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1/11. Thrombotic thrombocytopenic purpura: a syndrome of intravascular platelet consumption.

    In four of five patients with thrombotic thrombocytopenic purpura (TTP) in whom serial tests of hemostatic function were performed, severe thrombocytopenia, normal plasma fibrinogen concentrations and mildly increased concentrations of fibrinogen/fibrin degradation products were observed. Widespread platelet thrombi were found in arterioles and capillaries. Fibrin could be seen around some of the platelet clumps and was the main component in a small number of the thrombi in two patients. The observations show that TTP is a disorder in which intravascular platelet consumption results in disseminated platelet thrombosis. The coagulation system is apparently activated secondarily to platelet aggregation and variable quantities of fibrin are incorporated into the thrombi. Clinical improvement resulted from combined therapy with corticosteroids, heparin and drugs that suppress platelet function.
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2/11. Intravascular lymphomatosis.

    Intravascular lymphomatosis (IVL) is a rare angiotrophic large cell lymphoma producing vascular occlusion of arterioles, capillaries, and venules. Antigenic phenotyping shows that these lymphomas are mostly of B cell type, and less commonly T cell or Ki-1 lymphomas. The central nervous system and skin are the two most commonly affected organs; patients usually present with progressive encephalopathy with mental status changes and focal neurological deficits and skin petechia, purpura, plaques, and discolouration. Other involved organs include adrenal glands, lungs, heart, spleen, liver, pancreas, genital tract, and kidneys. bone marrow, blood, cerebrospinal fluid, and lymph nodes are typically spared. fever of unknown origin is another common presentation. Only one case of IVL presenting with disseminated intravascular coagulation and anasarca (generalised oedema) has been reported in the literature. This report describes a postmortem case of a patient with IVL who initially presented with disseminated intravascular coagulation complicated by intracerebral haemorrhage.
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3/11. Acute skin and fat necrosis during sepsis in a patient with chronic renal failure and subcutaneous arterial calcification.

    Calcification of small subcutaneous arteries and arterioles is commonly found in patients with chronic renal failure (CRF), but the syndrome of acute ischemic necrosis of the skin and subcutaneous fat supplied by these vessels is relatively uncommon. The necrosis occurs during dialysis and after successful renal transplantation, and it is often fatal. Occlusion of the calcified arteries and associated microvessels by thrombi is reported infrequently, but it is relevant to the necrosis. However, the pathogenesis remains enigmatic. In the patient described here, who had CRF, bacteremia, and laboratory evidence of disseminated intravascular coagulation (DIC), the distribution of thrombi and necrosis was mainly that of the calcified arteries which, therefore, probably played a role in the localization of the thrombi. An increased susceptibility of the endothelium of calcified vessels to the procoagulant effects of sepsis may be a contributing factor.
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4/11. purpura fulminans as clinical manifestation of atypical SLE with antiphospholipid antibodies: a case report.

    purpura fulminans and DIC were the main clinical manifestations of the antiphospholipid syndrome observed in a 62-year-old man. The patient was well until 44 years of age when he began to suffer from recurrent thrombophlebitis, without other symptoms suggestive of immune disease. At the time of hospital admission the pt. appeared acutely ill, showing high fever, severe anemia, massive urinary blood loss, multiple purpuric patches evolving to hemorrhagic bullae and gangrene rapidly spreading over about 30% of the total body area. No signs of neurological involvement or of visceral thrombotic occlusions were present. Clotting tests were consistent with a diagnosis of DIC, further confirmed by skin biopsy showing the presence of thrombi in dermal arterioles. The autoantibody research was positive as follows: Waaler-Rose 1:40, Anti-dna 1:80; ANF 1:640, aCA IgG 100 GPL. LA was diagnosed according to standard criteria: prolonged KCT and RVVT not corrected by a mixture of normal plasma and abnormal TTI. plasma exchange in association with heparin and prednisone was effective in arresting the progression of the skin lesion; nevertheless the patient died ten days after hospital admission for sepsi and acute renal failure.
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5/11. Complete necrotization of hepatocellular carcinoma by chemotherapy and subsequent intravascular coagulation: a case report.

    A 45-year-old man with hepatocellular carcinoma who developed intravascular coagulation following complete tumor regression by chemotherapy is described. After 2 doses of 10 mg of mitomycin C given into the hepatic artery at the time of selective angiography, and 16 intravenous doses of 5-fluorouracil and mitomycin C, 2 doses per week, subjective symptoms and hepatomegaly disappeared. Alpha-fetoprotein became negative and a remarkable change in tumor size and vasculature was noted in the arteriogram. Three months after chemotherapy, the patient developed thrombocytopenia, intravascular hemolysis, and acute renal failure. autopsy disclosed a 8 X 7 X 5 cm solitary, encapsulated hepatocellular carcinoma in the right lobe. The tumor was surrounded by a thick capsule and completely necrotized. Neither intrahepatic invasion nor extrahepatic metastasis was observed. In the kidney, generalized fibrin thrombi were seen in the afferent arterioles of glomeruli as accounted for by intravascular coagulation.
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6/11. purpura fulminans in an adult.

    purpura fulminans is a rare disease of childhood, manifested by massive hemorrhage into the skin, widespread thrombosis of venules and arterioles and a hematologic picture of disseminated intravascular coagulation. An unusual case in a 68-year-old man is presented and the mode of therapy discussed.
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7/11. Acute diffuse interstitial fibrosing pneumonitis and bilateral renal cortical necrosis.

