儿童噬血细胞综合征26例实验室检查分析

目的探讨儿童噬血细胞综合征的临床特点。方法回顾性分析26例感染相关性噬血细胞综合征病的临床及实验室资料。结果感染相关性17例(65.38%),非感染相关性3例(11.54%),原因不明6例(23.08%)。全组病例临床主要特点为持续高热(100.0%)、肝肿大(84.62%)、脾肿大(92.31%);外周血白细胞计数减少(76.92%)、血小板减少(88.46%)、血红蛋白减少(80.77%);骨髓涂片找到噬血细胞(100.0%)。结论儿童感染相关性噬血细胞综合征临床以持续高热、肝肿脾大为主要表现,实验室检查白细胞计数、血红蛋白及血小板中两系或三系减少、低纤维蛋白血症、高脂血症、肝功能、心功能异常及血清铁蛋白升高,骨髓找到噬血细胞等为主要特点。     

系统性红斑狼疮合并噬血细胞综合征

18岁女性患者,以腹痛、发热起病,进而出现肝酶升高及全血细胞减少、癫痫发作等,腹部CT提示假性肠梗阻,骨髓细胞学检查有噬血细胞,血清学检查抗核抗体阳性,EB病毒感染再激活。最终确诊为系统性红斑狼疮、EB病毒感染,并发假性肠梗阻和噬血细胞综合征。     

反应性噬血细胞综合征和系统性红斑狼疮

反应性噬血细胞综合征 (ｒｅａｃｔｉｖｅｈｅｍｏｐｈａｇｏｃｙｔｉｃｓｙｎ ｄｒｏｍｅ)是一从骨髓细胞学角度提出的 ,且有其一系列临床特征的综合征。主要表现为发热 ,肝脾淋巴结肿大和血细胞减少 ,最显著的特征是骨髓组织细胞良性增生且活跃吞噬各种血细胞。病因各异 ,常见的有感染、肿瘤及免疫缺陷病等。近年来 ,系统性红斑狼疮 (ＳＬＥ)并发反应性噬血细胞综合征的报道陆续见于西方报端 ,国内尚未见报道 ,但普遍认为是一种少见现象。我们复习本院十年来所收的 2 2 7例ＳＬＥ病案 ,发现 10例反应性噬血细胞综合征 ,现报道如下1　资料…     

结核性毁损肺外科胸膜全肺切除手术后ICU治疗28例分析

目的提高对结核性毁损肺行全肺切除患者的围手术期ICU的治疗水平。方法对结核性毁损肺28例患者,于手术后住ICU监测治疗:动态监测生命体征、出血情况、肺功能、血气分析;治疗合并疾病;纤维支气管镜吸痰;应用呼吸机进行无创或有创机械通气,治疗急性呼吸衰竭。结果20例患者经过ICU加强诊疗,术后顺利恢复;8例患者出现急性呼吸衰竭,经过无创及有创呼吸机机械通气治疗后好转;2例合并胸腔感染、支气管残端瘘患者,其中1例死亡。结论结核性毁损肺胸膜全肺切除手术后病人的围手术期诊疗中,在动态监测病情、防治手术后应激反应、治疗手术后急性呼吸衰竭等方面ICU有明显优势。     

结核性毁损肺外科胸膜全肺切除手术后ICU治疗28例分析

目的 提高对结核性毁损肺行全肺切除患者的围手术期ICU的治疗水平.方法 对结核性毁损肺28例患者,于手术后住ICU监测治疗：动态监测生命体征、出血情况、肺功能、血气分析;治疗合并疾病;纤维支气管镜吸痰;应用呼吸机进行无创或有创机械通气,治疗急性呼吸衰竭.结果 20例患者经过ICU加强诊疗,术后顺利恢复;8例患者出现急性呼吸衰竭,经过无创及有创呼吸机机械通气治疗后好转;2例合并胸腔感染、支气管残端瘘患者,其中1例死亡.结论 结核性毁损肺胸膜全肺切除手术后病人的围手术期诊疗中,在动态监测病情、防治手术后应激反应、治疗手术后急性呼吸衰竭等方面ICU有明显优势.     

