Case report on sickle cell anemia: a cascade of crisis

Children with sickle cell homozygous state often experience complications like an acute painful crisis, dactylitis, severe anemia, sequestration, acute febrile illness, and stroke because of its complicated pathophysiology. This is one of the main reasons for a child抯 frequent hospital visits or admissions, which is a significant cause of its morbidity. This case discussed a 7-year-old male child who presented to the hospital in vaso-occlusive crisis in the form of pain in the right elbow joint, followed by a rare occurrence of deteriorating multiple morbid complications of sickle cell disease in the same patient within a short span of his hospital stay.

Shoulder joint septic arthritis with scapular osteomyelitis a rare presentation in an infant with sickle cell disease: a challenging diagnosis; a case report from rural central India

Sickle-cell disease (SCD) a hereditary autosomal recessive disorder is the most common haemoglobinopathy worldwide. In India, it is the second most common haemoglobinopathy next to Thalassemia, prevalent in the tribal population of Central and Southern parts of India. The pathophysiology of the disease is point mutation in the beta globin chain leading to sickling of RBCs which causes obstruction in microvasculature leading to acute events like Vaso-occlusive crisis. Patients with SCD are also at an increase of orthopaedic manifestation like osteomyelitis, septic arthritis or osteonecrosis. We report a case of a 10 months old male child who presented with anaemia and fever. Child was diagnosed with SCD, later developed swelling of right shoulder joint and restrictions of movements. Diagnosis of septic arthritis of shoulder with scapular osteomyelitis was made with the help of radiological and laboratory investigations. Child was managed with injectable antibiotics and symptomatic treatment. Patients with SCD as a result of occlusion of microvasculature along with immunocompromised state are at higher risk of bacterial infections. Osteomyelitis is one of the dreaded complications. Its clinical presentation is similar to that of VOC hence there occurs a dilemma in diagnosis. A multidisciplinary approach including high degree of clinical suspicion, laboratory investigation and radiological imagining can help in early diagnosis and management.

A cross-sectional study on pain management during vaso-occlusive crisis in sickle cell disease

Background: Sickle cell disease is an inherited hematological disorder characterized by hard and sticky red blood cells that appears like a C-shaped “sickle” in contrast to the healthy, round red blood cells which tend to stick and clog the blood vessels during blood flow leading to pain or vaso-occlusive crisis. Methods: A cross-sectional study was conducted at Yashoda hospital in Hyderabad, India. Data regarding the management of acute pain during VOC in SCD patients was gathered from the hematology department on a daily basis during the duration of the study. The data was recorded in an online questionnaire form and a subsequent DUE was conducted. Results: Among all the patients that were enrolled in the study, acute pain as VOC was presented in 72%, whereas 28% did not experience pain. Pain was manifested as acute chest syndrome, generalized body pain, headache. Crizanlizumab, a monoclonal antibody was administered to patients who were ?16 years to decrease the frequency of VOC. Upon DUE, per prescription distribution of crizanlizumab was 11.2%, while 88.8% constituted of anti-metabolites, antibiotics, analgesics, opiate antagonist, etc. Frequency of use of crizanlizumab among SCD patients was 69.23% Conclusions: Management of VOC cannot be described as one size fits all. Interindividual variability must be considered at all times during planning and implementing a treatment regimen. Assessment of pain on pain scale to ensure targeted treatment at maximum effective concentration followed by titration of dose to reduce the occurrence of tolerance, withdrawal, dependence and addiction should be basis of management.

A systematic review on hydroxyurea therapy for sickle cell disease in India

Background & objectives: Sickle cell disease (SCD) constitutes frequently inherited haemoglobin disorders and poses a significant health burden in India. Hydroxyurea (HU), the most commonly used drug, has shown promising results in the clinical management of SCD. The present systematic review was undertaken to assess the efficacy and toxicity of HU in Indian sickle cell patients. Methods: A systematic review of studies on HU therapy was conducted to identify the application of HU and its outcome(s) across India. PubMed, Scopus and Cochrane Library was used as data sources for various studies on the efficacy and toxicity of HU therapy for treatment for SCD in India published between January 2001 and October 2021. Two authors independently extracted the data on study design, patient characteristics and therapeutic outcomes of HU in order to determine the study quality of the present review. Results: Overall, 14 studies were included for a systematic analysis. Of these 11 were prospective, two cross-sectional and one double-blind randomized controlled trial. Low-dose HU (10 mg/kg/day) was found to reduce the rates of vaso-occlusive crisis and hospitalization as well as decreased the requirement of blood transfusion in SCD patients. The foetal haemoglobin (HbF) level was recorded in 13 (80%) studies all of whom reported an elevation in the HbF levels, with a mean increase in per cent HbF from 15.8 to 21.4 per cent across studies. The common adverse events were reversible, mild-to-moderate cytopenia and anaemia. Interpretation & conclusions: The findings of the present review suggest that there is still insufficient information presently to determine the long-term or major adverse effects on organ damage, fertility as well as pregnancy on the use of HU therapy for SCD. Long-term multi-centric studies are thus required to address these problems.

Mortality in children, adolescents and adults with sickle cell anemia in Rio de Janeiro, Brazil

Abstract Objective: To determine the mortality rate of children, adolescents and adults with sickle cell anemia in Rio de Janeiro, Brazil. Methods: The number of deaths, the mortality rate and the causes of deaths in patients with sickle cell anemia who were treated and followed up at our institution for 15 years were determined and compared to data available for the Brazilian population. Results: The overall number of deaths was 281 patients with a mortality rate of 16.77%. Survival probability was significantly higher in females. The number of deaths and the mortality rate were age-specific with a significant increase in the 19- to 29-year-old age group. The remaining life expectancy of the patients with sickle cell anemia was less than that of Brazilians at large. The gap between the two was about 20 years for ages between one and five years with this gap decreasing to ten years after the age of 65 years. The most common causes of death were infection, acute chest syndrome, overt stroke, organ damage and sudden death during painful crises. Conclusion: To the best of our knowledge, this is the first Brazilian study in a single institution in Rio de Janeiro; the mortality rate was 18.87% among adult patients with sickle cell anemia. The mortality rates in children and adults are higher than those reported in developed countries of the northern hemisphere.

