Pulmonary hypertension and human immunodeficiency virus infection. Two reports and a review of the literature.

Pulmonary hypertension may be primary (idiopathic) or secondary. While the etiologies for secondary pulmonary hypertension are diverse, infection with the human immunodeficiency virus (HIV) has not been included. To date there have been 16 reported cases of pulmonary hypertension in the HIV-infected population. Plexogenic arteriopathy was the most common pathologic finding. We report two HIV-infected patients who were concomitantly found to have pulmonary hypertension with plexogenic arteriopathy. One patient had lymphocytic interstitial pneumonitis, an entity not previously associated with pulmonary hypertension. We review the 16 previous cases of pulmonary hypertension and HIV infection and discuss this association.     

Myelopathy revealing lupus. Two cases and review of the literature

INTRODUCTION: Myelopathy is a rare manifestation of systemic lupus erythematosus, occurring most often during the course of the disease. EXEGESIS: We report two cases of women with myelopathy as the first manifestation of systemic lupus erythematosus; both had an unusual course. We review the literature for previously reported cases. CONCLUSION: The clinical presentation of myelitis is heterogeneous. Usually, neurologic deficits evolve within a few hours (typically acute transverse myelitis) and outcome is usually poor. However, chronic or recurrent transverse myelitis has also been reported, including relapsing myelitis that resolved spontaneously. Myelopathy can be the first manifestation of the disease and this might be more common than initially thought. Magnetic resonance imaging (MRI) findings depend on the timing of the examination and the stage of the disease; the MRI may therefore be normal. An association with optic neuritis is frequently reported in the literature and differential diagnosis with multiple sclerosis may be difficult. Overlapping features between both diseases have been termed "lupoid sclerosis" and are actually classified as demyelinating syndromes associated with lupus. Myelopathy does not appear to be consistently associated with antiphospholipid antibodies, as has been previously suggested. The best treatment protocol has not been determined; however, in recent years, pulses of methylprednisolone and cyclophosphamide have gained acceptance by most authors.     

Nocardiosis in patients with human immunodeficiency virus infection. Report of 2 cases and review of the literature.

Nocardia infection is a rarely reported opportunistic infection in HIV-infected patients. Nocardiosis typically occurs in HIV-infected patients with advanced immunodeficiency (89% of cases), often as the initial serious opportunistic infection (42% of cases). In most HIV-infected patients, nocardia infection is disseminated at the time of diagnosis and is characterized by an indolent course that may be difficult to differentiate from other systemic infections. Invasive procedures to obtain tissue of fluid for culture are frequently necessary to make the diagnosis, although a Gram or modified acid-fast stain of sputum or other infected material may suggest the etiologic agent. Although trimethoprim-sulfamethoxazole is the most commonly used initial therapy, it was discontinued in 50% of cases because of adverse reactions. Even though the optimal treatment has not been defined, nocardiosis in HIV-infected patients can be treated successfully with or without sulfa-containing antimicrobial regimens, along with surgical drainage when necessary. Recurrence is noted after short duration of treatment, and consideration should be given to lifelong maintenance therapy.     

Unusual presentations of primary human immunodeficiency virus infection

Opportunistic infections during primary infection with human immunodeficiency virus (HIV) are rare, with the exception of oral and esophageal candidiasis. HIV-associated nephropathy (HIVAN) and Pneumocystis carinii pneumonia (PCP) typically occur during advanced HIV infection. We report two patients who developed HIVAN and a presumptive diagnosis of PCP, respectively, during primary HIV infection. Serologic testing demonstrated HIV seroconversion. Clinicians need to have a high index of suspicion when evaluating patients even when risk behaviors are not readily apparent.     

Atypical venous thrombosis and protein S deficiency: report of a case

A 29 year old man is admitted for hypovemic shock and abdominal pain. This critical condition was due to a diffuse mesenteric venous thrombosis and intestinal infarction. Five meters of small bowel are resected. Few days later a superficial brachial venous thromboembolism grows to superior cava venous and bilateral pulmonary embolism. A plasmatic protein S level was 17%. This deficiency is considered to be the support of these atypical extended and repetitive venous thromboembolism. With an optimal nutrition and long oral anticoagulation this patient is alive 17 months after his admission.     

