Reticuloendothelial Function Research Articles

Vesiculated erythrocytes as a determination of splenic reticuloendothelial function in pediatric patients with Hodgkin's disease.

To determine the effect of low-dose total, or subtotal, nodal irradiation (TNI/sub-TNI) on splenic reticuloendothelial function in pediatric patients with Hodgkin's Disease (HD). Pediatric and adolescent patients with advanced stage HD were accrued from two Pediatric Oncology Group studies and subdivided into three groups: the first had chemotherapy (CT) only; the second received chemotherapy and low-dose (2,100 cGy) TNI or subtotal TNI (sub-TNI); the third underwent staging laparotomy with splenectomy followed by CT, with or without low-dose radiotherapy. Vesiculated erythrocyte counts (VRBC) were performed on all patients using Nomarski interference phase optics at the conclusion of therapy. The mean VRBCs were 3.2%, and 3.8% for the non-splenectomized patients who received chemotherapy only, and chemotherapy plus low-dose splenic irradiation, respectively. For those who underwent splenectomy before chemotherapy, the VRBC was 36.7%. Statistical analysis revealed no difference in vesiculated erythrocyte percentages between the CT only group and the CT + TNI/sub-TNI patients; however, there was a significant difference between both of these groups and the splenectomized patients. Our results indicate that the addition of low-dose splenic irradiation to chemotherapy in children and adolescents with advanced-stage Hodgkin's disease does not adversely affect splenic reticuloendothial function.

P-259 Estimation of the reticulo-endothelial function of the liver and spleen by positron emission computed tomography (PET)

P-259 Estimation of the reticulo-endothelial function of the liver and spleen by positron emission computed tomography (PET)

Changes in distribution of reticuloendothelial function in transcatheter arterial embolization (TAE) for hepatocellular carcinoma (HCC), estimated by single photon emission computed tomography (SPECT)

Changes in distribution of reticuloendothelial function in transcatheter arterial embolization (TAE) for hepatocellular carcinoma (HCC), estimated by single photon emission computed tomography (SPECT)

An Intrapleural Lung Prosthesis: Rationale, Design, and Testing

Extracorporeal life support (ECLS or ECMO) is standard treatment for severe respiratory failure but poses many contraindications to future lung transplantation. The solution to this dilemma is the implantable gas exchange device (IGED) or artificial lung. Preliminary efforts to create such an artificial lung have been made since 1970 and include designs involving single devices, intravascular devices (i.e., IVOX), and combination heart-lung devices. Stringent requirements govern the design of such a device, the most important of which are high gas exchange efficiency, low resistance to blood flow, and size. This paper describes such a device. It incorporates large diameter inflow and outflow ports in close proximity and a low resistance wound hollow fiber core encapsulated in a compliant outer shell which conserves the work of the right ventricle. In a large animal model (adult sheep) this device was connected in line with the main pulmonary artery in series with the native lungs. This configuration has the advantages of using the lungs as an embolic filter, perfusing the lungs with fully oxygenated blood, and maintaining the integrity of the anatomy necessary for transplant. Laboratory experiments have run > 8 h. Preliminary data show that the animals have remained hemodynamically stable while the devices have supported the animals completely by supplying 100% O2 saturation with PO2 values ranging from 250-350 mm Hg. Additionally, this model makes possible the study of respiratory failure without introducing other variables such as extracorporeal circuits or pumps. The other metabolic, endocrine, and reticuloendothelial functions of normal and injured lungs can now be studied more precisely by excluding these variables.(ABSTRACT TRUNCATED AT 250 WORDS)

Ischemia/reperfusion, inflammatory signal transduction and apoptosis: the physiologic signalling role of reactive oxygen metabolites

