Refsgaardgrossman1404

Autoimmune hemolytic anaemia (AIHA) has traditionally been classified based on the temperature sensitivity of the autoagglutinins as warm (WAIHA), cold (CAIHA) and mixed type. Autoagglutinin may be of IgG or IgM type. The present prospective study was conducted to evaluate the profile of clinical picture, severity of haemolysis, treatment response of steroid. This study on patients of adult primary AIHA was conducted by taking complete history followed by detail physical examination. Laboratory investigations were performed to establish haemolytic anaemia and to assess severity of haemolysis. Immunehematological work up including blood grouping, direct antiglobulin test (DAT), IAT, antibody screening, adsorption elution was performed to diagnose type of AIHA. All cases were followed up to assess the response to prednisolone. All the data were collected and analysed by SPSS 19. Out of 62 primary AIHA cases, female were affected more than male (4121). WAIHA is most common type (42, 67.8%) followed by mixed (20.9%) and cold AIHA (11.3%). Severity of haemolysis showed significant correlation with the DAT strength and not with type of AIHA. (P  less then  0.05) On oral prednisolone, 22 cases attended complete remission, while relapse, drug dependency and partial remission was achieved in 13, 9, 3 cases respectively. learn more Severity of haemolysis in AIHA is directly related with DAT strength. WAIHA is most common type and can be managed with oral prednisolone (cr 45.2%), without red cell transfusion in most of cases. Mixed type AIHA cases were presented mostly with severe haemolysis, with minimum therapeutic response to prednisolone and maximum relapse/drug dependency.Bone marrow examination entails study of aspirate smears, touch imprints and trephine biopsy. Bone marrow aspirate smears can be prepared by the squash (crush) or wedge method. Both techniques have their own advantages and disadvantages. There is paucity of studies that have compared these smear types. This study was conducted over a period of one year. Two hundred and five bone marrow aspirates were included. Both squash and wedge smears were made. Blinded slide review was done. Bone marrow cellularity, megakaryocyte number, myeloid to erythroid (ME) ratio, morphology and final diagnosis on each smear type was compared. Chi square test, t-test and Kappa were applied to study the agreement between the wedge and squash smears. Among the 205 patients studied, squash smears showed significant over estimation of cellularity and megakaryocyte number (p  0.05) in the ME ratio and morphological scores. Most patients (188/205 [91.7%]) showed complete diagnostic concordance while 17 (8.3%) patients had discrepancy in diagnosis between the squash and wedge smears. In 8 (3.9%) of these, major discrepancies were seen while 9 (4.4%) patients had minor discrepancies. Bone marrow cellularity and megakaryocyte numbers were underestimated in wedge smears with no differences in ME ratio or morphology. Acceptable agreement for diagnosis was seen for patients with most disorders. Major diagnostic discrepancies were seen in patients with lesions known to have focal distribution-lymphoma, myeloma and tuberculosis (granulomas). Both squash and wedge smears should be studied for bone marrow examination.Dabigatran etexilate is an oral direct thrombin (Factor IIa) inhibitor approved for patients with atrial fibrillation and for management of risk of deep vein thrombosis and pulmonary embolism. Dabigatran offers advantages over treatment with warfarin, including limited laboratory monitoring. It is equivalent in prevention of stroke and deep vein thrombosis with essentially equivalent complication rates. In contrast to warfarin, reversal of the anticoagulation is less well established. Idarucizumab is available for reversal, however supporting research is mixed; the agent also happens to be quite expensive making availability difficult. Hemodialysis has been proposed as a method of reversal, but this is difficult in patients with life threatening hemorrhage, and is not available at many hospitals. Intravenous fat emulsion (IFE) has been used for treatment of overdose of lipophilic drugs. Most toxicologists only recommend IFE for patients in extremis after ingestion of a lipid soluble substance. Dabigatran is lipid soluble, although the pro-drug more so than the active metabolite. The authors sought to see if dabigatran-induced coagulopathy of human in vitro blood samples could be reversed with IFE. Blood samples were spiked with dabigatran or dabigatran plus IFE. Values for Ecarin clot time (ECT-primary outcome), PT/INR, and aPTT, were compared across both study arms. A total of 18 healthy volunteers were included in our study. There were no significant differences in the ECT, PT/INR, and aPTT between the dabigatran arm and the dabigatran plus IFE arm. Based on these methods, IFE does not reverse dabigatran-induced coagulopathy.

Inherited bleeding disorders can lead to lifelong bleeding; they are mainly caused by quantitative or qualitative defect of coagulation factors, von Willebrand factor (VWF) or platelets. No published data are available about the different types of inherited bleeding disorders in Iraq.

To describe types, severity and presentation of inherited bleeding disorders in pediatric patients in the major referral center in Iraq.

This is a cohort prospective descriptive study conducted at the National Center of Hematology, a referral center for bleeding disorders in Baghdad-Iraq, from January 2015 to December 2019. One hundred ninety-one patients, aged 1day to 14years, with suspected inherited bleeding disorder are included in this study. Each patient was interviewed, accompanied by a chaperone, mostly parent(s), with revision of personal and familial bleeding history, conducting a brief medical examination, and withdrawing blood for complete blood count, peripheral blood film, bleeding time, PT and APTT. Further rriage should be discouraged in our society to decrease hereditary bleeding disorders. Also, there is a need to increase awareness and knowledge of bleeding disorders to improve early identification, mitigate the risk of further bleeding and prevent complications.

86% of patients. Consanguineous marriage should be discouraged in our society to decrease hereditary bleeding disorders. Also, there is a need to increase awareness and knowledge of bleeding disorders to improve early identification, mitigate the risk of further bleeding and prevent complications.