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Hemolytic anemia

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https://www.readbyqxmd.com/read/29222317/thrombocytopenia-in-hospitalized-patients-approach-to-the-patient-with-thrombotic-microangiopathy
#1
REVIEW
Marie Scully
Thrombotic microangiopathies (TMAs), specifically, thrombotic thrombocytopenic purpura (TTP) and complement-mediated hemolytic uremic syndrome (CM-HUS) are acute life-threatening disorders that require prompt consideration, diagnosis, and treatment to improve the high inherent mortality and morbidity. Presentation is with microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) and variable organ symptoms resulting from microvascular thrombi. Neurological and cardiac involvement is most common in TTP and associated with poorer prognosis and primarily renal involvement in CM-HUS...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222291/evolving-treatment-paradigms-in-sickle-cell-disease
#2
REVIEW
Ramasamy Jagadeeswaran, Angela Rivers
Sickle cell disease (SCD) is an inheritable hemoglobinopathy characterized by polymerization of hemoglobin S in red blood cells resulting in chronic hemolytic anemia, vaso-occlusive painful crisis, and multiorgan damage. In SCD, an increased reactive oxygen species (ROS) generation occurs both inside the red blood cells and inside the vascular lumen, which augment hemolysis and cellular adhesion. This review discusses the evolving body of literature on the role of ROS in the pathophysiology of SCD as well as some emerging therapeutic approaches to SCD with a focus on the reduction of ROS...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222288/cardiovascular-complications-in-patients-with-sickle-cell-disease
#3
REVIEW
Mark T Gladwin
Sickle cell disease (SCD) is an autosomal recessive disease in which homozygosity for a single point mutation in the gene encoding the β-globin chain produces hemoglobin S molecules that polymerize within the erythrocyte during deoxygenation; the result is sustained hemolytic anemia and vaso-occlusive events. As patients live to adulthood, the chronic impact of sustained hemolytic anemia and episodic vaso-occlusive episodes leads to progressive end-organ complications. This scenario culminates in the development of 1 or more major cardiovascular complications of SCD for which there are no approved or consensus therapies...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222267/new-therapeutic-targets-in-transfusion-dependent-and-independent-thalassemia
#4
REVIEW
M Domenica Cappellini, Irene Motta
β-Thalassemias are characterized by reduced production of β-globin chain, resulting in α/β-chain unbalance and precipitation of α-globin-heme complexes and determining ineffective erythropoiesis. Ineffective erythropoiesis, chronic hemolytic anemia, and compensatory hematopoietic expansion are the disease hallmarks, and they are related to the severity of the chain unbalance. Several clinical forms of β-thalassemia, including the coinheritance of β-thalassemia with hemoglobin E resulting in hemoglobin E/β-thalassemia, have been described...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29221667/interleukin-27-and-interleukin-37-are-elevated-in-sickle-cell-anemia-patients-and-inhibit-in-vitro-secretion-of-interleukin-8-in-neutrophils-and-monocytes
#5
A E Alagbe, A S Justo Junior, L P Ruas, W V Tonassé, R M Santana, T H C Batista, I F Domingos, A S Araujo, M A C Bezerra, M N N Santos, M H S L Blotta
BACKGROUND AND OBJECTIVE: Inflammation is implicated in the pathogenesis of most complications seen in sickle cell anemia (SCA) patients. We aimed to evaluate serum levels of two newly discovered anti-inflammatory cytokines (IL-27 and IL-37), and pro-inflammatory cytokines among Brazilian SCA patients that are not on hydroxyurea therapy (HbSS), compared with hydroxyurea-treated patients (HbSSHU) and healthy controls (HbAA). Furthermore, we demonstrated the effect of IL-27, IL-37, and heme on in vitro secretions of IL-8 in human neutrophils and monocytes...
