1/44. Multiple autoimmune haemopoietic disorders and insidious clonal proliferation of large granular lymphocytes.We report a patient with clonal proliferation of CD3 8 TCRalphabeta large granular lymphocytes (LGL) presenting multiple episodes of autoimmune cytopenia, including autoimmune neutropenia, idiopathic thrombocytopenic purpura, autoimmune haemolytic anaemia, and pure red cell aplasia. Each disorder appeared separately or as a combination during an 11-year clinical course. The increase of blood CD3 8 TCRalphabeta LGL was detected 6 years after the initial diagnosis of cytopenia, but the absolute number of LGL cells was always < 1.0 x 109/l. LGL cells were of monoclonal origin and had a chromosomal abnormality. LGL cells transiently responded to cyclosporine A therapy, which was also effective on all of these autoimmune cytopenias. Accordingly, an undetectable level of proliferation of a clonal LGL population could cause various autoimmune haemopoietic disorders.- - - - - - - - - - ranking = 1keywords = thrombocytopenic, purpura (Clic here for more details about this article) |
2/44. 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 = 4keywords = thrombocytopenic, purpura (Clic here for more details about this article) |
3/44. Autosomal recessive inheritance of von willebrand factor-cleaving protease deficiency.A child with chronic relapsing thrombotic thrombocytopenic purpura (TTP/HUS) had recurrent thrombocytopenia, microangiopathic hemolytic anemia with fragmented erythrocytes, microthrombi in the lung vessels, and renal dysfunction. Assay of von willebrand factor (vWF)-cleaving protease showed a complete protease deficiency in the patient and subnormal activities in the mother and in two asymptomatic siblings. No inhibitor of vWF-cleaving protease was detected in the patient's plasma. Periodic transfusions of fresh-frozen plasma prevented further acute episodes of TTP/HUS. Specific diagnosis of the constitutional deficiency of vWF-cleaving protease helps to provide successful prophylactic therapy.- - - - - - - - - - ranking = 1keywords = thrombocytopenic, purpura (Clic here for more details about this article) |
4/44. splenectomy for refractory Evans' syndrome associated with antiphospholipid antibodies: report of two cases.The main haematological manifestations seen in patients with antiphospholipid antibodies (aPL) are thrombocytopenia, usually mild, and haemolytic anaemia with a positive coombs test. Owing to the shared characteristics with idiopathic thrombocytopenic purpura, similar rules are followed in the treatment of these cytopenias. Two patients with severe aPL associated cytopenias, who required splenectomy after being refractory to steroids, immunosuppressive agents, and other treatments (intravenous gammaglobulin, danazol), are described, and previously reported cases are reviewed.- - - - - - - - - - ranking = 1keywords = thrombocytopenic, purpura (Clic here for more details about this article) |
5/44. Preliminary report: treatment of the hemolytic-uremic syndrome with aspirin and dipyridamole.Three children with the hemolytic-uremic syndrome were treated with heparin, aspirin, and dipyridamole. Two of the children had remained profoundly thrombocytopenic in spite of platelet transfusion and heparin therapy. All three patients responded with prompt elevation of their platelet counts and apparent termination of the pathologic consumption of platelets. Our experience suggests not only that primary platelet consumption may play a critical role in the pathogenesis of the HUS, but also that such patients may benefit from therapy with drugs which inhibit platelet function.- - - - - - - - - - ranking = 0.83507892155475keywords = thrombocytopenic (Clic here for more details about this article) |
6/44. Microangiopathic haemolytic anaemia in metastasizing malignant tumours is not associated with a severe deficiency of the von willebrand factor-cleaving protease.Complete deficiency of von willebrand factor-cleaving protease (VWF-cp) has recently been identified as a pathogenetically important factor for thrombotic thrombocytopenic purpura (TTP). Microangiopathic haemolytic anaemia (MAHA) with thrombocytopenia in patients with metastasizing neoplasms is clinically similar to TTP, however, the pathogenesis of the condition is unclear. Partial deficiency of VWF-cp in metastasizing malignancy has recently been reported in patients without MAHA. Our study shows normal or subnormal VWF-cp activity in four patients with metastasizing neoplasia-associated MAHA but, in contrast to classical TTP, no complete deficiency of VWF-cp despite the full clinical picture of MAHA.- - - - - - - - - - ranking = 1keywords = thrombocytopenic, purpura (Clic here for more details about this article) |
