Kumar Vivek, Soni Parita, Dave Vishangi, Harris Jonathan
Maimonides Medical Center, Brooklyn, NY, USA.
J Investig Med High Impact Case Rep. 2017 Feb 1;5(1):2324709617690748. doi: 10.1177/2324709617690748. eCollection 2017 Jan-Mar.
A 67-year-old man presented with a 3-day history of abdominal pain, fever, and significant weight loss over 2 months. Physical examination revealed left upper quadrant tenderness, hepatomegaly, splenomegaly, and bilateral pitting edema but peripheral lymphadenopathy was absent. Laboratory tests showed anemia, thrombocytopenia, elevated prothrombin time (PT), partial thromboplastin time (PTT), and increased lactate dehydrogenase (LDH). PTT was corrected completely in mixing study. Further workup for the cause of coagulopathy revealed decreased levels of all clotting factors except factor VIII and increase fibrinogen levels, which ruled out disseminated intravascular coagulation (DIC). Flow cytometry of peripheral blood was normal. Contrast-enhanced computed tomography (CECT) revealed splenomegaly with multiple splenic infarcts without any mediastinal or intraabdominal lymphadenopathy. Further investigations for infective endocarditis (blood cultures and transthoracic echocardiography) and autoimmune disorders (ANA, dsDNA, RA factors) were negative. The patient received treatment for sepsis empirically without any significant clinical improvement. The diagnosis remained unclear despite extensive workup and liver biopsy was conducted due to high suspicion of granulomatous diseases. However, the liver biopsy revealed high-grade diffuse large B-cell lymphoma (DLBCL). Unfortunately, patient died shortly after the diagnosis. Here we report a case of high-grade DLBCL with hepatosplenomegaly and splenic infarcts in the absence of any lymphadenopathy or focal lesions. This case highlights the fact that unusually lymphoma can present in the absence of lymphadenopathy or mass lesion mimicking autoimmune and granulomatous disorders. The diagnosis in these cases can only be made on histology, and hence the threshold for biopsy should be low in patients with unclear presentations and multiorgan involvement.
一名67岁男性，有3天腹痛病史，发热，且在2个月内体重显著减轻。体格检查发现左上腹压痛、肝肿大、脾肿大和双侧凹陷性水肿，但无外周淋巴结病。实验室检查显示贫血、血小板减少、凝血酶原时间（PT）升高、部分凝血活酶时间（PTT）升高以及乳酸脱氢酶（LDH）升高。PTT在混合试验中完全纠正。对凝血病病因的进一步检查发现，除因子VIII外所有凝血因子水平降低，纤维蛋白原水平升高，排除了弥散性血管内凝血（DIC）。外周血流式细胞术正常。对比增强计算机断层扫描（CECT）显示脾肿大伴多发脾梗死，无任何纵隔或腹腔内淋巴结病。对感染性心内膜炎（血培养和经胸超声心动图）和自身免疫性疾病（抗核抗体、双链DNA、类风湿因子）的进一步检查均为阴性。患者接受了经验性脓毒症治疗，但无明显临床改善。尽管进行了广泛检查，诊断仍不明确，由于高度怀疑肉芽肿性疾病，进行了肝活检。然而，肝活检显示为高级别弥漫性大B细胞淋巴瘤（DLBCL）。不幸的是，患者在诊断后不久死亡。在此我们报告一例无任何淋巴结病或局灶性病变的伴有肝脾肿大和脾梗死的高级别DLBCL病例。该病例突出了这样一个事实，即非典型淋巴瘤可在无淋巴结病或肿块病变的情况下出现，类似自身免疫性和肉芽肿性疾病。这些病例的诊断只能依靠组织学，因此对于表现不明确且有多器官受累的患者，活检的阈值应较低。
