Sickle cell disease (SCD) is the most commonly inherited hemoglobinopathy with the potential for significant maternal and perinatal morbidity and mortality. Hyperhemolysis syndrome (HS) is a rare, and potentially fatal complication of blood transfusion characterized by destruction of both transfused and autologous red blood cells, often resulting in severe anemia with post-transfusion hemoglobin levels lower than pre-transfusion levels. Evidence-based guidance on the prevention and management of HS during pregnancy is limited.
View Article and Find Full Text PDFMixed autoimmune hemolytic anemia (AIHA) is a rare and clinically complex hematologic disorder defined by the simultaneous presence of both warm and cold autoantibodies, resulting in severe and often treatment-resistant hemolysis. Due to variability in diagnostic criteria and limited data, a comprehensive understanding of its epidemiology, clinical characteristics, and management remains incomplete. To address these gaps, we performed a systematic literature review employing stringent diagnostic criteria to evaluate epidemiologic patterns, clinical features, and therapeutic outcomes.
View Article and Find Full Text PDFAnti-Kell (anti-K) alloimmunization is a known cause of severe hemolytic disease of the fetus and newborn (HDFN), yet the utility of a critical maternal antibody titer in guiding clinical management remains debated. We conducted a systematic review and meta-analysis to evaluate the diagnostic accuracy of a maternal anti-K titer threshold of ≥8 for predicting the need for intrauterine intervention due to severe anti-K-mediated HDFN. In parallel, we characterized all reported cases of severe HDFN occurring in the setting of low maternal anti-K titers (<8).
View Article and Find Full Text PDFImmune thrombotic thrombocytopenic purpura (iTTP) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and microvascular occlusion secondary to acquired ADAMTS13 deficiency. Contemporary data regarding iTTP treatment practices in the US, including the use of caplacizumab, are lacking. We aimed to characterize the demographics and therapies, including medications and apheresis practices, in patients with iTTP in the US.
View Article and Find Full Text PDFDisparities persist throughout medicine, including among conference speakership invitations. The National Institutes of Health have highlighted the importance of diversity at academic conferences. We assessed the gender composition of speakers at the American Society for Apheresis (ASFA) annual meeting.
View Article and Find Full Text PDFObjectives: Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy associated with severe deficiency in ADAMTS13. ADAMTS13 deficiency may be secondary to absent or dysfunctional protein production due to mutations in the ADAMTS13 gene (congenital TTP) or autoantibody-mediated clearance and/or inhibition (immune-mediated TTP). This autoimmunity may, albeit rarely, occur secondary to certain medications (eg, ticlopidine).
View Article and Find Full Text PDFArticle processing charges are increasingly being levied on authors via publication fees to provide open access to readers. These charges may impose challenges to early career physicians seeking to publish research but pathology journal article processing charges have not been investigated to date. We aimed to quantify pathology journal article processing charges and investigate the potential associated factors.
View Article and Find Full Text PDFObjectives: Inadequate laboratory infrastructure and testing capabilities are a major impediment to addressing the infectious disease burden in Africa. Therefore, the aims of this study were to characterize the clinical microbiology/infectious disease laboratory capabilities among countries in Africa.
Methods: A survey to assess the microbiological testing capabilities at hospitals, government laboratories, and free-standing public and private laboratories in African countries was developed by subject matter experts.
Common variable immunodeficiency (CVID) is a disorder characterized by hypogammaglobulinemia resulting in recurrent infections. While autoimmune disorders are common in patients with CVID, no association has been reported between CVID and immune thrombotic thrombocytopenic purpura (iTTP), a disorder most often caused by autoantibodies that compromise the activity of the enzyme ADAMTS13. Reduced ADAMTS13 activity results in the accumulation of large von Willebrand factor multimers that can consume platelets and cause microvascular thrombosis and organ injury, ultimately resulting in mortality in most cases of untreated iTTP.
View Article and Find Full Text PDFBackground: Trauma remains the leading cause of pediatric mortality in the United States. Although use of massive transfusion protocols (MTPs) in this population is widespread, optimal pediatric resuscitation is not well established. We sought to assess contemporary pediatric MTP practice in the United States.
View Article and Find Full Text PDFAm J Hematol
November 2024
Prior studies have suggested that immune thrombotic thrombocytopenic purpura (iTTP) may display seasonal variation; however, methodologic limitations and sample sizes have diminished the ability to perform a rigorous assessment. This 5-year retrospective study assessed the epidemiology of iTTP and determined whether it displays a seasonal pattern. Patients with both initial and relapsed iTTP (defined as a disintegrin and metalloprotease with thrombospondin type motifs 13 activity <10%) from 24 tertiary centers in Australia, Canada, France, Greece, Italy, Spain, and the US were included.
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