Acta haematologica
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Review Case Reports
Dominant von Willebrand disease type 2A groups I and II due to missense mutations in the A2 domain of the von Willebrand factor gene: diagnosis and management.
Pertinent findings in patients with von Willebrand disease (VWD) type 2A include prolonged bleeding time (BT), consistently low von Willebrand factor (VWF):ristocetin cofactor activity (RCo)/antigen concentration (Ag) and VWF:collagen binding (CB)/Ag ratios, absence of high, and (depending on severity) intermediate and large VWF multimers, the presence of pronounced triplet structure of individual bands and increased VWF degradation products due to increased proteolysis caused by mutations in the A2 domain of VWF. Two categories of VWD type 2A can be distinguished: group I with severe and group II with mild VWD. ⋯ Such transient complete responses to desmopressin (DDAVP) lasting only a few hours may facilitate treatment and prophylaxis of minor bleedings, but may not be able to prevent bleeding during minor and major surgery. Most VWD type 2A patients have pronounced VWD with very low VWF:RCo, prolonged BT, PFA-100 closure times >250 s, and response to DDAVP is only transient, minor, poor or absent, with no correction of the BT despite some increase in VWF:RCo, thus being candidates for factor VIII-VWF concentrate substitution for the acute and prophylactic treatment of bleeding symptoms.
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Comparative Study
Self-monitoring of oral anticoagulation therapy in children.
This study aimed to investigate the accuracy of home International Normalized Ratio (INR) self-monitoring in pediatric patients on long-term oral anticoagulation therapy. Statistical and clinical agreement of INR values from capillary whole blood samples measured by 2 different portable prothrombin time monitors (CoaguChek S and XS) and venous blood samples measured by a laboratory coagulation analyzer were evaluated using the Bland-Altman analysis. Eighty-three INR comparisons (56 using the CoaguChek S and 27 using the CoaguChek XS) were obtained from 35 children aged 4 months to 18 years. ⋯ Expanded and narrow agreements for all patients were 97.6 and 94%, respectively. In conclusion, home INR self-monitoring is accurate for children requiring long-term oral anticoagulation therapy. Our data suggest that INR self-monitoring with the newer CoaguChek XS is more accurate than with the older CoaguChek S monitor.
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Despite elegant regulatory mechanisms, iron deficiency anemia (IDA) remains one of the most common nutritional deficiencies of mankind. Iron deficiency is the result of an interplay between increased host requirements, limited external supply, and increased blood loss. When related to increased physiologic needs associated with normal development, iron deficiency is designated physiologic or nutritional. ⋯ Stratification by age cohorts in autoimmune gastritis implies a disease presenting as IDA many years before the establishment of clinical cobalamin deficiency. It is likely caused by an autoimmune process triggered by antigenic mimicry between H. pylori epitopes and major autoantigens of the gastric mucosa. Recognition of the respective roles of H. pylori and autoimmune gastritis in the pathogenesis of iron deficiency may have a strong impact on the diagnostic workup and management of unexplained, or refractory IDA.