Reactive oxygen metabolites generated initially from xanthine oxidase activated from constituitively-expressed xanthine dehydrogenese by ischemia, generate a substantial component of post-ischemic injury at reperfusion in many organs. The key to these events is the activation of endothelial cell xanthine oxidase by ischemia, causing the oxidantmediated upregulation of endothelial integrins, with the consequent arrest and activation of circulating neutrophils in the venular microvasculature, resulting ultimately in parenchymal organ dysfunction. Because there is no evident natural selective advantage to reperfusion injury (and, indeed, no reperfusion in a natural environment), an obvious question is: What survival advantage provided the natural selective pressure for the evolution of this intricate biochemical process? Evidence from a number of laboratories indicates that endothelial xanthine oxidase can be activated by specific proinflammatory cytokines (TNF, IL-3, C'SA, etc.) and other mediators (endotexin, phorbol esters, oxidants, etc.). These and other data from our laboratory suggest that endothelial dehydrogensse to oxidese activation may well represent the central molecular trigger of reticuloendothelial function, transducing circulating inflammatory signals (cytokines) by means of the generation of reactive oxidants as second messengers to effect the trapping, phagocytosis and killing of circulating microorganisms. The marked induction of xanthine dehydrogenase expression in endothelial cells by yinterferon may represent the priming of this trigger mechanism. Recent evidence suggests that one consequence of this aberrant signal transduction is the activation of specific programs for heat shock gene expression in endothelial cells. This activation preempts constituitive programs of gene expression, resulting in cell death, not by necrosis, but by apoptosis, perhaps due to the preemption of the expression of a constituitive antioxidant like bcl-2, with resultant nucleolysis by ambient endogenous, or exogenous oxidant generation. Reperfusion injury may well represent the aberrant triggering of this antibacterial defense mechanism by ischemia, and apoptosis its pathophysiologic extreme. If this hypothesis proves correct, it provides another of the accumulating examples (eg:NO,C0) by which reactive oxidants function as second messengers to effect physiological signal transduction.

P158 Reticuloendothelial system function and activity of circulting neutrophils after severe trauma

P158 Reticuloendothelial system function and activity of circulting neutrophils after severe trauma

Obstructive Jaundice Impairs Reticuloendothelial Function and Promotes Bacterial Translocation in the Rat

Septic complications and renal insufficiency following biliary tract surgery are frequently seen in patients with obstructive jaundice. The precise mechanisms for understanding the susceptibility of the jaundiced patients to sepsis are, however, not clear. The present study aimed at investigating the influence of biliary obstruction on the reticuloendothelial function and bacterial translocation at various time intervals in the rat. Reticuloendothelial system (RES) function, as evaluated by measuring blood clearance of intravenously injected 125I-labeled Escherichia coli, and bacterial translocation were studied 3 days and 1, 2, and 3 weeks following either sham operation or common bile duct ligation (CBDL) and transection in the rat. RES function was significantly impaired and renal uptake of radiolabeled E. coli was significantly higher in jaundiced animals from Day 3 and on after CBDL (P < 0.01) concomitant with elevation of plasma levels of bilirubin and liver enzymes (P < 0.001) compared with their corresponding controls. The incidence of bacterial translocation 3 days and 1 and 2 weeks after biliary obstruction significantly increased (P < 0.05). We conclude that RES phagocytic function is impaired and the incidence of bacterial translocation is increased in jaundiced rats. These findings might contribute to explain the high susceptibility of postoperative septic complications and renal dysfunction in patients with obstructive jaundice.

Reticuloendothelial Function Following Cardiopulmonary Bypass: Laboratory and Electron Microscopy Findings

Several studies on the effects of cardiopulmonary bypass (CPB) on the reticuloendothelial system (RES) function have been performed previously, but different results have been reported. This study was carried out to determine the effect of CPB on RES function in a canine model simulating clinical CPB. Nine dogs undergoing surgery with CPB were compared with an identical number of dogs subjected to thoracotomy without CPB. A lipid emulsion test was performed in all dogs before and after the surgical procedure to measure RES phagocytic index, i.e., phagocytic function. Kupffer cell iron uptake under conditions of iron loading following CPB was examined histologically, and any ultrastructural changes in the Kupffer cells were observed by electron microscopy. In both groups, the RES phagocytic index showed a significant decline after surgery. However, a comparison of the two groups revealed that there was a significantly greater decrease in the CPB group (P < 0.05). Moreover, histologic evidence of iron uptake in Kupffer cells was strongly suppressed in the CPB group. Electron microscopy of the Kupffer cells showed that the number of phagosomes, especially deformed erythrocytes, increased after CPB. These findings suggest that RES function is significantly impaired following CPB, and an increase in the amount of material, especially deformed erythrocytes, to be processed by the RES is responsible, in part, for the depressed RES phagocytic function. The prevention of hemolysis during CPB may preserve the RES function following CPB.