December 5, 2017: Cytokine
https://www.readbyqxmd.com/read/29219891/thrombotic-thrombocytopenic-purpura-in-a-child-treated-for-acute-lymphoblastic-leukemia-case-report-and-review-of-literature
#6
Francesco De Leonardis, Roberta Koronica, Rosa Maria Daniele, Nicola Santoro
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy caused by deficiency of von Willebrand factor-cleaving protease ADAMTS13. Large von Willebrand multimer formation and intravascular platelet aggregation affecting the arterioles and capillaries can result in death unless early treatment is administered. We report on the case of a child with TTP associated with a human herpes virus type-6 infection occurring during chemotherapy for acute lymphoblastic leukemia who was effectively treated by fresh frozen plasma infusions and antiviral therapy...
December 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29219637/first-cases-of-hb-agrinio-described-in-patients-from-the-republic-of-macedonia
#7
Marija Dimishkovska, Maja Kuzmanovska, Svetlana Kocheva, Kata Martinova, Oliver Karanfilski, Zlate Stojanoski, Dijana Plaseska-Karanfilska
Previous molecular analyses of α-thalassemia (α-thal) in the Republic of Macedonia have identified the following genetic defects: -α3.7 (rightward), -(α)20.5 and - -MED I deletions and Hb Icaria [α142, Term→Lys (α2), HBA2: c.427T>A] and polyadenylation signal (polyA) [AATAAA>AATGAA (α2), HBA2: c.*92A>G] point mutations. Here, we report two unrelated patients from the Romani population in the Republic of Macedonia, homozygotes for the α2-globin gene variant Hb Agrinio [α29(B10)Leu→Pro; HBA2: c...
December 8, 2017: Hemoglobin
https://www.readbyqxmd.com/read/29214674/neutrophil-extracellular-traps-in-plasma-from-dogs-with-immune-mediated-hemolytic-anemia
#8
C Lawson, S A Smith, M O'Brien, M McMichael
BACKGROUND: Neutrophil extracellular traps (NETs) are part of the innate immune response and are essential in local pathogen control, but are associated with pathological inflammation, organ damage, autoimmunity, and thrombosis. Immune-mediated hemolytic anemia (IMHA) is a pro-inflammatory, prothrombotic disease associated with high mortality. HYPOTHESIS/OBJECTIVES: Neutrophil extracellular traps (NETs) are a feature of the inflammatory process in dogs with IMHA...
December 7, 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/29214442/shiga-toxin-triggers-endothelial-and-podocyte-injury-the-role-of-complement-activation
#9
REVIEW
Carlamaria Zoja, Simona Buelli, Marina Morigi
Shiga toxin (Stx)-producing Escherichia coli (STEC) is the offending agent in post-diarrhea-associated hemolytic uremic syndrome (HUS), a disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney failure, with thrombi occluding the renal microvasculature. Endothelial dysfunction has been recognized as the trigger event in the development of microangiopathic processes. Glomerular endothelial cells are susceptible to the toxic effects of Stxs that, via nuclear factor kappa B (NF-κB) activation, induce the expression of genes encoding for adhesion molecules and chemokines, culminating in leukocyte adhesion and platelet thrombus formation on the activated endothelium...
December 6, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29214337/sleep-disordered-breathing-in-patients-with-sickle-cell-disease
#10
REVIEW
Vikram M Raghunathan, Peter L Whitesell, Seah H Lim
Sickle cell disease is one of the most common hereditary hemoglobinopathies worldwide, and its vaso-occlusive and hemolytic crises cause considerable patient morbidity. A growing body of evidence has shown that sleep-disordered breathing, and in particular, obstructive sleep apnea, occurs at high frequency in the sickle cell population, and that there is significant overlap in the underlying pathophysiology of these two conditions. Through a variety of mechanisms including nocturnal hypoxemia and increased oxidative stress, production of pro-inflammatory cytokines, and endothelial dysfunction, sickle cell anemia and sleep-disordered breathing potentiate each other's clinical effects and end-organ complications...
