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Splenopathy in Patients with Sickle Cell Disease; However, It Provided the Original Work Is Properly Cited Open Access Review Article J Surgery March 2016 Vol:4, Issue:1 © All rights are reserved by Al-Jafar et al. Journal of Splenopathy in Patients with Surgery Hassan A. Al-Jafar1*, Sulaiman Almazeedi2 and Sickle Cell Disease Saleh Bubishate2 1Consultant hematologist, Amiri Hospital, Kuwait City, Kuwait 2Department of Surgery, Adan Hospital, Kuwait Abstract *Address for Correspondence Splenopathy with sickle cell disease refers to disorders that require Hassan A. Al-Jafar, Consultant hematologist, Amiri Hospital, Kuwait medical care and surgical treatment. It is common in patients with City, Kuwait, E-mail: [email protected] sickle cell disease. The genotype of patients with sickle cell disease Submission: 22 November 2015 could affect the characteristics of splenopathy. Patients with sickle cell Accepted: 09 March 2016 anemia genotype mainly experience auto-splenectomy, while those Published: 14 March 2016 with sickle cell thalassemia have splenomegaly. Splenopathy requires careful observations by the hematologists and surgeons, especially Copyright: © 2016 Al-Jafar HA et al. This is an open access article in emergencies as it could prove fatal. Splenic infarction is the main distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, reason for splenopathy in patients with sickle cell disease; however, it provided the original work is properly cited. does not explain the unresolved important clinical issues. Research is still going on to explore more about the relationships between sickle Reviewed & Approved by: Dr. Michael F Daily, Renal and Pancreas cell hemoglobinopathy and splenopathy. Transplantationan, University of Kentucky, USA Introduction genitourinary, musculoskeletal, and many other complications including splenic diseases [10]. New evidence shows that the Splenopathy refers to inflammatory, neoplastic, or traumatic pathogenesis of SCD is more complicated and is not limited to a anomalies of the spleen, and is a major complication of sickle cell simple distortion of the RBC and vascular occlusion. Such factors disease (SCD) [1]. SCD refers to a group of inherited hematological are related to the other hemoglobin variants along with HbS, which disorders that affect different races across the geographical areas reduce polymerization potential and response to oxygen stress; worldwide [2]. It is an autosomal recessive disorder of the beta (β)- moreover, they alter membrane lipids and adhesion molecules [11]. hemoglobin chain. In normal adults, there are two alpha (α) and two The spleen is one of the most commonly affected organs [12]. A wide β globin chains in hemoglobin A; however, in patients with SCD, a spectrum of diseases can affect the spleen secondary to SCD, with new mutation in the β globin chain leads to sickle cell hemoglobin (HbS) evidence surrounding their management, both medical and surgical. [3]. This mutation was first discovered by Linus Pauling in 1949. He This article aims to review the recent literature regarding these proved that this abnormal hemoglobin molecule causes detrimental important complications and their management. pathology [4]. Normally, there are three different types of hemoglobin A, A2, and F. HbS in patients with SCD contains an abnormal β globin Splenic Infarction chain encoded by a substitution in chromosome 11 genes that result The repeated vaso-occlusive attacks in SCA can result in splenic in valine instead of glutamic acid. α-Thalassemia (Thα) represents an infarction. Usually these infarcts are small and recurrent, resulting autosomal recessive disorder with reduced production or absence of in subsequent auto-splenectomy [13]. In majority of the cases, the α-globin chains that result in anemia. β-thalassemia (Thβ) represents splenic infarcts are mild and self-limited. In a few cases, however, an autosomal recessive disorder with reduced production or absence the infarcts can be severe and can result in acute splenic syndrome. of β-globin chains that also results in anemia. The main genotypes in In such cases, patients experience a triad of severe abdominal pain, patients with SCD are (1) homozygous HbSS sickle cell anemia (SCA) splenomegaly, and left upper quadrant tenderness. Additionally, the and (2) sickle cell thalassemia (SCTh) [5,6]. Further, SCTh includes patients can exhibit signs of peritonitis, guarding, abdominal rigidity either SC-Thα or SC-Thβ. as well as left pleural effusion and atelectasis. Most of these patients The genotype of SCD determines the size of the spleen [7]. can be managed conservatively with proper hydration, pain control, In childhood, a patient with SCA has splenomegaly; however, in oxygenation, and rest. However, splenic rupture may occur