结核性毁损肺外科胸膜全肺切除手术后ICU治疗28例分析

目的提高对结核性毁损肺行全肺切除患者的围手术期ICU的治疗水平。方法对结核性毁损肺28例患者，于手术后住ICU监测治疗：动态监测生命体征、出血情况、肺功能、血气分析；治疗合并疾病；主动纤维支气管镜吸痰；应用呼吸机进行无创或有创机械通气，治疗急性呼吸衰竭。结果20例患者经过ICU加强诊疗，术后顺利恢复；8例患者出现急性呼吸衰竭．经过无创及有创呼吸机机械通气治疗后好转；2例患者合并胸腔感染、支气管残端瘘，其中1例死亡。结论结核性毁损肺胸膜全肺切除手术后病人的围手术期诊疗中，在动态监测病情、防治手术后应激反应、治疗手术后急性呼吸衰竭等方面ICU有明显优势。     

慢性阻塞性肺疾病重度及极重度加重期并发心律失常临床分析

目的探讨慢性阻塞性肺疾病重度和极重度加重期并发心律失常的原因。方法回顾分析368例该病临床资料。结果加重期180例(48.9%),大多数以室上性心律失常为主,占76.3%;心律失常的治疗出现及严重程度与肺部疾病感染、低氧血症、心力衰竭、电解质紊乱密切相关。结论慢性阻塞性肺疾病并发心律失常的治疗应采取控制感染、改善通气功能、纠正心力衰竭、调节水电解质及酸碱失衡等措施。     

以肝功能损伤为首发表现的妊娠合并噬血细胞综合征1例

噬血细胞综合征是由免疫功能紊乱引起的一种致命性的临床综合征。主要表现为发热、全血细胞减少及脾大,可在骨髓或其他组织发现噬血细胞现象,其诊断主要依据临床表现及实验室的相关检查结果。本文报道1例以肝功能损害为首发表现的妊娠合并噬血细胞综合征患者,经糖皮质激素联合免疫球蛋白治疗后病情好转。     

继发于内脏利什曼病的噬血细胞综合征1例

噬血细胞综合征发展迅猛、病死率高, 需要早期诊断和治疗。在该病的诊断中, 应及时发现疑似病例并积极寻找潜在病因。内脏利什曼病并发噬血细胞综合征病例报道少见, 本研究报道1例继发于内脏利什曼病的噬血细胞综合征患者, 经应用糖皮质激素及葡萄糖酸锑钠等治疗后好转出院。通过病例讨论旨在加深对内脏利什曼病继发噬血细胞综合征诊治的理解, 在工作中对流行区患者要考虑到少见病原体感染的可能, 争取早期诊断和治疗。     

慢性阻塞性肺疾病急性加重合并呼吸衰竭48例临床分析

慢性阻塞性肺疾病急性加重（AECOPD）是导致患者死亡的主要原因,住院的AECOPD患者病死率高达11%,入住ICU的病死率为11%～20%[1,2]。COPD病情反复,往往因感染等因素而急性加重,并发呼吸衰竭、肺性脑病等,不及时救治,常危及生命。     

A case of spontaneous regression of malignant lymphoma, complicated with miliary tuberculosis, liver dysfunction and pancytopenia

An 82-year-old woman was admitted to Iwamizawa Rosai Hospital, Cardiovascular Medicine, for detailed examination of an aneurysm of abdominal aorta. CT scan revealed marked swelling of para-aortic lymph nodes which regressed spontaneously in three months. She was re-admitted to our hospital because of general malaise and gait disturbance. Her chest X-ray on the second admission revealed miliary disseminated shadows, which were confirmed to be tuberculous granuloma by lung biopsy. She was initially well controlled with anti-tuberculosis drugs, followed by severe liver dysfunction and pancytopenia, and died of respiratory and cardiac failure. At autopsy, wide-spread malignant lymphoma and miliary tuberculosis of the lung were found. The cause of liver dysfunction was strongly suspected to be due to infiltration of the lymphoma cells to portal triads of the liver. Hemophagocytosis found in the bone marrow, spleen and liver might be consistent with clinically so-called "hemophagocytic syndrome", causing pancytopenia, associated with infection.     