Autologous Splenic Transplantation for Splenic Trauma

The authors reviewed the experimental evidence, surgical technique, complications, and results of clinical trials evaluating the role of autologous splenic transplantation for splenic trauma. Splenorrhaphy and nonoperative management of splenic injuries have now become routine aspects in the management of splenic trauma. Unfortunately, not all splenic injuries are readily amenable to conventional spleen-conserving approaches. Heterotopic splenic autotransplantation has been advocated for patients with severe grade IV and V injuries that would otherwise mandate splenectomy. For this subset of patients, splenic salvage by autotransplantation would theoretically preserve the critical role the spleen plays in the host's defense against infection. The relevant literature relating to experimental or clinical aspects of splenic autotransplantation was identified and reviewed. Data are presented on the experimental evaluation of autogenous splenic transplantation, methods and complications of autotransplantation, choice of anatomic site and autograft size, and results of clinical trials in humans. The most commonly used technique of autotransplantation in humans involves implanting tissue homogenates or sections of splenic parenchyma into pouches created in the gastrocolic omentum. Most authors have observed evidence of splenic function with normalization of postsplenectomy thrombocytosis, immunoglobulin M levels, and peripheral blood smears. Some degree of immune function of transplanted grafts has been demonstrated with in vivo assays, but the full extent of immunoprotection provided by human splenic autotransplants is currently unknown. Multiple human and animal studies have established that splenic autotransplantation is a relatively safe and easily performed procedure that results in the return of some hematologic and immunologic parameters to baseline levels. Some aspects of reticuloendothelial function are also preserved. Whether this translates into a real reduction in the morbidity or mortality rates from overwhelming bacterial infection is unknown and requires further investigation.

Pharmacological, toxicologic, and microbiological considerations in the choice of initial antibiotic therapy for serious infections in patients with cirrhosis of the liver.

Infection remains a leading cause of death among patients with cirrhosis of the liver. The high level of susceptibility of these patients to septicemic infection is accounted for by decreased reticuloendothelial function and impairment of several components of cell-mediated and humoral immunity. Escherichia coli and Streptococcus pneumoniae are the pathogens most frequently involved and must be covered by any empirical antibiotic regimen administered to seriously ill cirrhotic patients. In addition, antibiotic therapy in this situation must take into account cirrhosis-induced changes in the kinetic and dynamic behavior of antibacterial agents. Given the great toxic potential of aminoglycosides in liver cirrhosis, these agents should be used very cautiously. The third-generation cephalosporins are currently advocated for the treatment of severe infections in cirrhotic patients because of their high level of intrinsic activity against the most commonly encountered pathogens as well as their safe use at high doses for patients with liver insufficiency. The role of the quinolones deserves further clinical evaluation; the limited activity of these agents against S. pneumoniae is undoubtedly a drawback to their use as empirical monotherapy. Despite the potent antibacterial agents that have recently become available, the overall prognosis of these patients is difficult to improve because it remains closely related to the severity of their underlying liver disease.

A study of endotoxaemia in patients with primary biliary cirrhosis.

Hepatic reticuloendothelial function may be diminished in patients with primary biliary cirrhosis. Endotoxin concentrations in peripheral blood and in the superior mesenteric vein were measured, by the beta-glucan sensitive, factor-free, endotoxin-specific limulus assay, in patients with primary biliary cirrhosis and liver cirrhosis (non-PBC cirrhosis). Endotoxaemia was detected in the peripheral blood of seven out of nine patients (78%) with asymptomatic primary biliary cirrhosis, but in only two out of thirteen patients (15%) with non-PBC cirrhosis. The endotoxin level was significantly higher in the earlier stages of primary biliary cirrhosis than in the later stages (P < 0.05). The endotoxin level in superior mesenteric vein blood was significantly lower in patients with primary biliary cirrhosis than in patients with non-PBC cirrhosis. Peripheral endotoxaemia in patients with primary biliary cirrhosis may be due to the diminished capacity of the hepatic reticuloendothelial system, for phagocytosis of endotoxin.

Subchronic 10 Day Immunotoxicity of Polydimethylsiloxane (Silicone) Fluid, Gel and Elastomer and Polyurethane Disks in Female B6C3F1 Mice

