| Haemolytic uraemic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are thrombotic microangiopathies increasingly reported in patients with hiv infection. However, characteristic features of thrombotic microangiopathies associated with hiv disease have not been defined yet. The typical courses of HUS and TTP in two patients are presented. The data as well as the analysis of cases published in the literature demonstrate the association of thrombotic microangiopathies with late-stage hiv disease. Moreover, differences between HUS and TTP can be detected. patients with HUS present with more severe immunologic deterioration. Although clinical symptoms are fewer, HUS implicates a very poor prognosis. life expectancy rarely exceeded 1 year after diagnosis. HUS and TTP should therefore be added to the international AIDS classification. ( info) |
2/539. Thrombotic microangiopathy as a complication of long-term therapy with gemcitabine. Three patients with pancreatic carcinoma treated with gemcitabine for 1 year developed clinical and laboratory findings compatible with an indolent form of the hemolytic-uremic syndrome. Renal biopsy specimens in two of these patients showed the characteristic features of thrombotic microangiopathy, and a skin biopsy specimen from the third patient, who presented with livedo reticularis, showed intravascular fibrin deposition. Thrombotic microangiopathy may represent a toxic effect of long-term gemcitabine therapy. ( info) |
3/539. Elevated reticulocyte count--a clue to the diagnosis of haemolytic-uraemic syndrome (HUS) associated with gemcitabine therapy for metastatic duodenal papillary carcinoma: a case report. In adults, the haemolytic-uraemic syndrome (HUS) is associated with probable causative factors in the minority of all cases. Cytotoxic drugs are one of these potential causative agents. Although metastatic cancer by itself is a recognized risk-factor for the development of HUS, therapy with mitomycin-C, with cis-platinum, and with bleomycin carries a significant, albeit extremely small, risk for the development of HUS, compared with all other cytotoxic drugs. Gemcitabine is a novel cytotoxic drug with promising activity against pancreatic adenocarcinoma. We are reporting on one patient with metastatic duodenal papillary carcinoma developing HUS while on weekly gemcitabine therapy. The presenting features in this patient were non-cardiac pulmonary oedema, renal failure, thrombocytopenia and haemolytic anaemia. The diagnosis of HUS was made on the day of admission of the patient to this institution. Upon aggressive therapy, including one single haemodialysis and five plasmaphereses, the patient recovered uneventfully, with modestly elevated creatinine-values as a remnant of the acute illness. Re-exposure to gemcitabine 6 months after the episode of HUS instituted for progressive carcinoma, thus far has not caused another episode of HUS. ( info) |
4/539. Postpartum haemolytic-uraemic syndrome. In a young woman a twin pregnancy and uneventful labour were complicated by the development of the postpartum haemolytic-uraemic syndrome. A number of unusual features of this syndrome were present, including early onset, accompanying hepatocellular necrosis, hepatic encephalopathy and bleeding diathesis. Early institution of heparin therapy combined with coagulation factor replacement was followed by cessation of haemorrhage and complete recovery from acute renal failure. ( info) |
| Colonic necrosis and intestinal perforation are the usual surgical complications of hemolytic syndrome. We present a case of right transverse colon stricture following hemolytic-uremic syndrome. ( info) |
6/539. Familial relapsing haemolytic uraemic syndrome and complement factor h deficiency. BACKGROUND: In a recent study of three families we have found that inherited haemolytic uraemic syndrome (HUS) maps to a region of chromosome 1q containing the gene for complement factor h. In one of these families and also in a case of sporadic D-HUS, we have identified mutations in the factor H gene. A further family with inherited HUS has therefore been investigated. methods: dna extracted from the family members and dna extracted from archival post-mortem material from a deceased family member, was studied. review of renal biopsies and study of complement components was also undertaken. RESULTS: This family demonstrates an inherited deficiency of complement factor h. Non-diarrhoeal HUS has affected at least two family members with half normal levels of factor H. CONCLUSION: These findings represent further evidence of the association between factor H dysfunction and HUS. ( info) |