Unusual thromboses associated with protein S deficiency in patients with acquired immunodeficiency syndrome: case reports and review of the literature

Recent reports indicate that patients infected with HIV are at increased risk for the development of thrombosis. Among other possibilities, an acquired deficiency of protein S (PS), one of the plasma's natural anticoagulants, might explain this tendency. PS deficiency can be classified in three types depending on the levels of total and free protein (antigenic assays) as well as anticoagulant activity (functional assay). Although the prevalence of inherited PS deficiency is not known because of its rarity, several conditions can lead to acquired forms of the disease. We report two AIDS patients with coexistent type III PS deficiency and thrombosis. Our first patient presented with bilateral chronic leg ulcers and a skin biopsy revealed dermal microthromboses. On laboratory evaluation he had PS deficiency and was started on anticoagulation, but was lost to follow-up. The second patient presented with hepatic vein thrombosis (Budd-Chiari syndrome) and was also PS deficient. On long-term anticoagulation, she experienced resolution of the thrombosis. Neither patient had prior personal or family history of venous thrombosis, nor acquired risk factors such as immobility, acute infection, recent surgery, or hormonal therapy. The literature contains a few reports of skin ulcers and Budd-Chiari syndrome associated with PS deficiency, although none in AIDS patients. While a larger number of studies describe an association between PS deficiency and HIV infection, the causal effect of this deficiency on the thrombophilic tendency in AIDS has not been established. We propose that awareness of the increased risk for thrombosis in HIV infection is important to the understanding of disease pathophysiology and management of these patients.     

Progressive multifocal leukoencephalopathy associated with human immunodeficiency virus infection. A review of the literature with a report of sixteen cases

Progressive multifocal leukoencephalopathy, a common complication of infections with human immunodeficiency virus (HIV), occurs in as many as 3.8% of patients with the acquired immunodeficiency syndrome (AIDS). We report 16 cases and review 12 previously reported cases of progressive multifocal leukoencephalopathy associated with HIV infection. This illness was the presenting manifestation of HIV infection in 8 cases. Limb weakness, gait abnormalities, visual loss, aand altered mental status were the commonest initial complaints. Computed tomography of the brain frequently showed hypodense, nonenhancing white matter lesions. Magnetic resonance imaging was more sensitive than computed tomography in detecting lesions. Cerebrospinal fluid analysis and electroencephalography were nondiagnostic. Impaired cell-mediated immunity was typically noted, even in the absence of other immunodeficiency-associated illnesses. Death occurred within 10 days to 18 months of the onset of symptoms in 22 patients. However, 4 patients remain alive at 3 to 23 months; of these 4, 2 have had significant improvement without treatment. Various therapies were unsuccessful.     

Thrombotic thrombocytopenic purpura in patients with human immunodeficiency virus infection: a report of three cases and review of the literature.

Three cases of thrombotic thrombocytopenic purpura (TTP) and coexistent human immunodeficiency virus (HIV) infection are presented with a review of 15 cases reported in the literature. Of the 18 total patients, one-half presented with no symptoms of HIV infection while nine patients presented with symptomatic HIV disease before or simultaneous to the diagnosis. The presenting symptoms were similar to those with classic TTP and included fever in 75% and 40% with neurologic symptoms. Laboratory parameters reflected the microangiopathic hemolytic anemia typically seen in patients with TTP. The median hematocrit was 19.4%, while the median platelet count was 16,000/mm3. As with classic TTP, patients with HIV-related TTP only had mild renal dysfunction (median creatinine of 1.2 mg/dl, range 0.8-4.8 mg/dl). Plasma exchange produced clinical remission in a majority of the patients. Importantly, approximately one-third of the patients died prior to the initiation of therapy. We conclude that TTP is a rare but treatable condition in patients with HIV infection. A TTP diagnosis should be considered in patients with HIV infection who present with severe anemia and thrombocytopenia. Plasma exchange should be considered as initial therapy. The role of both antiplatelet therapy and aspirin is unknown.     

