1/23. Cryosupernatant plasma exchange in the treatment of antiphospholipid antibody syndrome with lupus nephritis.We report a case of a 22-year-old female with antiphospholipid antibody syndrome (APS) associated with systemic lupus erythematosus in whom cryosupernatant plasma exchange was effective and improved both the refractory venous thrombosis in her legs and relapsing thrombocytopenia. A renal biopsy specimen showed not only features of active lupus nephritis but also renal arteriolar thrombosis which is considered to be a type of thrombotic microangiopathy (TMA). Because a pathological role of unusually large von willebrand factor (vWF) multimers has been reported in patients with TMA including thrombotic thrombocytopenic purpura, plasma exchange using replacement with cryosupernatant, which is free of unusually large vWF multimers, is likely to be an option of treatment modality for patients with refractory and chronic relapsing APS manifesting TMA.- - - - - - - - - - ranking = 1keywords = microangiopathy (Clic here for more details about this article) |
2/23. A case of primary antiphospholipid antibody syndrome with acute renal failure showing thrombotic microangiopathy.An 18-year-old woman complained of fever and edema and was admitted to Showa University Hospital for treatment of thrombocytopenia and deteriorating renal function. Laboratory studies demonstrated the presence of lupus anticoagulant (LA), prolongation of prothrombin time, hemolytic anemia, a negative Coombs' test, the absence of antinuclear antibodies, and a normal fibrinogen level. Renal biopsy revealed mesangial hypercellularity, severe endocapillary cell damage, and double contour of the basement membrane walls. Immunofluorescence studies demonstrated focal, peripheral, and finely granular deposits for IgG, IgM, and IgA but were negative for fibrinogen. Electron microscopy showed glomerular capillary loops with subendothelial widening and subendothelial deposits, mesangiolysis, mesangial interposition, and marked luminal narrowing. biopsy findings were consistent with thrombotic microangiopathy. The patient was treated with hemodialysis, methylprednisolone pulse therapy, and dipyridamole. After treatment, LA disappeared, the prothrombin time became normal, and renal function improved. The renal lesions in this patient were caused by primary antiphospholipid antibody syndrome. This case strongly suggests an important causal relationship between LA and renal lesions in thrombotic microangiopathy. We present this case to promote understanding of the pathogenesis of primary antiphospholipid antibody syndrome. copyright copyright 1999 S. Karger AG, Basel- - - - - - - - - - ranking = 6keywords = microangiopathy (Clic here for more details about this article) |
3/23. Cocaine-induced acute renal failure, hemolysis, and thrombocytopenia mimicking thrombotic thrombocytopenic purpura.Acute renal failure (ARF) can occur as a complication of cocaine abuse. We present a case of microangiopathic hemolytic anemia, ARF, and thrombocytopenia after inhalation of crack cocaine in a 38-year-old woman. Her renal failure ultimately required dialysis. She underwent renal biopsy because of persistent renal failure, hematuria, and thrombocytopenia. The biopsy findings consisted of thrombotic microangiopathy and glomerular ischemia. After treatment with fresh frozen plasma, her platelet count and bleeding resolved. The possible mechanisms involved in cocaine-induced thrombotic microangiopathy include: (1) endothelial injury, (2) vasoconstriction and/or impairment of vasodilatation, (3) procoagulant activity, and (4) antiplatelet activity. Although our patient survived after hemodialysis and transfusion of fresh frozen plasma, she continued to have residual renal insufficiency. One month later, the patient again used cocaine and presented with worsening ARF, anemia, and thrombocytopenia.- - - - - - - - - - ranking = 2keywords = microangiopathy (Clic here for more details about this article) |
4/23. heparin-induced skin necrosis in a patient with end-stage renal failure and functional protein s deficiency.skin ulceration is a well-characterized thrombotic complication of the heparin-induced thrombocytopenia (HIT) syndrome. We present the case of a 73-year-old diabetic woman nearing end-stage renal failure who developed extensive upper thigh, abdominal and buttock ulceration following initiation of subcutaneous heparin for prophylaxis against deep vein thrombosis. A preliminary diagnosis of calciphylaxis was made based on the classical distribution and macroscopic appearance of the ulceration in a patient with end-stage renal failure and secondary hyperparathyroidism. However skin biopsy showed complete absence of calcium deposits in the dermal microvasculature. The presence of extensive microthrombi within dermal vessels prompted serologic testing to detect a prothrombotic state. We identified the combined presence of heparin-dependent platelet activating (HIT) antibodies and functional protein s deficiency. To our knowledge this is the first reported case of a dialysis patient presenting with skin ulceration induced by heparin and protein s deficiency. This case highlights the importance of a skin biopsy and testing for a hypercoaguable state in patients with end-stage renal disease and skin ulceration. We suggest that HIT antibodies should be requested in all dialysis patients presenting with skin ulceration.- - - - - - - - - - ranking = 0.019062046196695keywords = diabetic (Clic here for more details about this article) |
