间，经验性给予脱氧胆酸两性霉素B治疗。其他临床措施包括输血、恢复抗逆转录病毒治疗（ART）和二级预防。真菌和分枝杆菌的骨髓培养及血培养结果均为阴性。患者最初的症状有所改善，临床和实验室检查也有部分好转。然而，住院23天后，他出现发热伴寒战和惊厥发作。患者被转入重症监护病房，并发感染性休克和呼吸衰竭。发病25天后死亡。尸检后，组织病理学显示在胰腺周围淋巴结、肝脏和脾脏中发现无数圆形真菌结构，符合荚膜组织胞浆菌，此外在脾实质中还发现噬血细胞现象。因此我们得出结论，当患者符合HLH的标准，如发热、肝脾肿大、高铁蛋白血症和全血细胞减少时，尽管进行了抗真菌和皮质类固醇治疗，但由于HLH具有侵袭性和快速致命的特点，病情进展迅速。因此，本病例报告强调，在HIV和播散性组织胞浆菌病患者中，尤其是在组织胞浆菌病高度流行的地区，当临床高度怀疑时，需要考虑噬血细胞综合征，以便尽早开始治疗。
