1/172. 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.- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
2/172. 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.- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
3/172. 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.- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
4/172. 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.- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
5/172. Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) following treatment with deoxycoformycin in a patient with cutaneous T-cell lymphoma (sezary syndrome): A case report.We present a case of a patient who developed all manifestations of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) acutely following treatment of cutaneous T-cell lymphoma (CTCL, sezary syndrome) with deoxycoformycin (pentostatin). Symptoms and signs included severe thrombocytopenia and microangiopathic hemolytic anemia; hallucinations, confusion and disorientation; oliguric acute renal failure requiring hemodialysis; and fever. No other etiology for these symptoms and signs was present. Complete recovery followed treatment for one month with plasma exchange and glucocorticoids. During the succeeding 20 months she has remained well and her CTCL remains stable on no further treatment. This case and two previously published cases suggest that acute and severe TTP-HUS may be a dose-dependent toxicity of deoxycoformycin (pentostatin).- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
6/172. Persistant pre-eclampsia post partum with elevated liver enzymes and hemolytic uremic syndrome.The spectrum of complications with pre-eclampsia, which may include AFLP (acute fatty liver of pregnancy) as well as the hellp syndrome (hemolysis, elevated liver enzymes, and low platelets), is resolved by early delivery. However, the ravages of HUS/TTP (hemolytic uremic syndrome/thrombotic thrombocytopenic purpura) require therapy usually by plasma exchange. Overlap between these two groups of syndromes has occurred on rare occasions and usually requires the therapy of the predominant or more dangerous or threatening form. Such overlap can be appreciated and then treated successfully without residual morbidity. The index case is presented and an extensive review of the two groups of syndromes is provided.- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
7/172. Basic fibroblast growth factor in hiv-associated hemolytic uremic syndrome.Endothelial injury is the primary pathogenic event leading to the renal thrombotic microangiopathic lesions typical of the hemolytic uremic syndrome (HUS). Basic fibroblast growth factor (bFGF) is an angiogenic growth factor released by injured endothelial cells. In a previous study we have found a significant accumulation of bFGF in human immunodeficiency virus (hiv)-transgenic mice with renal disease. Here we investigated whether bFGF was accumulated in the circulation and kidneys of two children with hiv-associated HUS (hiv-HUS), and studied the mechanisms involved in this process. The plasma levels of bFGF in children with hiv-HUS (124 /-20 pg/ml) were increased compared with five children with hiv nephropathy (49 /-6 pg/ml) and twenty hiv-infected children without renal disease (26 /-4 pg/ml, P<0.001). immunohistochemistry and receptor binding studies showed that bFGF was accumulated bound to heparan sulfate proteoglycans in renal glomeruli and interstitium surrounding renal tubules in hiv-HUS kidneys. Basic FGF stimulated the proliferation of mesangial and urinary renal tubular epithelial cells isolated from both patients. These findings support the hypothesis that bFGF and its low-affinity binding sites may play a relevant role in modulating the process of glomerular and renal tubular regeneration during the acute stages of hiv-HUS. A follow-up study in a larger sample population is required to confirm these results.- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
8/172. hemolytic-uremic syndrome with involvement of basal ganglia and cerebellum.hemolytic-uremic syndrome is a microangiopathy often associated with neurologic symptoms. Several patients with persistent lesions in cerebrum and basal ganglia have been reported. We present two children with bilateral basal ganglia and additional unilateral cerebellar lesions in magnetic resonance imaging. These resolved completely in one child. In the other child there were still residuals after 11 weeks. The neurologic symptoms of both improved after several therapeutic plasma exchanges and disappeared after months.- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
9/172. tacrolimus-induced hemolytic uremic syndrome and end-stage renal failure after liver transplantation.BACKGROUND: Hemolytic uremic syndrome (HUS) is a rare complication in solid organ transplantation. It can be associated with severe hypertension. Several risk factors have been identified including immunosuppressive drugs such as cyclosporin A and, more recently, tacrolimus. methods: Here we report a case of tacrolimus-induced HUS in a 61-yr-old woman after liver transplantation. hypertension, microangiopathic anemia and end-stage renal failure occurred 2 yr after liver transplantation. RESULTS: At admission, she had malignant hypertension with a severe hypertensive retinopathy, renal failure (creatininemia: 800 micromol/L) and microangiopathic anemia (Hb: 7.3 g/dL, a low platelet count and elevated lactate dehydrogenase). At renal biopsy, histologic findings were ischemic and sclerotic glomeruli with hyaline thrombi, severe mesangiolysis and interstitial fibrosis. CONCLUSION: Despite steroid treatment, antihypertensive agents and fresh frozen plasma therapy, end-stage renal failure was observed and chronic hemodialysis treatment was required.- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
10/172. quinine-induced hemolytic-uremic syndrome.quinine is still frequently used by practitioners for the treatment of nocturnal leg cramps, despite the lack of food and Drug Administration (FDA) approval. We report the 15th case of quinine-induced hemolytic-uremic syndrome (HUS) in the medical literature. The likely mechanism by which quinine induces HUS is via quinine-dependent antibodies to blood cellular constituents. These antibodies likely cause endothelial damage and the resultant nephropathy, microangiopathic hemolytic anemia, and thrombocytopenia that define HUS. Although there is no set guideline for the treatment of quinine-induced HUS, most authorities consider plasmapheresis as the standard of care, especially in severe cases. Our patient required the longest known treatment duration (16 plasmapheresis treatments over a 37-day period) for disease resolution. The prognosis of quinine-induced HUS is excellent, with no deaths reported in the literature.- - - - - - - - - - ranking = 2keywords = plasma (Clic here for more details about this article) |
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