|
 
 |
| EDITORIAL |
|
| Year : 2022 | Volume
: 12
| Issue : 1 | Page : 1-2 |
|
Sickle cell anemia and its complications
Anupam Dutta
Department of Medicine, Assam Medical College and Hospital, Dibrugarh, Assam, India
| Date of Submission | 16-Mar-2022 |
| Date of Acceptance | 17-Mar-2022 |
| Date of Web Publication | 18-Apr-2022 |
Correspondence Address:
Anupam Dutta
Assistance Editor, Department of Medicine, Assam Medical College and Hospital, Revti House, Purnananda Road, Shantipara, Dibrugarh 786001, Assam
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/2278-8239.343425
How to cite this article:
Dutta A. Sickle cell anemia and its complications. Assam J Intern Med 2022;12:1-2 |
| Introduction | |  |
Anemia may be defined as a reduction below the normal values of the total red cell mass. Anemia has been defined by the World Health Organization as a reduction of hematocrit value below the normal limits or anemia is considered to exist in adults whose hemoglobin levels are lower than 13 g/dL (males) or 12 g/dL (females).[1]
Sickle cell disease (SCD) is a major world health problem. It is estimated to affect at least 20% of the world population and approximately 0%–18% in northeastern India. In SCD, the RBCs contain an abnormal hemoglobin called sickle hemoglobin, which causes these cells to change to a stiff, sticky, curved shape like sickle or crescent moon. Sickle cell dies prematurely causing anemia. They also pile up, and form plugs in blood vessels causing micro- and macrovascular complications.
SCD causes an activated adhesive endothelium. These vasculopathy abnormalities are attributable to ischemia-reperfusion injury, hemostatic activation, microvascular occlusion, and formation of a procoagulant milieu. Vasculopathy of SCD has been implicated in the development of pulmonary hypertension, stroke, leg ulceration, and priapism, particularly associated with hemolytic severity and reported also in other severe hemolytic disorders. This vasculopathy might also play a role in other chronic organ dysfunction in patients with SCD.
| Pathophysiology | | |
Hemoglobin is a molecule that is responsible for carrying oxygen to all the tissues of the body from the lungs. Normal human hemoglobin is a tetramer formed by two pairs of globin chain attached to heme. There are four types of globin chain (α, β, δ, and γ) chains. Normal adult hemoglobin consists of two α and two β chains (α2β2). Hemoglobin A2(α2δ2) is a minor component which is less than 3.3% and less than 1% is HbF (α2γ2)
SCD occurs when there is a point mutation in the sixth position of globin chain of hemoglobin where glutamic acid is replaced by valine from the N terminal end of the chain. Whenever there is hypoxia and stress the β-globin chain promotes the non-covalent polymerization of hemoglobin, which distorts red blood cells into a sickle shape and decreases their elasticity. These cells fail to return to normal shape when normal oxygen tension is restored. As a consequence, these sickle cells are unable to deform as they pass through narrow capillaries, leading to vessel occlusion and ischemia.
| Complications in Sickle Cell Disease | | |
Pulmonary complications
Acute chest syndrome
Acute chest syndrome usually refers to acute lung injury syndrome. Usually, patients present with chest pain, tachycardia, tachypnea, cough fever, and hypoxemia.[2],[3]
Radiological finding shows consolidation of Alveoli. Prevalence is approximately 29%.
Restrictive lung disease
Restrictive lung disease in SCD patients is a complication of acute chest syndrome. Radiological analysis shows area of fibrosis at the basal areas of the lungs.
Diagnosis can be confirmed by spirometry where FEV1/FVC is normal or mildly raised. The development of advanced chronic lung disease in patients with SCD has been called sickle cell chronic lung disease.[4]
Asthma
The prevalence of asthma/reactive airway disease appears to be the same in SCD patients within pediatric age group (though in adult the percentage is less).[5]
Studies have suggested the association between ACS and asthma in SCD patients as in asthma due to ventilation-perfusion mismatched there will be increased chances of sickling of RBCs.
Pulmonary hypertension
PH is an increasingly recognized common complication of SCD and other hereditary or acquired hemolytic anemias.[6] The associated mortality rate is very high. Each increase of 10 mm Hg in mPAP is associated with a 1.7-fold increase in the rate of death of SCD patient. Diagnosis of PH is confirmed by using Doppler echocardiography. An elevated pulmonary artery systolic pressure calculated from the TRV of greater than 2.5 m/s was prospectively defined as abnormal.
Other complications
Anemia
Anemia present as pallor, easy fatigability, irritability, loss of appetite, and poor growth.
Infection
Young patients with SCD can present with extremely life-threatening infection like pneumonia, sepsis, meningitis, and osteomyelitis.
Body organ damage and failure
All organs are vulnerable, including the kidneys, liver, spleen, heart, and eyes, for tissue damage due to deprivation of blood and its nutrients and of oxygen due to repetitive plugging of blood vessels by sickle cells.
Stroke
Stroke is one of the most catastrophic and frequent medical problem encountered in SCD.
Hand foot syndrome
Commonly encountered in patients of pediatric age group.
Cholecystitis
Bilirubin, which is a breakdown product of RBCs, causes gallstones which on impaction of gallbladder duct causes cholecystitis and if untreated may lead to life-threatening condition like sepsis. The presenting symptoms are similar to jaundice.
Leg ulcer
More commonly seen in males aged between 10 and 50. Typically, they are open elevated sores with surrounding skin redness and thinning, located within patches of hyperpigmentation. Most commonly seen on ankle and can be single or multiple.
Avascular necrosis
It commonly involves the hip joint and patients present with painful limbs. Other vulnerable joints are the shoulders.
Renal and bladder involvement
Kidney and bladder infections are quite common in SCD. Patients present with painful and frequent micturation, incontinence, fever, lower abdominal pain, and backache. Due to repetitive clogging of the blood vessels of the kidneys renal failure can be developed.
Priapism and impotence
Men with sickle cell anemia may present with priapism. Overtime, priapism can damage the penis and lead to impotence.
Eye involvement
Eye involvement is the sequlee of microvascular complication that usually affects the retina but rarely causes blindness.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Allen JB, Allen FB The minimum acceptable level of hemoglobin. Int Anesthesiol Clin 1982;20:1-22.  |
| 2. | Gladwin MT, Vichinsky E Pulmonary complications of sickle cell disease. N Engl J Med 2008;359:2254-65. |
| 3. | Platt OS The acute chest syndrome of sickle cell disease. N Engl J Med 2000;342:1904-7. |
| 4. | Powars D, Weidman JA, Odom-Maryon T, Niland JC, Johnson C Sickle cell chronic lung disease: Prior morbidity and the risk of pulmonary failure. Medicine (Baltimore) 1988;67:66-76. |
| 5. | Anthi A, Machado RF, Jison ML, Taveira-Dasilva AM, Rubin LJ, Hunter L Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension. Am J RespirCrit Care Med 2007;175:1272-9. |
| 6. | Jison ML, Gladwin MT Hemolytic anemia-associated pulmonary hypertension of sickle cell disease and the nitric oxide/arginine pathway. Am J RespirCrit Care Med 2003;168:3-4. |
|
Search Pubmed for