Annals of hematology
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Annals of hematology · Apr 2003
Comparative StudyDiagnostic value of serum transferrin receptor and glycosylated hemoglobin on hemolytic anemia.
Soluble serum transferritin receptor (sTfR) is a new diagnostic tool for iron depletion and erythropoiesis. Glycosylated hemoglobin (GHb) can be used to detect hemolysis. The present study was thus conducted to compare the diagnostic value of sTfR and GHb (measured as Hb A(1)c) in patients with hemolytic anemia. ⋯ Reticulocyte count was significantly different only between the two hemolytic groups. Hb A(1)c and sTfR were both good for the diagnosis of hemolysis. Reticulocyte count was a good tool for distinguishing EE from IE.
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Annals of hematology · Mar 2003
Review Case ReportsAllogeneic stem cell transplant from HLA-identical sibling for chronic granulomatous disease and review of the literature.
Chronic granulomatous disease (CGD) is a rare primary immunodeficiency caused by an abnormal function of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase in the phagocytic cells, which results in an increased susceptibility to severe bacterial and fungal infections. We report on a 12-year-old boy with X-linked CGD who was successfully treated with allogeneic bone marrow transplantation from an HLA-identical sibling following a conditioning regimen consisting of busulphan (BU, 16 mg/kg) and cyclophosphamide (CY, 200 mg/kg). ⋯ A review of the literature revealed that 20 of 24 CGD patients are alive and disease free 1-7 years after transplant. Most of these patients were conditioned with the BUCY combination, which should be considered the recommended regimen.
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Annals of hematology · Feb 2003
Case ReportsEffect of prothrombin complex concentrate on INR and blood coagulation system in emergency patients treated with warfarin overdose.
We investigated the effect of prothrombin complex concentrate (PCC) on the international normalized ratio (INR) and blood coagulation system in two emergent patients treated with warfarin for secondary prevention of cardioembolic stroke due to nonvalvular atrial fibrillation. An 80-year-old woman developed massive subcutaneous hemorrhage and swelling on her right upper extremity with weak pulsation of the right radial artery and had an INR above 10. An 83-year-old man had pleural effusion with an INR value of 6.69 and pleural puncture was immediately required. ⋯ Anticoagulation was restarted in the latter patient after 14 days of PCC administration. There were no embolic episodes during the month after PCC administration. In conclusion, a small amount of PCC may be effective in immediately correcting increased INR levels with increased plasma levels of protein C and coagulant factors IIa, VIIa, IXa, and Xa and may partially activate the coagulation system without any effects on plasma levels of D-dimer.
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Mastocytosis consists of a group of disorders characterized by a pathologic increase in mast cells in tissues including skin, bone marrow, liver, spleen, and lymph nodes. Mastocytosis is a rare disease. Because of this, general practitioners have limited exposure to its clinical manifestations, diagnosis, classification, and management. ⋯ Despite recent advances in knowledge about the pathophysiology, diagnosis, and classification of mastocytosis, a curative treatment for mastocytosis does not now exist. Management of patients within all categories of mastocytosis includes: (1) a careful counseling of patients (parents in pediatric cases) and care providers, (2) avoidance of factors triggering acute mediator release, (3) treatment of acute mast cell mediator release, (4) treatment of chronic mast cell mediator release, and if indicated (5) an attempt to treat organ infiltration by mast cells. The goal of this manuscript is to provide an overview of the mediators produced and released by mast cells, the diagnostic criteria for the different variants of mastocytosis, and the treatment options currently available.
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Annals of hematology · Dec 2002
Bilateral adrenal swelling as a cause of chest, back, and upper abdominal pain in essential thrombocythemia and polycythemia vera is due to microvascular ischemic thrombosis rather than to hemorrhage.
Bilateral massive adrenal swelling (BAS) on computed tomography (CT) scan with no enhancement after injection of intravenous contrast media has been observed in two completely different clinical settings. On the one hand, BAS is the result of ischemic necrosis and subsequent hemorrhagic infarction in patients with sepsis and hypotension in critically ill situations. On the other hand, BAS is the result of microvascular thrombosis, ischemia, and secondary inflammatory swelling in the setting of thrombotic conditions such as antiphospholipid syndrome (APS), heparin-induced thrombocytopenia and thrombosis (HITT), and thrombocythemia. In this study we present evidence that the etiology of unilateral or BAS in reported cases of essential thrombocythemia (ET) and polycythemia vera (PV) is similar to the etiology of microvascular circulation disturbances in thrombocythemia caused by platelet-mediated inflammation and thrombosis in the peripheral, cerebral, and/or coronary endarterial microvascular circulation.