in severe adulthood the patient has spleen atrophy [8]. In SCTh, the spleen acute splenic syndrome necessitating surgical splenectomy [14]. progressively increases in size, which could require splenectomy in Hyposplenism and Functional Asplenia adulthood. There are many theories about how the deformed red blood cells (RBC) containing HbS cause the clinical manifestations of Hyposplenism and functional asplenia are well-recognized SCD. Traditionally, it has been considered that the distortion of the complications of SCA and are characterized by impaired and loss diseased RBC causes sickling crises. This distortion is precipitated by of the reticuloendothelial function of the spleen, respectively [15]. states of low oxygen tension including dehydration, surgery, trauma, This dysfunction is considered to be a consequence of splenic physiological or pathological stresses and temperature extremes. fibrosis caused by repeated vascular occlusion and infarction [16]. Because of RBC deformity, blood viscosity increases leading to Hyposplenism can also result secondary to the surgical removal of disruption of blood flow, consequent vascular obstruction, and the spleen following therapeutic splenic immobilization and as a even necrosis of the end organs [9]. Such affected organs can cause complication of certain medical conditions [17]. The diagnosis of numerous systemic complications including body pain syndrome, hyposplenism does not always correlate with a decrease in the spleen neurological, pulmonary, ophthalmological, hepatobiliary, renal, size per se; rather it has been shown to be related to decrease splenic Citation: Al-Jafar HA, Almazeedi S, Bubishate S. Splenopathy in Patients with Sickle Cell Disease. J Surgery. 2016;4(1): 4. Citation: Al-Jafar HA, Almazeedi S, Bubishate S. Splenopathy in Patients with Sickle Cell Disease. J Surgery. 2016;4(1): 4. ISSN: 2332-4139 vascularization. This condition can be diagnosed by assessing the Splenic Abscess filtering function of the spleen, as measured by the levels of the Howel- Jolly bodies in RBCs as well as by nuclear imaging [18]. Patients Splenic abscess is a very rare condition that affects patients with with hyposplenism and/or functional asplenia are at an increased SCD and is associated with a high mortality rate [33]. About only 600 risk of infection due to “impaired immunity [17]. These patients cases have been reported in the literature. These have occurred mostly are particularly susceptible to infection by encapsulated bacteria, in the tropics. The causes of splenic abscess include a direct extension especially Streptococcus pneumonia, Neisseria meningitides, and from the infected contiguous organ, hematogenic spread from another Haemophilus influenzae. In addition, other factors have been found site of the body (most commonly bacterial endocarditis), infection that predispose a patient to this overwhelming infection. These secondary to spleen trauma, and in the immuno-compromised include dysfunction of immunoglobulin IgG and IgM response, [34]. Patients usually have non-specific symptoms that can make defects in the alternative pathway of the complement system, and the diagnosis very difficult. Frequently the symptoms and signs dysfunction of phagocytic opsonization [19,20]. The prevention of include fever, abdominal pain, and upper left quadrant tenderness, such infections is best achieved by proper immunization, antibiotic splenomegaly, leukocytosis, and left lower chest clinical problems. prophylaxis, and optimal patient education [21]. Diagnosis can be made by performing either computed tomography or abdominal ultrasonography. There is currently no consensus Acute Splenic Sequestration regarding the cutoff for the percutaneous drainage of the abscess Acute splenic sequestration is a life-threatening event and is versus splenectomy. As a general rule, unilocular abscesses < 3 cm in a leading cause of deaths in children with SCD [22]. In patients, size with thin liquid content can be safety drained under radiological who are homozygous for HbSS, the incidence of acute splenic guidance [33]. When abscess size is >10 cm or non-surgical treatment sequestration is reported to be between 7% and 30%. It can occur as has failed, splenectomy has been shown to be a safe alternative to treat early as 8 weeks of age with 76% of the cases as less than 2 years of the condition [35]. age [23,24]. The pathophysiology remains unclear. However, it has Surgical Splenectomy Indications in SCD been suggested that the diversion of the blood flow through the intra- splenic shunt causes splenomegaly, RBC retention, and capillary Certain clinical situations warrant surgical splenectomy in engorgement. Patients usually experience signs of hypovolemic shock, SCD. First, patients with acute splenic
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