Virus-associated hemophagocytic syndrome. Diagnosis and treatment

Virus-associated haemophagocytic syndrome (VAHS) is a rare disease characterized by fever, splenomegaly, cytopenia and histiocytic proliferation with haemophagocytosis in the reticuloendothelial system. The clinical course of VAHS can be dramatic and the prognosis is often poor. The pathogenesis of VAHS is not well understood. Many believe that viral infection provokes an abnormal immune response in predisposed individuals leading to hyperactivation of Th1 helper cells, macrophage proliferation and secretion of large amounts of cytokines. The resultant hypercytokinaemia may be responsible for the clinical and biochemical manifestations of VAHS. In this article the clinical features, presumed pathogenesis, diagnostic criteria and treatment of VAHS are discussed.     

Virus-associated hemophagocytic syndrome after hepatic resection: a case report

Virus-associated hemophagocytic syndrome (VAHS) is associated with a systemic viral infection and is mainly observed in immunosuppressed adult patients. This rare disease is characterized by symptoms which include a high fever, pancytopenia, and splenomegaly and sometimes results in a fatal outcome. However, thus far, little has been reported on VAHS in general surgical patients. We herein report this rare complication which occurred in a patient with hepatocellular carcinoma, as well as chronic hepatitis C, after a hepatic resection. A 66-year-old man with chronic hepatitis C and recurrent hepatocellular carcinoma underwent a repeat hepatic resection without any blood transfusions. In the early postoperative period, he recovered uneventfully. However, he suddenly began to suffer from a high fever (38.4 degrees C) and severe pancytopenia 8 days after surgery. Activated macrophages, which phagocytosed erythrocytes, were identified by a cytological study of the bone marrow. The patient was therefore treated with methylprednisolone pulse therapy 13 days after surgery. On the day following the initial administration of methylprednisolone, his clinical condition drastically improved. Fortunately, with methylprednisolone therapy, our patient recovered from acute, severe pancytopenia. In general surgery, it is often difficult for surgeons to use steroids due to their negative side effects. However, when symptoms such as fever, general fatigue and pancytopenia are observed, even in posthepatectomy patients with hepatocellular carcinoma and hepatitis, a bone marrow aspiration should be performed as soon as possible, and when VAHS is suspected, steroid pulse therapy should be the first treatment of choice. This rare but sometimes fatal complication should thus be taken into consideration in the postoperative management of hepatectomized patients with chronic hepatitis C.     

Pyogenic myositis: caused by viridans streptococci in an adult with tetralogy of Fallot

A 34-year-old man came to this Emergency Room because of fever, swelling and pain in the right thigh. Tetralogy of Fallot with bicuspid pulmonary valve was diagnosed after serial examination, and computed tomography of the right thigh revealed pyogenic myositis. Surgical drainage of the right thigh abscess and a further pus culture yielded viridans streptococci. There has been no record in the medical literature of a pyogenic myositis caused by viridans streptococci in an adult tetralogy of Fallot. This is thus the first case reported.     

Dental findings in an adult with Lowe's syndrome

This paper describes the dental findings and care of a Chinese adult affected with oculo-cerebro-renal syndrome--Lowe's syndrome--a rare, X-linked recessive disorder, whose clinical manifestations include cataracts, mental retardation, and renal tubular dysfunction. Approximately 150 cases have been reported in the last 45 years. Little has been reported in the dental literature. This patient is described with particular reference to the dental findings, which include gross periodontal disease with severe bone loss, impaction of teeth in the permanent dentition, taurodontism, and underdevelopment of the maxilla and mandible. Dental treatment was aimed at arresting the periodontal disease and preserving the remaining dentition.     

Rational use of rubella vaccine for prevention of congenital rubella syndrome in the Americas

Seventy-three spinal cord injured patients with central cord syndrome who had undergone inpatient rehabilitation, were studied retrospectively with regard to their demographic, neurologic and functional characteristics. There were 67 males and six females with a mean age of 53.5 years. Falls was the commonest mechanism of injury (54.8%) followed by motor vehicle accidents. Eleven patients sustained cervical fractures and 41 had radiological evidence of cervical spondylosis. Seventeen patients had sensory impairment and significant spasticity was present in 14 patients. Significant improvements in the admission/discharge ASIA motor scores and Modified Barthel Index (MBI) scores (P < 0.001) were noted after rehabilitation. Ninety-two percent of patients were continent of bladder on discharge compared to 64.4% on admission. Multiple regression analysis revealed three factors associated with a better functional outcome, namely, higher admission MBI scores, absence of spasticity and younger age (P < 0.05).     