A case of hemophagocytic syndrome caused by tuberculosis]

A 59-year-old woman was admitted to our hospital with a picture of high fever and dyspnea. Pneumonia was diagnosed by chest CT scan and was treated. Laboratory findings showed pancytopenia and examination of aspirated bone marrow showed mature histiocytes with marked hemophagocytosis. She was treated with prednisolone, but her respiratory condition worsened, so she was ventilated. An acid-fast bacterial smear of bronchoalveolar lavage fluid from the left lingula showed Gaffky's scale No. 5, and so tuberculosis was diagnosed. Though anti-tuberculosis therapy was started, she died after eight days of hospitalization. Infection-associated hemophagocytic syndrome is caused by various infections. Until now, 26 cases of tuberculosis-associated hemophagocytic syndrome have been reported, and many of these diseases were caused by severe tuberculosis. If a patient with severe tuberculosis presents with leucocytopenia or thrombocytopenia, investigations should serve a differential diagnosis that takes into account disseminated intravascular coagulation, tuberculous granuloma of the bone marrow, side effects of anti-tuberculous drugs, and hypersplenism from hemophagocytic syndrome.     

Virus associated hemophagocytic syndrome accompanied by acute respiratory failure caused by influenza A (H3N2)

A 40-year-old Japanese woman was admitted to Oita University Hospital with progressive dyspnea, consciousness disturbance and severe cytopenias. Her chest roentgenogram showed diffuse bilateral infiltrates. She was therefore forced to receive mechanical ventilation. Bone marrow aspiration disclosed numerous hemophagocytic histiocytes, thus suggesting her condition to be hemophagocytic syndrome. In addition, she also developed myocarditis and renal failure. Pulsed methylprednisolone, gamma-globulin, granulocyte colony-stimulating factor and sivelestat sodium hydrate were administrated, and thereafter the patient recovered from cytopenia and organ failure. Afterwards, influenza A H3N2 was detected from bronchial extracts. We should recognize that an influenza A virus infection can induce hemophagocytic syndrome and acute respiratory failure as the initial manifestations of multiple organ failure.     

Life-threatening hemophagocytic syndrome triggered by disseminated toxoplasmosis in a young patient with previously unknown AIDS

Hemophagocytic syndrome is a rare life-threatening disorder that can be triggered by various conditions such as HIV infection and opportunistic agents. We report a case of disseminated toxoplasmosis complicated with severe hemophagocytic syndrome and revealing an unknown acquired immunodeficiency syndrome. The patient presented with multiple organ failure in intensive care unit. Once diagnosed, he benefitted from etoposide infusion, administration of specific anti-toxoplasmosis treatments and secondary antiretroviral therapy. He was alive at intensive care unit discharge and returned home with little sequalae. This case illustrates both the importance of rapid investigations of hemophagocytic syndrome etiologies in HIV positive patients and the necessity to prompt etoposide and specific treatments in order to improve potentially dramatic outcomes.     

A case of new type scrub typhus (tsutsugamushi disease) presenting with acute respiratory failure and hemophagocytic syndrome]

A 47-year-old male was referred to our hospital with persistent high fever. He had received antibiotics (ASPC) for the diagnosis of acute infectious disease, which failed to respond, and subsequently developed marked lymphocytopenia and thrombocytopenia. During his hospitalization, hypoxic respiratory failure rapidly developed. A bone marrow aspiration revealed marked hemophagocytosis, leading to the tentative diagnosis of opportunistic respiratory infection associated with malignant histiocytosis. Treatment for suspected malignancy and Pneumocystis carinii pneumonia was commenced. However, because of the initial lack of response to ASPC, generalized skin rashes without an ordinary stick wound which had diminished before the hospitalization, and a history of working outdoors in a nearby mountain area, the possibility of new type tsutsugamushi disease was strongly suggested. Intravenous administration of minocycline promptly improved his symptoms and laboratory data. The diagnosis was confirmed by a significant increase in the titer of antibody to Rickettsia tsutsugamushi. The thrombocytopenia was considered to have been caused by co-existent hemophagocytic syndrome.     

