Strongyloides hyperinfection syndrome complicating (ectopic) Cushing syndrome

Strongyloidiasis can present with a wide variety of symptoms and can lead to a potentially fatal hyperinfection. Although any factors that suppress the host defense mechanisms can potentially trigger hyperinfection, prolonged steroid use has been quite well described. A patient with disseminated small cell lung cancer suffered a Strongyloides stercoralis hyperinfection syndrome complicating ectopic adrenocorticotropic hormone (Cushing syndrome). Evaluation revealed lymphopenia, elevated levels of adrenocorticotropic hormone in the setting of elevated cortisol levels, a normal pituitary, and metastatic malignancy. S. stercoralis larval forms were seen in the stool and sputum. At autopsy, S. stercoralis larval forms were seen in the lung along with evidence of metastatic small cell lung carcinoma.     

Paraproteinemia in patients with acquired immunodeficiency syndrome (AIDS) or lymphadenopathy syndrome (LAS)

Eight of 15 patients with acquired immunodeficiency syndrome (AIDS) and six of nine patients with lymphadenopathy syndrome (LAS) had paraproteins in their sera. Twelve of these 14 were IgG kappa; the other two had no demonstrable light chains. The relationship of the paraprotein to the pathogenesis of AIDS is not clear, but we discuss its relation to derangements of B-cell immune regulation and function and to B-cell tumors in AIDS patients.     

Acquired immunodeficiency syndrome (AIDS)

This review article on acquired immunodeficiency syndrome (AIDS) covers its epidemiology, clinical spectrum, and etiology. Despite intensive efforts, the cause and pathogenesis of this syndrome remain unknown and effective therapy is not yet available. In addition, the clinical presentation of AIDS is variable, ranging from generalized lymphadenopathy to dermatologic lesions, pneumonias, enteritis, ophthalmologic lesions, disseminated disease, malignancies, neurologic conditions, paresthesias, radiculopathies, seizures, and psychiatric manifestations. The population at risk for AIDS icludes homosexual or bisexual men (71% of cases), intravenous drug abusers (17%), those born in Haiti who recently migrated to the US (5%), and hemophiliacs (1%). 6% of cases reported to the US Centers for Disease Control (CDC) fall in none of these categories. 47% of AIDS patients are 30-39 years of age at time of diagnosis. The CDC's strict surveillance definition of AIDS represents only 1 end of the clinical spectrum and could result in an underestimate of the size of the problem. It ignores the prodromal or milder forms of AIDS-related illnesses that have less clearly defined clinical courses. The persistent, irreversible immunosuppression characteristic of this syndrome creates a complex management problem for physicians, but the early recognition of AIDS by emergency and primary care physicians may improve the grim prognosis for AIDS victims.     

Lymphoma-associated hemophagocytic syndrome(LAHS)

Hemophagocytic syndrome(HPS) is characterized by a systemic proliferation of histiocytes showing hemophagocytosis in bone marrow, liver, or spleen. Clinical features of HPS are idiopathic fever, cytopenia, liver dysfunction, and coagulopathy. Adult HPS includes lymphoma-associated hemophagocytic syndrome(LAHS) and the incidence of LAHS was about 40% of the patients with HPS according to our study. About 70% of patients with LAHS had hepatosplenomegly without significant peripheral lymphoadenopathy or nodal lesions and showed HPS at the time of presentation. These clinical features were previously diagnosed as malignant histiocytosis (MH). The prognosis of LAHS depends mostly on the immunophenotype of lymphomas, T/NK-LAHS or B-LAHS. The median survival is 69 days in T/NK-LAHS and 242 days in B-LAHS.