蛋白S缺乏导致静脉窦血栓形成合并硬脑膜动静脉瘘一例并文献复习

颅内静脉窦血栓形成(CVST)是一种少见的脑血管疾病,尤其是合并硬脑膜动静脉瘘形成(DAVF)时。蛋白S是机体重要的抗凝剂,其缺乏时可导致CVST,而临床上罕见蛋白S缺乏诱发CVST及DAVF的病例。本文报道1例此患者,并结合相关文献进行分析。     

Iron deficiency and thrombosis: literature review.

Compared to primary thrombocytosis such as that caused by essential thrombocytosis, reactive thrombocytosis is generally regarded as benign. However, reactive thrombocytosis has infrequently been reported to cause severe and even fatal complications. Two fatal cases of reactive thrombocytosis and iron deficiency anemia associated with peripheral/pulmonary vascular and cerebrovascular thrombosis are described. The literature on thrombosis and reactive thrombocytosis associated with iron deficiency anemia is reviewed.     

Pulmonary hypertension associated with human immunodeficiency virus infection: a review of 4 cases

Several cardiorespiratory diseases can complicate human immunodeficiency virus (HIV) infection. Primary pulmonary hypertension is a rare clinical disorder with a poor prognosis. We describe this syndrome in four HIV- positive patients who were examined in our hospital.     

Fulminant leptospirosis in a patient with human immunodeficiency virus infection: case report and review of the literature.

We report a case of fulminant leptospirosis that was acquired in New York City by a patient with underlying infection with human immunodeficiency virus (HIV). Review of the literature on leptospirosis in HIV-infected persons showed that all patients were severely ill but responded well to treatment, which highlights the importance of recognizing this potentially life-threatening illness, especially in unusual settings.     

Bacteremia due to Mycobacterium tuberculosis in patients with human immunodeficiency virus infection. A report of 9 cases and a review of the literature

Mycobacterium tuberculosis bacteremia is being reported more frequently in patients with human immunodeficiency virus, type 1 (HIV-1) infection. We report 9 patients with bacteremia due to M. tuberculosis and HIV infection who were identified over a 36-month period. Of the 9 patients studied, 8 were male, 8 were black, 6 were born in Haiti, 3 were homeless, 2 were intravenous drug users, and 1 was homosexual. At the time of diagnosis, 3 patients had the acquired immunodeficiency syndrome (AIDS) and 5 patients had CD4 lymphocyte counts less than or equal to 170 cells/mm3, indicating marked immunodeficiency. All 9 patients presented with temperature greater than 38.3 degrees C, 5 (50%) had abnormal chest roentgenogram on admission, and each of the patients tested had elevations of at least 2 liver function tests. Eight patients (80%) had M. tuberculosis isolated from sputum or other body fluids and tissues. All blood isolates of M. tuberculosis were identified from Dupont Isolator tubes. Antibiotic-resistant isolates of M. tuberculosis were cultured from 3 of the 6 patients born in Haiti. One patient died before diagnosis and received no antimycobacterial therapy; 7 of the remaining 8 patients appeared to respond to treatment. Our data, and a review of the literature, suggest that bacteremia due to M. tuberculosis is becoming more frequent, and that blood cultures may be helpful in establishing or confirming a diagnosis of tuberculosis in patients with HIV-1 infection.     

2 cases of recurrent deep venous thrombosis with protein C deficiency

Because of their gravity and the complications involved, repeated deep venous thromboses require everything to be done to produce an aetiological diagnosis, for only this will make a preventive treatment possible. Amongst causes of phlebitis, haemostatic disorders and coagulation factor anomaly should be systematically looked for, as these can sometimes be corrected. Following the discovery of the Antithrombin III deficiency, the protein C deficiency shows clear progress along these lines. The author here describes two cases of the protein C deficiency in patients who have suffered repeated deep and superficial venous thrombosis, with thromboembolic family antecedents.