December 6, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/29212341/scavenging-reactive-oxygen-species-production-normalizes-ferroportin-expression-and-ameliorates-cellular-and-systemic-iron-disbalances-in-hemolytic-mouse-model
#11
Naveen Kumar Tangudu, Betuel Alan, Francesca Vinchi, Katharina Woerle, Dilay Lai, Sabine Vettorazzi, Kerstin Leopold, Maja Vujic Spasic
AIMS: The release of large amounts of free heme into the circulation, overproduction of reactive oxygen species (ROS) and the activation of toll-like receptor (Tlr)-4 dependent responses are considered critical for the ability of heme to promote oxidative stress and to initiate pro-inflammatory responses, posing a serious threat to the body. A deep understanding of the consequences of heme overload on the regulation of cellular and systemic iron homeostasis is however, still lacking. RESULTS: The effects of heme on iron metabolism were studied in primary macrophages and in mouse models of acute and chronic hemolysis...
December 6, 2017: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/29200157/an-infant-with-unusually-high-unconjugated-hyperbilirubinemia-due-to-coexistence-of-hereditary-spherocytosis-and-gilbert-syndrome
#12
Ivona Butorac Ahel, Kristina Baraba Dekanic, Goran Palcevski, Jelena Roganovic
Hereditary spherocytosis is the most frequent congenital hemolytic anemia and is characterized with variable degree of anemia, jaundice, and splenomegaly. In the case of severe hyperbilirubinemia out of proportion with hemolysis, other causes of hyperbilirubinemia must be considered. Gilbert syndrome (GS) is an autosomal dominant disorder characterized with intermittent hyperbilirubinemia without any other sign and symptom of liver disease as a result of reduced activity of uridine diphosphate-glucuronyl transferase 1A1...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29198692/successful-recombinant-thrombomodulin-treatment-for-thrombotic-microangiopathy-after-liver-transplantation-a-case-report
#13
T Motomura, T Yoshizumi, M Ohira, Y Mano, T Toshima, S Itoh, N Harada, N Harimoto, T Ikegami, Y Soejima, Y Maehara
Transplant-associated thrombotic microangiopathy (TA-TMA) is a rare but severe complication after liver transplantation. In contrast to other thrombotic microangiopathies, treatment for TA-TMA has yet to be clarified. A 52-year-old male patient with liver cirrhosis due to hepatitis C underwent split liver transplantation from a deceased donor. His clinical course was without complication until 4 days after transplantation, when he experienced impaired consciousness, hemolytic anemia with fragmented erythrocytes, and marked thrombocytopenia...
December 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/29194292/fetal-splenomegaly-a-review
#14
Marta Moreira, Rafael Brás, Daniela Gonçalves, Inês Alencoão, Gonçalo Inocêncio, Maria Rodrigues, Jorge Braga
Enlargement of the fetal spleen is usually found secondary to systemic diseases and is frequently associated with hepatomegaly. By far, the most common causes of fetal splenomegaly are infectious. Other etiologies responsible for this sign are hemolytic anemia, congestive cardiac failure, metabolic disorders, and rarely, leukemia, lymphoma, and histiocytosis.We report a case of prenatal splenomegaly diagnosed at 35 weeks, confirmed in the postnatal period. The postnatal workup showed the newborn had a familial type 3 form of hemophagocytic lymphohistiocytosis (HLH)...
November 29, 2017: Ultrasound Quarterly
https://www.readbyqxmd.com/read/29193111/clinical-input-of-anti-d-quantitation-by-continuous-flow-analysis-on-autoanalyzer-in-the-management-of-high-titer-anti-d-maternal-alloimmunization
#15
Cécile Toly-Ndour, Haifa Mourtada, Stéphanie Huguet-Jacquot, Emeline Maisonneuve, Stéphanie Friszer, Françoise Pernot, Pauline Thomas, Jean-Marie Jouannic, Bruno Carbonne, Anne Cortey, Agnès Mailloux
BACKGROUND: In addition to titration by indirect antiglobulin test most widely used, anti-D quantitation by continuous-flow analysis (CFA) may be performed to assess severity of maternal immunization. Only five studies have reported its added value in the management of pregnancies complicated by anti-D immunization. STUDY DESIGN AND METHODS: A retrospective study of 74 severe anti-D-immunized pregnancies was conducted from January 1, 2013, to December 31, 2014, in the Trousseau Hospital in Paris (France)...