    An autopsy case of a 69-year-old male with acute diffuse interstitial fibrosing pneumonitis complicated by bilateral renal cortical necrosis was presented. autopsy revealed acute diffuse interstitial fibrosing pneumonitis, bilateral renal cortical necrosis, non-bacterial thrombotic endocarditis, involving the aortic and mitral valves, and some interesting vascular lesions, dissemination of fibrinoid change of arterioles and fibrin thrombus of small vessels in various organs; accumulation of polymorphonuclear leukocytes in the lumen of the smaller interlobular arteries and arterioles of the kidney with cellular infiltration and disintegration of the wall; severe disorganization of the wall with intraluminar and intramural fibrinous exudation in smaller branches of the hepatic artery; diminution and disarrangment of muscle fibers and patchy hyalinization in the media of the renal and interlobar arteries. The inter-relationship between acute diffuse interstitial fibrosing pneumonitis, bilateral renal cortical necrosis which may be regarded as a 'hallmark' of the generalized Shwartzman reaction, and disseminated intravascular coagulation was discussed.
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8/11. Overwhelming infection after splenectomy in spite of some spleen remaining and splenosis. A case report.

    A fatal case of overwhelming postsplenectomy pneumococcal sepsis is presented occurring in a 37-year-old female 11 years after removal of the spleen because of traumatic rupture. The patient died 11 h after admission to hospital and about 32 h after sudden onset of illness. At necropsy splenic tissue, splenosis, disseminated intravascular coagulation, and thrombi within the arterioles consisting of gram-positive cocci and adrenal hemorrhage were found. The clinical, laboratory, and postmortem findings are described. Reports had been published of 41 other cases of overwhelming postsplenectomy infection (OPSI) in patients aged 20 years or more, but only three of these cases of OPSI syndrome occurred in spite of remaining splenic tissue. The longest interval between extirpation of spleen and subsequent sepsis was 42 years, indicating a small but lifelong risk of severe infection in asplenic patients. In view of the literature, the role of spleen in infection defence, the splenic function in blood clearance, and the prevention of postsplenectomy infections by antibiotic prophylaxis, pneumococcal vaccine, and reimplantation of autochthonous splenic tissue or infrared contact coagulation are discussed.
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9/11. radiation nephritis and intravascular coagulation.

    A 23 year old male with embryonal cell carcinoma of the testicle was treated with radiation, receiving 2250 Rads to his abdomen twice, only once with kidney shielding. He developed acute renal failure approximately eleven months later. Associated with the renal failure were hemolytic anemia, thrombocytopenia, hypofibrinogenemia, and other evidence for intravascular coagulation. The kidney biopsy showed fibrinoid necrosis of arteries and arterioles. By electron microscopy, there was extensive endothelial cell damage and subendothelial electron lucent material compatible with radiation nephritis. The renal pathologic abnormalities suggest that the local renal vascular injury induced by radiation could have been responsible for intravascular coagulation in the kidneys and the systemic hematologic abnormalities. prednisone appeared to accelerate the renal and hematologic dysfunction and heparin is proposed as a more promising therapy.
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10/11. Comparative immunopathologic studies of thrombotic thrombocytopenic purpura, hemolytic-uremic syndrome and disseminated intravascular coagulation.

    The vascular lesion of thrombotic thrombocytopenic purpura was characterized by two distinct types of changes in the arteriole of the heart and brain: (1) multiple incomplete vascular occlusions due to the subendothelial deposits composed of a homogeneous fibrin fibrinogen-like substance and platelets without the formation of polymerized fibrin; (2) vascular wall thickening due to the intramural granular deposits of IGM and beta 1C. A patient with hemolytic-uremic syndrome 9 days after the onset of the disease, exhibited (1) the formation of fibrin-thrombi in the glomerular capillary lumens, and (2) the granular deposits of IgM and beta 1C along the glomerular capillary walls and in the mesangium. In contrast, the case in which the symptoms subsided five weeks after the onset exhibited neither fibrin-thrombus formation nor the deposits of IgM and beta 1C in the glomeruli. The vascular lesions of disseminated intravascular coagulation accompanied by pancreas carcinoma was located mainly in the capillaries, and were characterized by the formation of numerous fibrin-thrombi. Although the glomeruli contained numerous thrombi, there was neither endocapillary proliferation nor deposits of immunoglobulins and complement components.
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