7/539. Hemolytic uremic syndrome due to capnocytophaga canimorsus bacteremia after a dog bite. The hemolytic uremic syndrome (HUS) is known to have several causes, including infectious diseases, drugs, pregnancy, and malignant disease. We report a patient who developed acute renal failure attributable to HUS in the course of capnocytophaga canimorsus bacteremia. Acute tubular necrosis as well as HUS should be considered as a cause of acute renal failure in the setting of capnocytophaga canimorsus bacteremia. ( info) |
8/539. Postpartum hemolytic-uremic syndrome associated with lupus anticoagulant. A case report. BACKGROUND: Hemolytic uremic syndrome is a rare thrombotic microangiopathy characterized by acute renal failure, thrombocytopenia and hemolysis. The underlying abnormality is currently thought to involve enothelial injury within the microcirculation. CASE: A 30-year-old woman, gravida 2, para 1, underwent emergency cesarean delivery at 36 /- 2 weeks' estimated gestational age for repetitive late decelerations and presumed severe preeclampsia. Postoperatively, the blood pressure remained persistently elevated despite multigent hypertensive therapy. By postpartum day 4 the patient continued to display acute oliguric renal failure, persistent severe thrombocytopenia and worsening hemolysis. Percutaneous renal biopsy was consistent with the clinical diagnosis of hemolytic uremic syndrome. Lupus anticoagulant was present, corroborated by markedly abnormal tissue thromboplastin inhibition and platelet neutralization procedures. With supportive therapy and daily plasmapheresis, the patient was discharged 22 days after delivery, with full recovery of renal function and resolution of the hemolytic process. CONCLUSION: Hemolytic uremic syndrome can be associated with lupus anticoagulant. This autoantibody may promote localized platelet aggregation, causing endothelial damage. ( info) |
9/539. Thrombotic microangiopathy associated with interferon therapy for patients with chronic myelogenous leukemia: coincidence or true side effect? BACKGROUND: interferon-alpha (rIFN-alpha) is an established therapy for patients with myeloproliferative disorders. Unusual immune-mediated side effects have been associated with rIFN-alpha therapy. The association of rIFN-alpha therapy with hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) has been reported infrequently. methods: Two patients with chronic myelogenous leukemia (CML) treated with rIFN-alpha-based regimens at the University of texas M. D. Anderson Cancer Center developed thrombotic microangiopathy (HUS/TTP). The course of their disease is described. A third patient who developed renal failure while receiving rIFN-alpha therapy and had no other causative factor for his renal failure is also described. RESULTS: The patients were ages 24, 49, and 36 years, and they had received rIFN-alpha therapy for 37, 67, and 92 months, respectively, prior to the development of the disorder. One patient had discontinued rIFN-alpha 1 month before the event because of presumed rIFN-alpha-related cardiomyopathy. Two patients received hydroxyurea and cytarabine as part of their therapy. No patient was receiving any medication known to be associated with HUS/TTP. None had a history of diarrheal illness, but escherichia coli OH157.H7 was grown from the stool of one patient. Two patients responded to plasmapheresis with normalization of counts and other indices, but both developed renal failure and became dependent on dialysis. One patient had evidence of disease progression and died of multiorgan failure. The third patient required dialysis for 18 months but is currently off dialysis; this patient has some residual renal impairment. CONCLUSIONS: Although no definitive association between rIFN-alpha therapy and thrombotic microangiopathies can be concluded from these data, these and other previously reported cases suggest that HUS/TTP is a rare side effect of rIFN-alpha therapy that should be managed in the standard fashion. Hypotheses regarding the mechanism underlying this association are discussed in this article. ( info) |
10/539. Hemolytic uremic syndrome associated with immunoglobulin a nephropathy: a case report and review of cases of hemolytic uremic syndrome with glomerular disease. A 35-year-old man with immunoglobulin a (IgA) nephropathy who developed hemolytic uremic syndrome (HUS) presented with transient elevation of serum creatinine, thrombocytopenia, and hemolytic anemia with fragmented red cells with nephrotic syndrome. Hemolytic anemia and the temporarily deteriorated renal function were improved after hemodialysis and plasma exchange. Histological findings were consistent with HUS and IgA nephropathy. Including this case, we reviewed the cases of HUS accompanied by glomerular diseases reported from 1969 to 1996. Surprisingly, most cases showed nephrotic syndrome at the onset of HUS. Several possible relationships between HUS and nephrotic syndrome are discussed. ( info) |