Correlation of serum C-reactive protein in painful vaso-occlusive crisis in sickle cell disease and its response to analgesic therapy

Background: Sickle cell disease is characterized by chronic hemolysis, frequent infections and recurrent occlusions of microcirculations, which causes painful crisis and results in chronic organ damage and failure. Occlusion of microcirculation and infection are important factors that stimulates production of cytokines and acute phase proteins like C- reactive protein. Aim: To estimate serum C-reactive protein levels in cases of painful vaso-occlusive crisis of sickle cell disease, to study correlation of level of serum C-reactive protein with duration of painful vasoocclusive crisis of sickle cell disease, to study relation between level of serum C-reactive protein and treatment response of analgesic versus analgesic opioid in cases of painful vaso-occlusive crisis of sickle cell disease. Materials and methods: This hospital based observational case control study was carried out at IGGMC, Nagpur, Maharashtra, India. Total 31 cases of sickle cell vaso-occlusive crisis, of age 12 years and above, admitted in hospital and 31 controls included in study. Study protocol was followed in each case. Results: Musculoskeletal, joint pain (83.87%) was most common form of presentation. Exhaustion and severe physical activity (25.80%) was most common precipitating factor. Mean hemoglobin, bilirubin showed statistically significant difference in cases compared to control. Mean CRP on day 1 (40.87±17.22 mg/L) was significantly high compared to control (3.67±0.77 mg/L) with statistical significance p<0.0001.

Análisis de pacientes drepanocíticos tratados con hidroxiurea en el Hospital Nacional de Niños / Evaluation of sickle cell patients treated with hidroxyurea at the National Children's Hospital

Antecedentes: desde 1984 estudios comprobaron que la hidroxiurea aumentaba los niveles de hemoglobina fetal y del volumen corpuscular medio, por esta razón a partir de 1998 fue el primer fármaco aprobado para el tratamiento en la drepanocitosis. El objetivo de este estudio fue describir la respuesta y complicaciones de los pacientes drepanocíticos en tratamiento con hidroxiurea en el Hospital Nacional de Niños. Métodos: estudio transversal y retrospectivo, realizado en pacientes con drenopacitosis atendidos en el servicio de Hematología del Hospital Nacional de Niños de enero 2006 a diciembre 2009; se incluyeron pacientes cuyo expediente clínico reunía los criterios de inclusión...

La drepanocitosis en Cuba. Estudio en niños / Drepanocytosis in Cuba: Study in children

La drepanocitosis es la anemia hemolítica determinada genéticamente más frecuente en el mundo. En Cuba, la frecuencia del estado de portador es del 3,08 por ciento en la población general. La fisiopatología de la oclusión vascular es muy compleja; involucra la polimerización de la Hb S, las alteraciones de la membrana del hematíe, las moléculas de adhesión, las citocinas inflamatorias, los factores de la coagulación y lesiones del endotelio vascular. Las manifestaciones clínicas más frecuentes son: las crisis vasooclusivas dolorosas, el síndrome torácico agudo, la crisis de secuestro esplénico, la crisis aplástica, la necrosis aséptica de la cabeza del fémur y la úlcera maleolar. El cuadro clínico es muy variable: desde niños que mueren temprano en la vida hasta pacientes que alcanzan la sexta década de la vida. En el Instituto de Hematología e Inmunología existe un Programa de Atención Integral que incluye: seguimiento sistemático desde temprano en la vida en una consulta especializada, la administración de ácido fólico de forma permanente y de penicilina oral profiláctica los primeros 5 años de la vida; así como la educación del niño y de sus padres. Desde 1986 se realiza esplenectomía parcial en la crisis de secuestro esplénico con excelentes resultados. Entre 2004-2008 fallecieron solamente 16 enfermos en todo el país y en 397 adultos la sobrevida fue de 53 años en la anemia drepanocítica y de 58 en la hemoglobinopatía SC. Como resultado de este programa, en los últimos años la sobrevida ha aumentado, la calidad de vida del paciente ha mejorado y han disminuido los costos invertidos en el tratamiento de las complicaciones

Drepanocytosis is the hemolytic anemia more frequent genetically determined in the world. In Cuba, the frequency of carrier status is of 3,08 percent in general population. The pathophysiology of vascular occlusion is very complex; includes the polymerization of the Hb S, the alterations of red-blood cells, the adhesion molecules, the inflammatory cytokines, the coagulation factors and the lesions of the vascular endothelium. The more frequent clinical manifestations are: painful vaso-occlusive crises, the acute thoracic syndrome, the splenic sequestration crisis, the aplastic crisis, the aseptic necrosis of femur head and malleolar ulcer. The clinical picture is very variable: from children dying early in life up to patients achieve the sixth decade of life. In the Institute of Hematology and Immunology there is an Integral Care Program including: systematic follow-up from early in life in a specialized consultation, permanent administration of folic acid and of prophylactic oral penicillin during the first 5 years of age; as well as the child education and of parents. From 1986 it is carried out the partial splenectomy in crises of splenic sequestration with excellent results. Between 2004-2008 in all the country deceased only 16 patients and in 397 adults the survival rate was of 53 years in the drepanocythemia and of 58 in the SC hemoglobinopathy. As result of this program, in past years the survival has increased, the quality of life of patient improved and the costs spent in treatment of complications has decrease