Millions of people have been exposed to silicones because of the widespread use in consumer products such as cosmetics and toiletries, food products, household products and paints. Silicones have wide use in medical practice, including lubricants in tubing and syringes, and as implantable devices. The most prevalent silicone in medical use is polydimethylsiloxane. This study was undertaken to determine the subchronic immunotoxicologic potential of the principal constituents of breast implants: silicone fluid, silicone gel and silicone elastomer. An alternative covering for devices containing silicone gels, polyurethane, was also included in the study. Silicone fluid and gel were injected subcutaneously into female B6C3F1 mice (1 ml/mouse) and 6 mm disks of silicone elastomer or polyurethane were implanted subcutaneously. There were no treatment-related deaths or overt signs of toxicity. None of the tested materials had notable effects on body or organ weights, erythrocytes or leukocytes in the blood, blood chemistries such as alanine aminotransferase, urea nitrogen, glucose, albumin or total protein. The cellularity of the bone marrow and responses to CSF-GM and CSF-M were normal. The tested silicones did not alter the distribution of B cells and T cells in the spleen, but polyurethane perturbed the distribution of CD4+CD8+ and CD4-CD8- T cells. The antibody response to sheep erythrocytes was not markedly altered, nor were proliferative responses to concanavalin A, phytohemagglutinin, lipopolysaccharide or allogeneic cells. Reticuloendothelial function was normal, but polyurethane evoked an enhanced phagocytosis of Covaspheres by adherent peritoneal cells. Natural killer cell activity and serum complement were not altered. All silicone materials afforded modest protection to a challenge with Listeria monocytogenes that killed 40 to 58% of control mice. Host resistance to Streptococcus pneumoniae or the B16F10 tumor was not affected by any of the treatments. There is a pattern indicative of some perturbation of T cell differentiation in mice implanted with a polyurethane disk.

Immunotoxicity of 180 Day Exposure to Polydimethylsiloxane (Silicone) Fluid, Gel and Elastomer and Polyurethane Disks in Female B6C3F1 Mice

Millions of people have been exposed to silicones which are present in consumer goods such as cosmetics and toiletries, processed foods and household products. In addition, silicones have been used extensively in medical practice as a lubricant in tubing and syringes, and as implantable devices. A silicone widely used in medical practice is polydimethylsiloxane. This study was undertaken to determine the immunotoxicologic potential of long term exposure to the principal constituents of breast implants: silicone fluid, silicone gel and silicone elastomer. An alternative covering for devices containing silicone gels, polyurethane, was also included in the study. Silicone fluid and gel were injected subcutaneously into female B6C3F1 mice (1 ml/mouse) and 6 mm disks of silicone elastomer or polyurethane were implanted subcutaneously. There were no treatment-related deaths or overt signs of toxicity during the 180 day exposure. None of the tested materials had notable effects on body or organ weights, erythrocytes or leukocytes in the blood, blood chemistries such as alanine aminotransferase, urea nitrogen, glucose, albumin or total protein, or serum CH 50 or C3 levels. The cellularity of the bone marrow and responses to CSF-GM and CSF-M were normal. The tested silicones and polyurethane marginally reduced the level of Ig+ cells in the spleen but did not consistently alter the distribution of T cell surface markers. The antibody response to sheep erythrocytes was not markedly altered, nor were proliferative responses to concanavalin A, phytohemagglutinin, lipopolysaccharide or allogeneic cells. Reticuloendothelial function was normal, as was phagocytosis of chicken erythrocytes and Covaspheres by adherent peritoneal cells. Natural killer cell activity was depressed in all silicone treatment groups and in mice implanted with polyurethane. No silicone or polyurethane treatment group displayed altered susceptibility to a challenge with Listeria monocytogenes, Streptococcus pneumoniae or the B16F10 tumor. The only consistent effect of 180 day exposure to silicone materials or polyurethane was a modest depression of natural killer cell activity.

Effect of isolated portal hypertension on kupffer cell function

The increased incidence of infection in cirrhotics may in part be attributable to dysfunction of the reticuloendothelial system (RES) in removing pathogens from the circulation. The portosystemic shunting (PSS) that results from portal hypertension in cirrhotics may compromise RES function by allowing enteric pathogens to be shunted away from the Kupffer cells. A well-characterized model of portal hypertension induced by partial portal vein ligation (PVL), in which there is no hepatic parenchymal cell damage, was used. Kupffer cell function is unaltered and the effect of PSS alone on overall RES function can be evaluated. In addition to the usual immunologically inert [99mTc]sulfur colloid, an actual pathogen was also evaluated. PVL and sham-ligated rats were given either [99mTc]sulfur colloid or E. coli via the ileocolic vein. The right femurs, lungs, livers and spleens of the animals receiving 99mTc were excised and the radioactivity counted. The lungs, livers, and spleens of the animals receiving E. coli were liquefied and the bacteria were quantified. For both groups the ratios of 99mTc or E. coli in the lung, spleen, and femur to liver were calculated. PVL rats had significantly more 99mTc in the lung, spleen, and femur than the sham rats. There were also significantly more E. coli in the lungs for PVL rats but no significant difference in the spleen counts. These results imply that even in the absence of Kupffer cell dysfunction, PSS alters reticuloendothelial system function by causing a greater distribution of pathogens to the periphery. This altered distribution may contribute to an increased susceptibility to infection in cirrhotics.

