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Sickle Cell Disease
Published in Vincenzo Berghella, Maternal-Fetal Evidence Based Guidelines, 2022
An abnormal variant of hemoglobin is HbS, which occurs because of a single nucleotide mutation in which valine is substituted for glutamic acid at position 6 (E6V substitution). This substitution replaces a hydrophobic amino acid in place of a hydrophilic amino acid in the β-globin gene. This mutation does not change the morphology of hemoglobin under normal oxygen concentration, but allows HbS to polymerize when in conditions of low oxygen concentration. This polymerization triggers a cascade of repeated injury to the red-cell membrane, which causes the red blood cell to assume a characteristic sickle shape. The sickled red blood cells are brittle, causing increased hemolysis and difficulty passing through small capillaries, leading to vessel occlusion and ischemia. This tissue ischemia leads to acute and chronic pain, as well as to end-organ damage. As vaso-occlusion can occur in any vessel, this is a systemic disease that can affect multiple organs. The life span of a sickle cell is about 10–20 days compared to the 120 days life span of a normal red blood cell. This chronic hemolysis contributes to the anemia [1, 5, 6]. Dehydration, infection, decrease in oxygen tension, and acidosis, are common triggers of cell sickling and sickle cell crisis. Sickle cell crisis is a term used to label several different and independent acute conditions occurring in patients with sickle cell disease (vaso-occlusive crisis, aplastic crisis, hemolytic crisis).
Sickle cell disease
Published in Hung N. Winn, Frank A. Chervenak, Roberto Romero, Clinical Maternal-Fetal Medicine Online, 2021
Marc R. Parrish, John C. Morrison
The occurrence of a vaso-occlusive crisis during labor offers additional challenges to the provider. Obviously, with painful uterine contractions, the diagnosis of a vaso-occlusive crisis may be more difficult. If delivery is expected within a short time, a simple transfusion of two units of leukocyte-reduced washed red cells can be considered. During labor, patients should remain in the lateral recumbent position and receive oxygen by tight-fitting face mask. Careful monitoring of maternal and fetal vital signs is essential. Usually, when crisis has been diagnosed during labor and late decelerations appear, infusion of blood products has been associated with resolution of suspected fetal hypoxemia. Maternal blood gas assessment, if necessary, is carried out, but invasive hemodynamic monitoring is avoided unless other concomitant disease processes such as preeclampsia, and so on, are present. Urinary catheters, as well as intrauterine catheters, are discouraged, because of their association with increased infection.
Rheology of the Hemolytic Anemias
Published in Gordon D. O. Lowe, Clinical Blood Rheology, 2019
As in the case of ISC, serial rheological studies of density-gradient separated RSC during vaso-occlusive crises have not been described, so that the etiological role of these cells in vaso-occlusion is conjectural. Recent rheological studies of oxygenated unfractionated populations of sickle cells indicate, however, that significant changes in RSCs occur at the time of onset of vaso-occlusive crisis. A temporary loss of filterability through 5 μm diameter pores, which reversed as crisis resolved,156 has been demonstrated using positive-pressure167 and gravity filtration166 techniques. Sickle cell crisis is particularly difficult to study using filtration techniques, however, owing to the acute-phase increases in fibrinogen concentration and leukocyte count which themselves impair filtration.157 An accompanying reticulocytosis, which has been reported in some studies159 but not others,167 a macrocytosis,168 and an increase in normoblasts will also complicate the interpretation of impaired filtration in sickle cell crisis. One filtration study, of a leukocyte- and protein-free erythrocyte suspension, in which ISC did not increase, showed impaired filtration of oxygenated erythrocytes on day 2 of crisis166 implying that rheological changes had occurred within the RSC population.
Audit of clinical and laboratory parameters of hemoglobin SS patients in a Nigerian teaching hospital
Published in Annals of Medicine, 2022
Titilola Stella Akingbola, Oladapo Wale Aworanti, Sunday Peter Ogundeji
This was a descriptive cross-sectional study of 90 adult HbSS patients diagnosed with hemoglobin electrophoresis who are being followed up at the Hematology department of the Teaching Hospital in Nigeria between January and June 2017. They were classified into three groups based on their clinical presentations: steady state, hemolytic and vaso-occlusive crises with 30 individuals in each group. Steady state patients presented for routine follow up visits at the Hematology Out-patient clinic while the other two groups presented in either Hemolytic or Vaso occlusive crisis to either the Hematology Day-care Unit (HDCU) or the emergency department of the hospital. Steady state is defined as stable health state in HbSS patients who did not have bone pain or any other crisis and no blood transfusions in the previous 2 months [10]. Vaso-occlusive crisis (VOC) group: Vaso-occlusive crisis is defined as the occurrence of pain in the extremities, back, chest (ribs, sternum) that lasted for at least 2 h, led to a hematology day-care unit visit, and could not be explained except by sickle cell disease without features of hemolysis [11,12]. Hemolytic crisis is defined as ≥3% reduction in hematocrit, marked reticulocytosis, circulating nucleated red blood cells, polychromasia, unconjugated bilirubinemia and increased urobilinogen in sickle cell anemia [11,12]. The individuals with concurrent overt infection, pregnancy, other SCD, those with concurrent crises and on hydroxyurea (HU) were excluded. Those on Hydroxyurea were excluded due to low penetration of HU in our environment.
Clinical Features and Outcome of Sickle Cell Disease in a Tertiary Center in Northern Lebanon: A Retrospective Cohort Study in a Local, Hospital-Associated Registry
Published in Hemoglobin, 2021
Adlette Inati, Chadi Al Alam, Cristel El Ojaimi, Taghrid Hamad, Hemanth Kanakamedala, Virginia Pilipovic, Ramzieh Sabah
Sickle cell disease, a multi-system disorder caused by a single gene mutation, is characterized by hemolytic anemia, vaso-occlusion, endothelial dysfunction, organ failure, and significant lifetime morbidity and early mortality [1,2]. Sickle cell disease is a disorder of global importance with both economic and clinical significance [1]. Many children with sickle cell disease, specifically those born in underdeveloped countries, die undiagnosed or in early childhood from sepsis and/or acute splenic sequestration (ASS) due to a lack of access to medical care [3]. Others may die later from disease complications, including stroke, acute chest syndrome (ACS), and end-organ failure or from the consequences of under-recognized chronic iron overload resulting from blood transfusions [4]. Recurrent episodes of vaso-occlusive crisis (VOC), the clinical hallmark of sickle cell disease, are unpredictable and extremely painful events that can lead to serious acute and chronic complications and repeated inpatient hospitalization [5–8].
Drepa-Opia: A Pilot Study to Determine the Predictive Factors of Morphine Use and Consumption in Hospitalized Adult Patients with Sickle Cell Disease
Published in Hemoglobin, 2018
Marie Sabrie, Giovanna Cannas, Karim Tazarourte, Solène Poutrel, Philippe Connes, Arnaud Hot, Céline Renoux, Jihane Fattoum, Philippe Joly
Sickle cell disease is a leading world genetic disease affecting about 4.0% of the world’s population [1]. Vaso-occlusive crisis (VOC), the most common cause of hospitalization of patients with sickle cell disease, occurs when microcirculation is obstructed by sickled red blood cells (RBCs), causing ischemic injury to organs and acute pain. Vaso-occlusive crisis outcome is highly unpredictable and may be triggered by known (sepsis, pregnancy, dehydration, and asthenia) and unknown risk factors [2–5]. Evidence from several randomized controlled trials and observational studies support the use of opioid therapy in treating VOCs pain [6–8]. According to the 2014 American Expert Panel and to the last British and French guidelines [9–11], parenteral morphine is the drug of choice for severe pain requiring hospitalization.