Hematology
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Three sets of diagnostic criteria for polycythaemia vera (PV); the Polycythaemia Vera Study Group (PVSG) criteria (1975), the British Committee for Standards in Haematology (BCSH) criteria (1996) and the World Health Organisation (WHO) criteria (2001) have been described. We compared the ability of each set of criteria to accurately diagnose PV and differentiate it from secondary, apparent and idiopathic erythrocytosis. A cohort was drawn from a clinical database of erythrocytosis patients currently attending the Belfast City Hospital and the relevant information from the time of diagnosis for each patient was assessed according to each set of criteria, with the BCSH criteria used as a comparator. ⋯ The PVSG criteria were limited by the unavailability of required data for some patients resulting in a sensitivity and specificity of 50% for PV and specificity of 100% for all other groups. The Janus kinase 2 (JAK2) V617F mutation was present in 34 (85.3%) PV, 2 (50%) IE, 1 (50%) apparent and no secondary erythrocytosis cases. We concluded that the BCSH criteria were the most accurate diagnostic criteria for PV as they had an acceptable level of sensitivity and could differentiate between PV and other erythrocytoses.
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The objective of this paper was to study the incidence, risk factors, clinical outcome, management and prevention of pure red cell aplasia (PRCA) following major ABO-incompatible allogeneic hematopoietic stem cell transplantation (allo-HSCT). We retrospectively analyzed 11 cases of PRCA from a series of 42 patients undergoing major ABO-incompatible allo-HSCT from April 1997 to December 2005. Eleven out of the 42 patients developed PRCA (26.1%). ⋯ We conclude that blood group A/O in donor/recipient pair is identified as being significantly associated with the occurrence of PRCA by multivariate analysis. Donor-type plasma exchange and anti-CD20 monoclonal antibody is an effective approach for the treatment of PRCA. PRCA could be prevented by plasma exchange prior to transplantation.
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Graft-versus host disease (GVHD) complicating allogeneic hematopoeitic stem cell transplantation (HSCT) is often attributed to mismatching of minor histocompatibility antigens (mHags), which are poorly defined in humans. CD31 is a candidate human mHag relevant to acute GVHD (aGVHD), but reports disagree about its level of significance. Therefore, we examined the impact of CD31-matching on the outcome of HSCT in different hematological and immunological diseases. ⋯ The CD31 nonidentity showed statistical significant relation with aGVHD (II-IV) versus no aGVHD (p = 0.012 and OR = 7.14) and also, aGVHD (0-I) versus the no aGVHD (p = 0.03, OR = 3.13, respectively). A statistical significant relation was found between CD31 nonidentity in patient-donor pairs and relapse (p = 0.014 and OR = 4.21). In conclusion, the donor-recipient CD31 nonidentity is a significant risk factor for aGVHD and relapse in HLA-identical sibling.
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Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by hemolytic anemia caused by fragmentation of erythrocytes and thrombocytopenia due to increased platelet aggregation and thrombus formation, eventually leading to disturbed microcirculation with reduced organ perfusion. Although several disease states may manifest as TMA, the two most relevant conditions associated with TMA are thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), characterized by prominent brain or renal lesions, respectively. However, occasionally the clinical distinction between these two conditions can be difficult. In this review, we focus on the epidemiologic and diagnostic criteria as well as on the most recent insights into the pathophysiology and treatment of these two conditions.
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Case Reports
The care of a child with multiple trauma and severe anemia who was a Jehovah's Witness.
Jehovah's Witness followers do not accept blood derived transfusions and available methods for avoiding transfusion have been used with degrees of success, demonstrating that the probability of death after trauma in these patients may not be significantly different from religious groups. In this report, we describe the case of a child victim of a multiple trauma with severe anemia due to blood loss, whose family would not authorize blood transfusion because of their Jehovah's Witness faith. We discuss the current indications for restricting transfusion, as well as highlighting new tools that contribute to the success of minimizing blood loss, thus avoiding transfusion.