Brown syndrome in an adult patient with morbus Still

A 25 year-old woman, known to be suffering from destructive rheumatoid arthritis, was admitted to the hospital due to influenza-like symptoms lasting three weeks. Adult Still's disease was diagnosed. During her disease she developed painful ophthalmoplegia due to tenosynovitis of the superior oblique muscle and tendon sheath (Brown's syndrome). Total resolution was obtained within three weeks of corticosteroid therapy.     

The Williams-Campbell syndrome diagnosed in an adult female patient

This article outlines the planning and setting up of an advice and discussion group for people with osteoporosis and their families to give a fuller understanding of the condition and how to manage it.     

Fulminant, CMV-associated, haemophagocytic syndrome following unrelated bone marrow transplantation

We report a case of haemophagocytic syndrome (HPS) occurring after allogeneic bone marrow transplantation (BMT) for acute promyelocytic leukaemia (APL) in a patient in fourth complete remission (CR). Anti-cytomegalovirus (CMV) antibody (Ab) was negative in this patient before BMT. BMT was performed from an HLA-identical unrelated donor who was positive for CMV Ab. After bone marrow engraftment and haematological recovery, severe acute graft-versus-host disease (GVHD) developed. This patient was treated with methylprednisolone in addition to cyclosporin A (CsA). Acute GVHD showed partial improvement, but CMV antigenaemia was observed. Despite administration of gancyclovir and immunoglobulin, CMV antigenaemia showed no improvement and HPS developed. As no other infections or malignancies were observed, we suspect that CMV infection was the trigger for development of HPS.     

Pseudo-Meigs'' syndrome caused by paraovarian fibroma

Meigs' syndrome includes an ovarian tumor, usually fibroma, associated with hydrothorax and ascites. It is accepted that uterine tumors, like fibromas, can also be associated with ascites and hydrothorax, but this is extremely rare. The mechanism of formation of peritoneal and pleural effusion is not well documented. The most likely pathogenesis ascribes the fluid formation to the filtration of interstitial fluid in the peritoneal through the tumor capsule, and the diffusion to the pleural space through the diaphragm lymphatic vessels at the foramen of Bochdalek. Paraovarian fibromas are also extremely rare neoplasms, probably of paramesonephric origin. It has been hypothesised that they can develop by proliferation of connective tissue cells around the Wolfian remnants. In this article, probably for the first time, a case of paraovarian fibroma with ascites and hydrothorax is presented.     

The thrombocytopenia syndrome caused by heparin

Heparin represents the basis for drug treatment and prevention of thrombosis. However, heparin itself may produce side effects, among others two types of heparin-induced thrombocytopenia (HIT). The more frequent type 1 occurs in about 10% of patients within the first days of the treatment and disappears spontaneously without sequelae. HIT type 2 represents a far more serious complication occurring in 0.1-1% of patients somewhat later, between days 4 and 10 of heparin therapy, and may provoke severe arterial and venous thrombosis. Apart from the differences in clinical picture, data regarding platelet count and platelet aggregation test are significant for the diagnosis. Heparin should be discontinued immediately and another anticoagulant therapy introduced. We are presenting the case of a patient with thrombosis of the left external iliac artery following surgery for ovarian cancer as a complication of HIT type 2, which was recanalized successfully and the leg was saved.     

Tuberculosis associated haemophagocytic syndrome: two cases with a favourable outcome

Haemophagocytic syndrome is a heterogenous disease characterized by disordered macrophage activation associated with viral, bacterial or parasitic infection. The few reports of haemophagocytosis occurring in the presence of mycobacterial infection show a high mortality rate and we present two further cases notable for their favourable issue. Rapidity of diagnosis and immediate treatment could explain the avoidance of a fatal outcome.     

Intravascular lymphomatosis: a case presenting with encephalomyelitis and reactive haemophagocytic syndrome diagnosed by renal biopsy

OBJECTIVE: To determine the frequency of characteristics associated with unprotected heterosexual intercourse in HIV infected adults in an urban area. DESIGN: Retrospective comparison of sexual risk transmission behaviour between HIV infected men and women from a drug treatment site and between women from the drug site and HIV infected women from an urban medical centre. METHODS: HIV infected women and men were asked questions on sexual behaviour for a 1 year period before enrollment. The outcome variable was heterosexual risk behaviour (HRB) defined as having vaginal sex at least once in the previous year and not always using condoms. RESULTS: 73% of the drug clinic females, 72% of the drug clinic males, and 42% of the medical centre female engaged in HRB. Using logistic regression analysis, women and men in drug treatment engaged in similar rates of HRB; however, women in drug treatment were four times (95% CI = 2.0-8.3) more likely to engage in HRB risk behaviour than women from the medical centre. CONCLUSION: The data suggest that a surprisingly large portion of HIV infected patients under treatment engaged in HRB, especially former drug users. Without specifically targeted interventions, the heterosexual spread of HIV in urban areas will continue to be a serious problem.