1/9. Evans' syndrome complicating chronic graft versus host disease after cadaveric liver transplantation.Acute graft versus host disease (GVHD) occurred in a patient after cadaveric liver transplantation from an HLA disparate donor. immunosuppression resulted in a remission, but chronic GVHD with a scleroderma-like syndrome ensued. This was further complicated by immune hemolytic anemia and thrombocytopenia (Evan's syndrome). Semi-quantitative microsatellite analysis of circulating lymphoid cells showed that T cells were predominantly of donor origin, thereby explaining the chronic GVHD. The marrow hematopoietic cells remained recipient, so that the immune cytopenias were expected to be alloimmune in nature. However, the red cell antibodies were shown to have anti-C and anti-e specificity, with both the donor (R1R1) and recipient (R1r) possessing the C and e antigens. Therefore, the immune hemolysis might be considered both alloimmune and autoimmune. The patient finally died of sepsis. This case illustrates that chronic GVHD due to stable donor T cell engraftment may rarely occur in liver transplantation despite HLA disparity. immunosuppression may result in dysregulation of T cell functions, leading to alloimmune and autoimmune problems.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/9. Multicentric plasma cell variant of Castleman's disease with cutaneous involvement.BACKGROUND: Castleman's disease (CD) is a rare low-grade B-cell lymphoproliferative disorder that can be associated with a variety of antibody-mediated paraneoplastic syndromes. The disease is classified clinically by two forms and three histologic variants. methods: We describe the clinical and pathological features of a 44-year-old woman who presented with an autoimmune hemolytic anemia, thrombocytosis, polyclonal gammopathy, axillary lymphadenopathy, hepatosplenomegaly, and several erythematous and violaceous nodules and plaques without scaling involving the trunk and extremities. RESULTS: Histologic examination of the skin lesions revealed a deep dermal and subcutaneous nodular mononuclear infiltrate composed primarily of polyclonal plasmacytoid cells without atypia and an increased vascular proliferation. Additional studies including a bone marrow and lymph node biopsy, serum and urine protein electrophoresis, and computed tomography scans supported the diagnosis of multicentric plasma cell variant of CD with an associated autoimmune paraneoplastic hemolytic anemia. CONCLUSION: Cutaneous involvement in CD is part of the multicentric nature and should be considered in the differential diagnosis of a polyclonal plasma cell-rich lymphoproliferative disorder associated with paraneoplastic autoimmune disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/9. Intravascular lymphoma - a rare cause of hemolytic anemia and neurologic disorders.Intravascular lymphoma is an uncommon and often overlooked form of non-Hodgkin's lymphoma characterized by extensive proliferation of lymphoid cells within the lumina of small and medium-sized vessels. Clinical symptoms of the disease are variable and often nonspecific, mostly neurologic in nature. With an aggressive course, intravascular lymphomatosis has a poor prognosis and is rarely diagnosed ante mortem. We describe here a 76-year-old woman with the clinical diagnoses of hemolytic anemia and progressive lethargy where intravascular lymphomatosis turned out as the underlying cause of the disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/9. Potential value of intraoperative Doppler colour flow mapping in operations for complex intracardiac shunting.An eight year old boy presented with multiple residual intracardiac shunts and considerable persisting haemolytic anaemia five years after the repair of a single perimembranous ventricular septal defect. Preoperative transthoracic colour flow mapping showed an "acquired" left ventricular right atrial shunt and three small residual ventricular septal defects around the patch. These had been poorly visualised by both angiography and conventional cross sectional echocardiography and pulsed and continuous wave Doppler. Clearly, to stop the haemolysis effectively, closure of all residual intracardiac shunts was required. Intraoperative pre-bypass colour flow mapping was successfully used to confirm the precise number and nature of the defects and more importantly studies after bypass were used to confirm immediate closure of all defects.