Assessment of the hemostatic effectiveness of human platelets treated with aminomethyltrimethyl psoralen and UV A light using a rabbit ear bleeding time technique

The photochemical aminomethyltrimethyl psoralen (AMT), in conjunction with UV A light (UVA), has been shown to inactivate human immunodeficiency virus-1 and model viruses in platelet suspensions under conditions that have only a minimal effect on in vitro platelet properties. A rabbit ear bleeding time technique was used to assess the hemostatic effectiveness of human platelet suspensions treated with AMT/UVA. New Zealand White rabbits were made thrombocytopenic by a combination of irradiation and heterologous antirabbit platelet antiserum. Reticuloendothelial function in these rabbits was suppressed by the intravenous administration of ethyl palmitate. The hemostatic function of 1- and 5-day-old human platelet suspensions (14.5% plasma) that had been treated on day 1 with 40 micrograms/mL AMT and 24 kJ/m2 UVA (1 x UVA) was evaluated by measuring microvascular bleeding times after a standard incision. Comparable bleeding times were observed after infusion with both control and AMT/UVA-treated platelets stored for either 1 or 5 days. With the transfusion of AMT/1 x UVA-treated platelets stored for 5 days, the mean (+/- SD) bleeding time was 156.3 +/- 39.2 seconds (n = 10). With untreated platelets (no AMT/no UVA), stored for 5 days, the mean bleeding time was 189.2 +/- 36.4 seconds (n = 10). Neither AMT nor 1 x UVA treatment alone influenced the observed bleeding times. In contrast, the hemostatic effectiveness of human platelet suspensions was diminished if they were exposed to three times the standard UVA dose (72 kJ/m2) on day 1 and stored for 4 more days, regardless of whether AMT was present, with the mean bleeding time increasing to 442.2 +/- 122.6 seconds (n = 15, AMT present) or 396.0 +/- 45.9 seconds (n = 10, AMT absent). These results are consistent with data obtained from in vitro studies and indicate that virucidal AMT/1 x UVA treatment does not influence platelet hemostatic function. However, the final conditions to achieve these results must be carefully controlled.

Assessment of the Hemostatic Effectiveness of Human Platelets Treated With Aminomethyltrimethyl Psoralen and UV A Light Using a Rabbit Ear Bleeding Time Technique

The photochemical aminomethyltrimethyl psoralen (AMT), in conjunction with UV A light (UVA), has been shown to inactivate human immunodeficiency virus-1 and model viruses in platelet suspensions under conditions that have only a minimal effect on in vitro platelet properties. A rabbit ear bleeding time technique was used to assess the hemostatic effectiveness of human platelet suspensions treated with AMT/UVA. New Zealand White rabbits were made thrombocytopenic by a combination of irradiation and heterologous antirabbit platelet antiserum. Reticuloendothelial function in these rabbits was suppressed by the intravenous administration of ethyl palmitate. The hemostatic function of 1- and 5-day-old human platelet suspensions (14.5% plasma) that had been treated on day 1 with 40 micrograms/mL AMT and 24 kJ/m2 UVA (1 x UVA) was evaluated by measuring microvascular bleeding times after a standard incision. Comparable bleeding times were observed after infusion with both control and AMT/UVA-treated platelets stored for either 1 or 5 days. With the transfusion of AMT/1 x UVA-treated platelets stored for 5 days, the mean (+/- SD) bleeding time was 156.3 +/- 39.2 seconds (n = 10). With untreated platelets (no AMT/no UVA), stored for 5 days, the mean bleeding time was 189.2 +/- 36.4 seconds (n = 10). Neither AMT nor 1 x UVA treatment alone influenced the observed bleeding times. In contrast, the hemostatic effectiveness of human platelet suspensions was diminished if they were exposed to three times the standard UVA dose (72 kJ/m2) on day 1 and stored for 4 more days, regardless of whether AMT was present, with the mean bleeding time increasing to 442.2 +/- 122.6 seconds (n = 15, AMT present) or 396.0 +/- 45.9 seconds (n = 10, AMT absent). These results are consistent with data obtained from in vitro studies and indicate that virucidal AMT/1 x UVA treatment does not influence platelet hemostatic function. However, the final conditions to achieve these results must be carefully controlled.