7/44. Upshaw-Schulman syndrome revisited: a concept of congenital thrombotic thrombocytopenic purpura.Upshaw-Schulman syndrome (USS) is a congenital bleeding disorder characterized by repeated episodes of thrombocytopenia and microangiopathic hemolytic anemia that respond to infusions of fresh frozen plasma. Inheritance of USS has been thought to be autosomal recessive, because 2 siblings in the same family are often affected but their parents are asymptomatic. Recently, chronic relapsing thrombotic thrombocytopenic purpura (CR-TTP), reported almost exclusively in adults, was shown to be caused by inherited or acquired deficiency in the activity of a plasma von willebrand factor-cleaving protease (vWF-CPase). The pathogenesis of USS is unknown, and a relationship between CR-YEP and USS has not been reported. We studied 3 unrelated USS patients (ST, SY, and KI) who presented with severe indirect neonatal hyperbilirubinemia. All 3 patients had undetectable vWF-CPase activity, and the inhibitors to vWF-CPase were all negative. In their parents with no clinical symptoms, vWF-CPase activities as a percentage of control samples (mother/father) were 17/20 for ST, 60/45 for SY, and 36/5.6 for KI. Thus, USS and vWF-CPase activity appear to be coinherited as autosomal recessive traits. Transfusion of fresh frozen plasma in 2 patients (ST and SY) resulted in the expected maximal increment of approximately 7% to 8% in vWF-CPase activity at 1 to 4 hours, but the levels became less than 3% within 2 days. After this decrease, platelet counts increased, plateaued in the normal range at 10 to 12 days, and declined thereafter. Thus, the 2 to 3 weeks of therapeutic benefit from plasma infusions will be discussed in relation to the intravascular lifetime of vWF-CPase.- - - - - - - - - - ranking = 5keywords = thrombocytopenic, purpura (Clic here for more details about this article) |
8/44. Severe microangiopathic hemolytic anemia and thrombocytopenia in a child with brucella infection.We present a case of severe microangiopathic hemolytic anemia and thrombocytopenia with epistaxis, gross hematuria, hemoglobinuria, and skin purpura in a child with brucella septicemia proven by culture. The patient showed the features of this illness: leukopenia, severe hemolytic anemia, thrombocytopenia, fragmentation of erythrocytes in the peripheral blood smear, increased erythropoiesis, megakaryopoiesis, and granulomata cell invasion in the bone marrow. The patient was treated with rifampin and doxycycline. Platelets and leukocyte numbers rose to normal values by the 6th day. She was discharged on the 14th day. Follow-up of the patient 1 year later showed normal bone marrow morphology. Differential diagnosis, pathogenesis, and therapy of brucella infection are discussed.- - - - - - - - - - ranking = 0.16492107844525keywords = purpura (Clic here for more details about this article) |
9/44. Anaesthetic management of splenectomy in Evan's syndrome during pregnancy with pregnancy induced hypertension.The management of idiopathic thrombocytopenic purpura (ITP) during pregnancy, especially with ongoing bleeding diathesis, has not been highlighted sufficiently in the literature. Aortocaval compression and reduction in uteroplacental circulation resulting in foetal hypoxia and acidosis, Mendelson's syndrome due to gravid uterus, trauma to airway with resultant haemorrhage and aspiration into lungs, compromised airway due to short neck, anasarca and heavy breast, limitation in using invasive monitoring and regional anaesthesia and uncontrolled bleeding leading to placental hypoperfusion and foetal hypoxia are some of the important risks. In the present case report, anaesthetic management for splenectomy during pregnancy complicated with pregnancy induced hypertension and bleeding diathesis secondary to ITP is described with reference to above risks.- - - - - - - - - - ranking = 1keywords = thrombocytopenic, purpura (Clic here for more details about this article) |
10/44. Intravenous anti-D immune globulin-induced intravascular hemolysis in Epstein-Barr virus-related thrombocytopenia.RhoD immune globulin intravenous (anti-D IGIV) increases platelet counts in patients who have not undergone splenectomy and are positive for RhoD with idiopathic thrombocytopenic purpura. After treatment, in most patients, anemia develops as a result of immune-mediated red cell destruction in the spleen. Although intravascular hemolysis (IVH) is not expected, life-threatening IVH has been recently reported by the food and Drug Administration, and physicians are encouraged to report their experience with patients with idiopathic thrombocytopenic purpura in whom IVH develops after anti-D administration. Severe IVH was observed after treatment with anti-D IGIV in two adolescent girls with acute thrombocytopenia related to Epstein-Barr virus. They did not have hemolytic anemia before treatment. The authors believe that anti-D IGIV triggered an unusual virus-induced immune response causing hemolysis; therefore, anti-D IGIV should not be used in patients with Epstein-Barr virus-related thrombocytopenia, particularly during the acute phase of infection.- - - - - - - - - - ranking = 2keywords = thrombocytopenic, purpura (Clic here for more details about this article) |
| | Next -> |