5/23. Congenital microangiopathic hemolytic anemia and thrombocytopenia with unusually large von willebrand factor multimers and von willebrand factor-cleaving protease.Infantile or congenital cases of thrombotic microangiopathy have been reported that were familial and characterized by ongoing microangiopathic hemolysis and thrombocytopenia in the absence of regular fresh-frozen plasma transfusions. The authors describe a child with congenital microangiopathic hemolytic anemia and thrombocytopenia (CMHAT) who has received regular fresh-frozen plasma transfusions since infancy and has never had thrombotic complications. von willebrand factor (vWF)-cleaving protease activity was studied in the patient's pretransfusion and posttransfusion plasma samples as well as in her parents' plasma. The effects of the patient's and a control subject's plasma on human microvascular endothelial cells were also investigated. Unusually large vWF multimers were present in the patient's plasma both before transfusion (thrombocytopenic) and after transfusion. Unlike cases of chronic relapsing thrombotic thrombocytopenic purpura, vWF-cleaving protease activity was present and treatment of cultured human endothelial cells with the patient's plasma did not induce apoptosis. These findings suggest that the patient with CMHAT may represent a different group in the broad spectrum of thrombotic microangiopathies.- - - - - - - - - - ranking = 1keywords = microangiopathy (Clic here for more details about this article) |
6/23. Shigella septicaemia in adults: report of two cases and mini-review.INTRODUCTION: We report 2 cases of Shigella septicaemia in adult patients. CLINICAL PICTURE: Two 57-year-old women presented with non-bloody diarrhoea and fever. The first patient was an inmate of a long-term care facility who was schizophrenic and the second patient was a diabetic who recently travelled to Medan, indonesia. Both patients were febrile, hypotensive and dehydrated. The first patient was neutropenic, thrombocytopenic and had acute renal failure. blood cultures yielded shigella flexneri and stool cultures gave negative results for both patients. TREATMENT: Rehydration and intravenous ceftriaxone were instituted. OUTCOME: The patients' symptoms and hypotension resolved. CONCLUSION: Appropriate antibiotics can decrease the severity and duration of Shigella septicaemia.- - - - - - - - - - ranking = 0.019062046196695keywords = diabetic (Clic here for more details about this article) |
7/23. Cancer-related thrombotic microangiopathy secondary to von willebrand factor-cleaving protease deficiency.Cancer-related thrombotic microangiopathy (TM) is a serious complication with a short-term life-threatening prognosis. This complication shares certain similarities with thrombotic thrombocytopenic purpura and hemolytic uremic syndrome, both characterized by circulating platelet aggregates containing ultralarge multimers of von willebrand factor (VWF). We report a case of cancer-related thrombotic microangiopathy secondary to disseminated metastatic cancer with undetectable serum von willebrand factor-cleaving protease activity and no evidence of serum inhibitory antibody. A concomitant decrease of Ca 19-9 level and hemolysis was observed during chemotherapy, in parallel with normalization of von willebrand factor-cleaving protease activity. The role of ultralarge multimers of von willebrand factor in platelet aggregation in the context of metastatic disease is discussed with respect to our findings in this case of cancer-related thrombotic microangiopathy.- - - - - - - - - - ranking = 7keywords = microangiopathy (Clic here for more details about this article) |
8/23. High-dose verapamil trandolapril-induced thrombotic microangiopathy.Thrombotic microangiopathy (TMA) is a syndrome characterized by microangiopathic haemolytic anaemia, thrombocytopenia, and several variable signs of organ damage due to the platelet thrombi in the microcirculation. This article reports a case with TMA which developed after ingestion of a high-dose combination of verapamil and trandolapril. To the authors' knowledge, no prior cases of TMA induced by trandolapril (an angiotensin-converting enzyme inhibitor) and verapamil (a calcium channel blocker) have been reported in the literature.- - - - - - - - - - ranking = 5keywords = microangiopathy (Clic here for more details about this article) |
9/23. Withdrawal of interferon-alpha results in prompt resolution of thrombocytopenia and hemolysis but not renal failure in hemolytic uremic syndrome caused by interferon-alpha.This case report describes 2 patients with chronic myeloid leukemia in whom hemolytic uremic syndrome developed while being treated with interferon-alpha and hydroxycarbamide. Hemolytic uremic syndrome was recognized by progressive renal dysfunction, thrombocytopenia, microangiopathic hemolytic anemia, and histologic features of thrombotic microangiopathy in the kidney. Although renal dysfunction progressed to dialysis-dependent renal failure in one patient despite treatment with prednisolone and plasmapheresis but not in other, withdrawal of the treatment resulted in a prompt resolution of thrombocytopenia and microangiopathic hemolytic anemia in both patients.- - - - - - - - - - ranking = 1keywords = microangiopathy (Clic here for more details about this article) |
10/23. rhabdomyolysis in diabetic ketoacidosis.rhabdomyolysis is a potentially lethal disorder, characterized by elevated serum concentrations of creatine kinase (CK) due to skeletal muscle injury. In this paper a patient with diabetic ketoacidosis (DKA) is reported who developed rhabdomyolysis (maximum CK level, 37,700 U/L; normal, < 170 U/L), anemia (6.2 g/dL) and thrombocytopenia (16,000/microL). This combination of rhabdomyolysis with anemia and thrombocytopenia has not yet been reported in DKA. The pathogenic mechanism leading to rhabdomyolysis in DKA remains unsettled. From the literature it seems that those patients who develop rhabdomyolysis have very high glucose levels and a high osmolality on admission. Low phosphate levels can play a role as well. The etiology of anemia and thrombocytopenia in our patient remains obscure. Intravascular hemolysis could not be demonstrated but intramedullar hemolysis, due to osmolar shift or hypophosphatemia, cannot be excluded. A review of the literature data revealed that rhabdomyolysis is not so uncommon in DKA. However, to obtain incidence data in children, prospective studies are necessary.- - - - - - - - - - ranking = 0.095310230983475keywords = diabetic (Clic here for more details about this article) |
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