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/9. Erythrocyte glutathione S-transferase deficiency and hemolytic anemia.A patient with unexplained erythrocyte glutathione-S-transferase (GST) deficiency has been detected among 513 unrelated persons with hemolytic anemia. An otherwise healthy adult male, the deficient individual had a mild hemolytic anemia with splenomegaly, indirect hyperbilirubinemia, and cholelithiasis. Because he was adopted and childless, the hereditary nature of the defect could not be established. The residual enzyme activity was only about 15% of mean normal. Depletion of glutathione (GSH) from the cells by 1-chloro-2,4-dinitrobenzene (CDNB), a substrate for GST, was somewhat decreased in the red cells from the patient, suggesting that a functional defect existed. The kinetic properties of the residual enzyme and the ratio of activity to antigenicity were normal. Modest decreases in leukocyte and platelet GST activities were documented. Although a cause-and-effect relationship between the GST deficiency and hemolysis may exist, this cannot be proven in the absence of affected family members.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/9. Acute hemolysis associated with hepatitis a.A 62-yr-old woman, admitted to Mitsui Memorial Hospital with complaints of jaundice, general malaise and anorexia, was diagnosed as acute hepatitis a by positive IgM anti-HA. On the 13th hospital day, indirect hyperbilirubinemia (total bilirubin 57 mg/dl, indirect bilirubin 38.5 mg/dl) and splenomegaly were noted. Hemoglobin concentration decreased to 8.4 g/dl and reticulocytes increased to 6%. bone marrow showed hyperplastic marrow with erythroid hyperplasia. Coombs test was negative. After administration of prednisolone 40 mg daily for 30 days, hemoglobin concentration increased to 11.9 g/dl and total bilirubin was decreased to 0.4 mg/dl. The liver biopsy specimen obtained on the 62nd day, revealed recovery stage of acute hepatitis. jaundice with increased indirect bilirubin, anemia with reticulocytosis, elevated plasma hemoglobin concentration and predominance of LDH1 were indicative of hemolysis. The enzyme activities in the red blood cells, which were taken 1 year after the onset, were within normal limits. Scanning electron microscopy of the red cells showed no significant abnormalities in their shape and the nature of the surface.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/9. methemoglobinemia: nitrobenzene ingestion.In an unusually severe case of multiple toxin ingestion, a 48-year-old man ingested approximately 300 cc of Hoppe's Gunpower Solvent #9, which contains kerosene, denatured ethyl alcohol, oil and nitrobenzene. The major manifestation was nitrobenzene-induced methemoglobinemia of rapid onset. Treatment involves the judicious use of 1% methylene blue plus, in severe cases, hemodynamic and ventilatory support. The patient recovered after 48 hours and was transferred to the psychiatric service with continued follow-up for a mild poison-induced hemolytic anemia.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/9. quinidine-induced hemolytic anemia: immunohematologic characterization.A 40-year-old white male with quinidine induced hemolytic anemia but no thrombocytopenia is described. hemolysis was extravascular in nature and via the immune-complex mechanism. The quinidine antibody was an IgG immunoglobulin that did not activate complement.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/9. Heinz-body anemia: "bite cell" variant--a light and electron microscopic study.light and scanning electron microscopic studies of blood from five patients with drug-induced oxidant hemolysis are presented. None of the patients had a previous history of hemolytic disease and laboratory studies indicated no evidence of either glucose-6-phosphate dehydrogenase (G6PD) deficiency or unstable hemoglobinopathy. Although the red cell deformities in our patients overlapped to some extent with those reported in patients with microangiopathic hemolytic anemia (MAHA) and in patients with G6PD deficiency undergoing oxidant hemolysis, striking differences were also observed. Cell fragments, commonly found in patients with MAHA, and eccentrocytes, frequently found in patients with G6PD deficiency undergoing oxidant hemolysis, were seldom found in blood samples from the five patients in this study. Bite cells were extremely common in our patients. They are rare in patients with either of the above disorders. An awareness of the morphologic abnormalities detailed in this report may help characterize the nature of a hemolytic process so that appropriate therapy can be initiated.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |