Acute pancreatitis in a child with sickle cell anemia.

A case of pancreatitis that occurred as a complication of a vaso-occlusive crisis in a child with sickle cell anemia is reported. We encourage others to consider pancreatitis as a cause for abdominal pain in children with multisystem diseases, particularly those that may cause ischemic organ injury such as sickle cell anemia.     

Microcytosis associated with sickle cell anemia.

Sickle cell (Hb SS) anemia is considered a normochromic-normocytic hemolytic disorder. In 53 patients with Hb SS (mean reticulocyte values 16.8%), the authors observed that mean corpuscular hemoglobin (MCH) was 29.8 +/- 2.4 mu microgram and mean corpuscular hemoglobin (MCV) was 88.1 +/- 6.8 cu micrometers. In contrast, patients in a comparable hemolytic-disease group unrelated to hemoglobinopathies (mean reticulocyte count = 15.7%) had a higher MCH (33.0 +/- 1.8 mu microgram) and larger MCV (97 +/- 5.3 cu micrometers). These data indicate that Hb SS disease is associated with "relative microcytosis," presumably a consequence of reduced hemoglobin production.     

Group A beta-hemolytic streptococcal bacteremia in a patient with sickle cell anemia on penicillin prophylaxis.

Serious invasive bacterial infections, particularly those due to Streptococcus pneumoniae and Hemophilus influenzae, are a well-known complication in patients with sickle cell disease. Early penicillin prophylaxis has been shown to prevent these infections and also to improve survival. This article describes a child with sickle cell anemia who, while on penicillin prophylaxis, developed a group A streptococcal bacteremia, a pathogen not commonly associated with bacteremia in sickle cell disease.     

A case report: unusual Gerbich antibody in a patient with sickle cell anemia

A patient whose red blood cells (RBCs) typed as Ge:2,3 produced an alloantibody to a high-frequency antigen in the Gerbich system. This antibody was shown to be nonreactive with Ge:-2,-3 KBCs using adsorption-elution studies. A monocyte monolayer assay (MMA) suggested that transfusion of Ge:2,3 KBCs to this patient would have reduced in vivo survival.     

Fatal delayed transfusion reaction in a sickle cell anemia patient with Serratia marcescens sepsis

Patients with sickle cell anemia may require repeated red cell transfusion, putting them at risk for minor blood group alloimmunization and the development of delayed hemolytic transfusion reactions. Although Streptococcus pneumoniae is the most common cause of life-threatening infection in patients with sickle cell anemia, those who have been recently hospitalized are at risk for infection with resistant hospital-associated organisms, and blood transfusion may put the patient at risk of infection with transfusion-associated organisms such as Serratia marcescens and Yersinic enterocolitica. We recently cared for an adolescent with sickle cell anemia who presented to the emergency department with a severe, delayed hemolytic transfusion reaction and Serratia marcescens infection. The patient had been discharged from the hospital five days previously, and had been transfused and treated with antibiotics while hospitalized. In addition to demonstrating the potential severity of delayed hemolytic transfusion reactions, our case illustrates the importance of providing relatively broad-spectrum antibiotic coverage to patients with sickle cell anemia and possible infection who have recently been hospitalized.     

Cerebrovascular accident during a delayed hemolytic transfusion reaction in a patient with sickle cell anemia

A 28-year-old woman with sickle cell anemia suffered a left hemispheric cerebrovascular accident associated with severe right-sided weakness during a delayed hemolytic transfusion reaction owing to anti-rh' (C) and anti-S. The anti-rh' (C) had been identified four years earlier at a different hospital but neither the patient, her family, nor any member of the staff of the hospital where she was transfused was aware of this information. It is postulated that spherocytes, formed during hemolysis, could slow capillary flow, thereby increasing red cell sickling and producing vaso-occlusion. The patient had no clinically apparent neurologic complications during the preceding 24 years and has had no further neurologic events during the subsequent 20 months. This patient's reaction underscores the compelling need for sensitive pre-transfusion tests as well as the obligation to inform patients and their families of the presence and potential consequences of alloantibodies in the event of future transfusion.     

Physical performance decrements in children with sickle cell anemia.

Prior studies have suggested that cardiorespiratory dysfunction might contribute to the inability of children with sickle cell anemia to exercise competitively with normal children. This article presents a study designed to detect differences in performance of routine physical activities between groups of children having homozygous hemoglobin of sickle cell anemia (HbSS) and those with normal hemoglobin (HbAA). Thirty 10-year-old girls were divided into two equal groups exhibiting no significant differences in height, weight, or body surface area. Each subject performed 20-yd swimming, 40-yd swimming, and 100-yd "potato" foot-racing activities. Results showed significant performance decrements in HbSS compared with HbAA children. Performance decrements on the 20-yd swimming were found to be significantly greater than in either the 40-yd swimming or the 100-yd "potato" races. Assessment of 20-yd swim time as a fraction of 40-yd swim time showed diminished capacity of HbSS children for "burst activity." It is concluded that distance might play a role in the capacity of HbSS children to compete with HbAA children in racing activities such as those encountered in school-based physical education programs. Parents and educators should consider that short distance racing might exaggerate the inability of children with sickle cell anemia to compete with normal children.     

Transfusion in sickle cell anemia

Although blood transfusion (BT) therapy remains a key component of the weaponry used to treat acute and chronic sickle cell disease complications, its indications and modalities are currently the focus of a critical reappraisal prompted by the introduction of hydroxyurea, recent improvements in allogeneic bone marrow transplantation, and increasing attention to safety concerns. Expected benefits of each BT should be carefully weighed against the risks of infections, immunologic complications, and iron overload. Simple or exchange BT can be used. In emergency situations, the only effective means of improving tissue oxygenation and limiting blood vessel occlusion is dilution or removal of HbS by simple or exchange BT, respectively; simple BT is indicated in severe anemia or acute hypovolemia and exchange BT in acute vasoocclusive crisis or acute infection. In nonemergency situations, long-term exchange BT programs geared to maintain the HbS level around 30% are used to stabilize existing lesions and to prevent recurrences; they have been proved effective in preventing recurrent stroke in patients who are not candidates for allogeneic bone marrow transplantation. Situations in which BT therapy is widely used despite controversy regarding its value and modalities include the prevention of complications of pregnancy, the prevention of perioperative complications, and the prevention of recurrences of severe vaso occlusive crisis in patients eligible for hydroxyurea therapy. Advances have been made in the minimization of BT-related complications (alloimmunization, viral infections, iron overload) through critical appraisal of the need for each BT, careful selection of the most appropriate blood product, and a change in BT technique resulting in a reduction in the number of blood donors.     

Hand-foot roentgen findings in sickle cell anemia.

X-ray findings in the hands and feet of 420 sickle cell anemia patients were studied during the six-year period, 1972-1978. A correlation between roentgen findings and clinical presentations is emphasized.     

Management of adults with sickle cell anemia

The management of adults with sickle cell disease should be geared to the profile of the disease in adulthood. The chronic hemolytic anemia impacts everyday activities. Paroxysmal complications include painful vasoocclusive crises, acute chest syndrome, priapism, and infections. Potentially life-threatening chronic complications should be detected and treated early; they include cardiopulmonary, renal, and hepatic involvement. Osteonecrosis of the hip can result in functional impairment. Pregnancy and anesthesia require special precautions. A multifaceted personalized management program, if possible at a sickle cell disease center working closely with other health care providers and social workers, offers the best hope for providing ever-increasing gains in quality of life for sickle cell disease patients.     

Treatment of sickle cell anemia with hydroxyurea and erythropoietin.

BACKGROUND. Hydroxyurea increases the production of fetal hemoglobin (hemoglobin F) in patients with sickle cell anemia and therefore has the potential for alleviating both the hemolytic and vaso-occlusive manifestations of the disease. There is preliminary evidence that recombinant human erythropoietin may also increase hemoglobin F production. METHODS and RESULTS. We treated five patients with sickle cell disease with escalating doses of intravenous erythropoietin for eight weeks. Three of these patients were subsequently treated with daily doses of oral hydroxyurea. After the optimal dose was determined, erythropoietin was then given along with hydroxyurea for four weeks. Treatment with erythropoietin, either alone or in combination with hydroxyurea, had no significant effect on the percentage of hemoglobin F-containing reticulocytes (F reticulocytes) or red cells (F cells). In contrast, hydroxyurea treatment was associated with a 3-to-25-fold increase in F reticulocytes, a 1.6-to-7-fold increase in F cells, and a 2.3-to-16-fold increase in the percentage of hemoglobin F. In all three patients given hydroxyurea, treatment with this drug was associated with reduced hemolysis, shown by decreases in serum bilirubin and lactic dehydrogenase and prolongation of red-cell survival. Hydroxyurea treatment also resulted in a decrease in the percentage of irreversibly sickled cells and sickling at partial oxygen saturation, an increase in oxygen affinity and total red-cell cation content, and a reduction in potassium-chloride cotransport. All three patients had a decrease in the number of pain crises. CONCLUSIONS. This study confirms that hydroxyurea therapy increases hemoglobin F production and provides objective evidence that hydroxyurea reduces the rate of hemolysis and intracellular polymerization of hemoglobin S. In contrast, recombinant human erythropoietin, whether alone or in combination with hydroxyurea, offers no measurable benefit.     

Infection and sickle cell anemia

Sickle cell disease is associated with frequent and often severe infections as a result of immune function impairment and functional asplenia. Also, infection can trigger a vasoocclusive crisis. Pneumococcal bacteremia and meningitis are so severe as to warrant prophylactic penicillin therapy, which has provided a dramatic decrease in early mortality. Bacterial pneumonia is common in patients younger than four years, with most cases being due to S. pneumoniae, Haemophilus influenzae, Mycoplasma pneumoniae, and Chlamydia pneumoniae. Acute chest syndrome is both a difficult differential diagnosis and a common concomitant of bacterial pneumonia. Osteomyelitis is generally due to a salmonella, most often S. enteritidis; multiple foci are common and treatment is difficult, with some patients developing chronic osteomyelitis with sequestration. Parvovirus B 19 infection causes acute bone marrow failure. Malaria does not result in cerebral malaria but can lead to severe anemia or vasoocclusive crisis, and should therefore be effectively prevented. Antimicrobials are generally selected for efficacy against pneumococci (septicemia, meningitis), Salmonella (septicemia, meningitis, osteomyelitis), and mycoplasmas (pneumonia). Prophylactic therapy is of paramount importance and relies on long-term or lifelong penicillin therapy started at four months of age and on closely-spaced immunizations, most notably against pneumococci, the hepatitis B virus, S. typhi, and H. influenzae. Resistant pneumococcal strains have not been reported to cause prophylactic treatment failures. Conjugated pneumococcal vaccines are effective in protecting infants and should therefore be used in sickle cell patients.