Initial experiences of chondroitin sulfate iron colloid-enhanced MR imaging in patients with hepatocellular carcinoma: comparative study with CT during arterial portography

Abstract Chondroitin sulfate iron colloid (CSIC), a stable iron colloid preparation, is used for the treatment of iron deficiency anemia in Japan. CSIC administered intravenously accumulates in reticuloendothelial cells and reduces the signal intensity of the liver at magnetic resonance imaging (MRI) because of its T2*-shortening effect. Accordingly, CSIC produces a contrast between normal liver tissue and hepatocellular carcinoma (HCC) which has diminished reticuloendothelial function, enabling improved detection of HCC by MRI. In this study, we compared CSIC-enhanced MRI with computed tomography during arterial portography (CT AP), which is considered the most reliable imaging modality to detect HCC. Of 20 HCCs pathologically confirmed in 16 patients, 18 (90%) were detectable with CT-AP. Sixteen HCCs (80%) were detectable with unenhanced MRI. After the administration of CSIC, 18 (90%) could be detected. CSIC-enhanced MRI was found to be superior to CT-AP in the detection of HCCs in patients with portal flow abnormalities, i.e., porto-systemic shunting, laminal flow of contrast material and wedge-shaped perfusion defect. In contrast, CT-AP was found to be superior to CSIC-enhanced MRI in detecting HCCs of less than 1 cm in diameter. We consider that these imaging procedures are complementary to each other, and their combined use is the most reliable method by which to detect HCC.

Chondroitin Sulfate Iron Colloid as MR Contrast Agent in Differentiation between Hepatocellular Carcinoma and Adenomatous Hyperplasia

Using a 1.5 T MR imaging unit, T1- and T2-weighted images were obtained before and after i.v. administration of chondroitin sulfate iron colloid (CSIC) in order to differentiate hepatocellular carcinoma (n = 20) from adenomatous hyperplasia without atypia (n = 16). Differentiation was made from the tumor-liver contrast to noise ratio (CNR) and visual evaluation of the nodule, with reference to signal intensity relative to that of the surrounding liver. The CNR of adenomatous hyperplasia was on T1-weighted images significantly decreased after CSIC administration (p &lt; 0.01). On T2-weighted images, there was no significant difference in CNR after CSIC administration. On the other hand, the CNR of hepatocellular carcinoma was significantly increased after CSIC administration on both T1- and T2-weighted images (p &lt; 0.01). CSIC reflects intratumor reticuloendothelial cellular functions, and is therefore useful in differentiating hepatocellular carcinoma from adenomatous hyperplasia without atypia.

Bone Marrow Transplantation Corrects the Splenic Reticuloendothelial Dysfunction in Sickle Cell Anemia

In sickle cell anemia (SCA), the loss of reticuloendothelial function is the result of vasoocclusive events occurring in the spleen. Such asplenia occurs early in the course of the disease and is considered to be permanent in late childhood. In this report, three patients 10, 11, and 14 years of age suffering from severe SCA and found to be asplenic were treated by bone marrow transplantation (BMT). Before transplantation, all three patients had loss of reticuloendothelial splenic function, as assessed by the presence of abundant Howell-Jolly bodies on blood smears and absence of technetium 99m (99mTc) splenic uptake. After BMT, Howell-Jolly bodies disappeared from blood smear, whereas 99mTc isotopic scan found normal isotope uptake. Our data indicate that BMT can correct "permanent asplenia" in SCA patients. However, it remains to be determined if such treatment can also correct other SCA-related organ dysfunctions.

Bone marrow transplantation corrects the splenic reticuloendothelial dysfunction in sickle cell anemia

In sickle cell anemia (SCA), the loss of reticuloendothelial function is the result of vasoocclusive events occurring in the spleen. Such asplenia occurs early in the course of the disease and is considered to be permanent in late childhood. In this report, three patients 10, 11, and 14 years of age suffering from severe SCA and found to be asplenic were treated by bone marrow transplantation (BMT). Before transplantation, all three patients had loss of reticuloendothelial splenic function, as assessed by the presence of abundant Howell-Jolly bodies on blood smears and absence of technetium 99m (99mTc) splenic uptake. After BMT, Howell-Jolly bodies disappeared from blood smear, whereas 99mTc isotopic scan found normal isotope uptake. Our data indicate that BMT can correct “permanent asplenia” in SCA patients. However, it remains to be determined if such treatment can also correct other SCA-related organ dysfunctions.