November 29, 2017: Transfusion
https://www.readbyqxmd.com/read/29187036/persistence-of-villous-immaturity-in-term-deliveries-following-intrauterine-transfusion-for-parvovirus-b19-infection-and-rhd-associated-hemolytic-disease-of-the-fetus-and-newborn
#16
Whitney A McCarthy, Edwina J Popek
Common causes of fetal anemia and hydrops include parvovirus B19 infection during the first 2 trimesters of pregnancy, as well as maternal alloimmunization to RhD with subsequent hemolytic disease of the fetus and newborn (HDFN) in an RhD positive fetus. Although both of these conditions have historically caused significant fetal morbidity and mortality, the advent of intrauterine transfusion (IUT) over the last few decades have dramatically improved outcomes. Prior literature has extensively documented placental changes associated with untreated parvovirus infection and RhD HDFN in intrauterine fetal demises and preterm births; however, histopathologic changes in term placentas from term infants treated with IUT have not been reported...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29181179/efficacy-of-bendamustine-on-thrombocytopenia-and-hemolytic-anemia-secondary-to-cd5-positive-b-cell-lymphoma-with-massive-splenomegaly-in-a-patient-with-rheumatoid-arthritis
#17
Yuzuru Hosoda, Hiroshi Hagino, Norihiko Hino, Toru Motokura
Chemotherapy for lymphoma may be avoided in the presence of coincident cytopenia. In case of immune cytopenia secondary to lymphoma, treatment of cytopenia is the same for primary cases, however, chemotherapy for lymphoma may be effective at the cost of severe hematological toxicity. The present study reports a complex case of thrombocytopenia and direct antiglobulin test-negative hemolytic anemia, thus mimicking Evans syndrome, secondary to cluster of differentiation 5-positive B-cell lymphoma with massive splenomegaly, in a patient suffering from rheumatoid arthritis for two decades...
November 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/29174474/iron-overload-in-hematological-disorders
#18
Eitan Fibach, Eliezer A Rachmilewitz
While most common symptom of impairment of iron homeostasis is iron deficiency anemia, some hematological disorders are associated with iron overload (IO). These disorders are related mainly to chronic severe hemolytic anemia, where red blood cells (RBC) or their precursors are destroyed prematurely (hemolyzed), leading to anemia that cannot be compensated by increased production of new RBC. In such cases, IO is mainly due to repeated RBC transfusions and/or increased uptake of iron in the gastrointestinal tract...
November 22, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/29171101/in-vitro-inhibition-of-canine-complement-mediated-hemolysis
#19
D M Hernandez, R Goggs, E Behling-Kelly
BACKGROUND: Immune-mediated hemolytic anemia (IMHA) is the most common hematologic immune-mediated disease in dogs. Complement fixation on erythrocytes causes hemolysis. Complement inhibition decreases hemolysis in people with the hemolytic disease and also may prove effective in treating IMHA in dogs. HYPOTHESIS/OBJECTIVES: Evaluate the in vitro efficacy of 2 complement inhibitors used in humans against canine complement. METHODS: The inhibitory activity of the C3-inhibitor compstatin and recombinant human C1-esterase inhibitor (C1-INH) was evaluated using an in vitro hemolytic assay and spectrophotometric measurement of released hemoglobin...
November 24, 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/29169341/prevalence-genetic-variants-and-clinical-implications-of-g-6-pd-deficiency-in-burkina-faso-a-systematic-review
#20
Abdoul Karim Ouattara, Pouiré Yameogo, Lassina Traore, Birama Diarra, Maléki Assih, Tegwindé Rébéca Compaore, Dorcas Obiri-Yeboah, Serge Théophile Soubeiga, Florencia Wendkuuni Djigma, Jacques Simpore
BACKGROUND: It is now well-known that some antimalarials such as primaquine may induce severe hemolytic anemia in people with G-6-PD deficiency. Antimalarial drug prescriptions must, therefore take into account the patient's G-6-PD status in malaria endemic areas such as Burkina Faso, where the prevalence of this genetic abnormality is relatively high. Although great clinical heterogeneity is observed depending on the molecular nature of the deficiency and the residual enzyme activity in the red blood cell, there is very poor data on the prevalence of G-6-PD deficiency and the distribution of involved genetic variants in Burkina Faso...
November 23, 2017: BMC Medical Genetics
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