Secondary pulmonary alveolar proteinosis occurring in two patients with acquired immune deficiency syndrome

This report describes two patients with acquired immune deficiency syndrome (AIDS) in whom respiratory failure and opportunistic infection associated with secondary alveolar proteinosis developed. In one patient, the alveolar proteinosis was apparently secondary to Mycobacterium tuberculosis and in the other to Pneumocystis carinii and cytomegalovirus infection. Both patients died of respiratory failure, and it was suspected that secondary alveolar proteinosis could have been a contributing cause of death.     

Drug-induced liver injury with autoimmune features complicated with hemophagocytic syndrome

A 60-year-old woman was admitted to our hospital with non-coma acute liver failure. Based on a 1-month history of supplement use, negative viral hepatitis markers, positive antinuclear antibody test, high IgG level, positive HLA-DR4, liver biopsy findings of centrizonal necrosis, and inflammatory cell infiltration in the portal area, she was diagnosed with drug-induced liver injury (DILI) with autoimmune features or the acute hepatitis phase of autoimmune hepatitis (AIH). Although her liver disorder was ameliorated by administration of prednisolone and plasma exchange, anemia and thrombocytopenia were observed during the course of treatment. A bone marrow examination showed hemophagocytosis. Therefore, with no other evidence suggesting infection or malignancy, we determined that the patient had DILI complicated by hemophagocytic syndrome (HPS). Although HPS is very rarely seen in patients with DILI with autoimmune features or the acute hepatitis phase of AIH, this condition should be considered if cytopenia is observed in a patient with DILI.     

A case of hemophagocytic lymphohistiocytosis after the primary Epstein-Barr virus infection.

The Epstein-Barr virus (EBV) infection is known to result in infectious mononucleosis, hemophagocytic syndrome, chronic active EBV infection, and lymphoma. Among them, hemophagocytic syndrome sometimes causes thrombocytopenia, which is often life threatening because of its hemorrhagic complications such as gastrointestinal bleeding and pulmonary alveolar hemorrhage. A young adult case of critical hemophagocytic syndrome after primary EBV infection is presented. Chemotherapy was performed using methyl prednisolone succinate, prednisolone, cyclosporin A, and 20 mg/kg of cyclophosphamide. The patient received intensive care, including plasma exchange for hepatic failure, extracorporeal membrane oxygenation for acute respiratory failure, and splenectomy for hemophagocytosis; however, the patient died of multiple organ failure, including fulminant hepatic failure. The pathologic examination of the resected specimen demonstrated infiltrated macrophages containing many phagocytosed erythrocytes. Further immunopathologic examination of these cells showed that the histiocyte markers were positive, whereas the T-cell marker was negative. In view of these findings, definite diagnosis of EBV-related hemophagocytic lymphohistiocytosis could not be made at that time. The immunohistologic examinations on the liver necropsy specimen provided the evidences suggesting the morbid activation of the hepatic stellate macrophage by EBV-infected T/NK cells and subsequent apoptosis induction of the liver cells through the Fas ligand pathway.     

Transient blood plasmacytosis in parvovirus B19 infection: a report of two cases

Two patients with sickle cell disease were diagnosed with aplastic crisis caused by acute parvovirus B19 infection. One patient also developed thrombocytopenia associated with hemophagocytic histiocytosis in the bone marrow biopsy. Both patients developed transient blood plasmacytosis and hypocomplementemia soon after admission to the hospital. Transient blood plasmacytosis may be an immunological response to acute parvovirus B19 infection.