Rescue splenic artery embolization in an adult patient of sickle cell disease presented with acute splenic sequestration crisis.

BACKGROUND: Splenic sequestration crisis is a potentially fatal complication of sickle cell disease, mainly seen in young children. Only a few case series describe the acute splenic sequestration crisis in adults and its management, which primarily consists of supportive care and, in some cases, splenectomy. Splenic artery embolization has seldom been described in sickle cell disease. This is probably the first case in which an adult with sickle cell disease presented with an acute splenic sequestration crisis was managed successfully through splenic artery embolization. RESULTS: This 22-year-old female, a known case of sickle cell disease, presented with severe pain in the abdomen and low-grade intermittent fever for two days, secondary to an acute splenic sequestration crisis. The diagnosis of acute splenic sequestration was made based on clinical and blood parameters, ultrasonography, and computed tomography. Even with adequate supportive care and blood transfusions, the patient's condition worsened with a rapid fall in the hemoglobin and total platelet count. Considering splenectomy to be a high-risk procedure for this patient, a decision of rescue splenic artery embolization was taken, which was successful. CONCLUSION: Splenic artery embolization may be considered a lifesaving procedure in patients with acute splenic sequestration, where the risk of splenectomy can be high. Adequate post-procedure supportive care is vital for preventing complications.

Microfluidic study of retention and elimination of abnormal red blood cells by human spleen with implications for sickle cell disease.

The spleen clears altered red blood cells (RBCs) from circulation, contributing to the balance between RBC formation (erythropoiesis) and removal. The splenic RBC retention and elimination occur predominantly in open circulation where RBCs flow through macrophages and inter-endothelial slits (IESs). The mechanisms underlying and interconnecting these processes significantly impact clinical outcomes. In sickle cell disease (SCD), blockage of intrasplenic sickled RBCs is observed in infants splenectomized due to acute splenic sequestration crisis (ASSC). This life-threatening RBC pooling and organ swelling event is plausibly triggered or enhanced by intra-tissular hypoxia. We present an oxygen-mediated spleen-on-a-chip platform for in vitro investigations of the homeostatic balance in the spleen. To demonstrate and validate the benefits of this general microfluidic platform, we focus on SCD and study the effects of hypoxia on splenic RBC retention and elimination. We observe that RBC retention by IESs and RBC-macrophage adhesion are faster in blood samples from SCD patients than those from healthy subjects. This difference is markedly exacerbated under hypoxia. Moreover, the sickled RBCs under hypoxia show distinctly different phagocytosis processes from those non-sickled RBCs under hypoxia or normoxia. We find that reoxygenation significantly alleviates RBC retention at IESs, and leads to rapid unsickling and fragmentation of the ingested sickled RBCs inside macrophages. These results provide unique mechanistic insights into how the spleen maintains its homeostatic balance between splenic RBC retention and elimination, and shed light on how disruptions in this balance could lead to anemia, splenomegaly, and ASSC in SCD and possible clinical manifestations in other hematologic diseases.

Case Report of Acute Splenic Sequestration Crisis in an Adult Patient with Hb S Disease and Suspected Hereditary Persistence of Fetal Hemoglobin.

Acute splenic sequestration crisis (ASSC) is a life-threatening complication of sickle cell disease characterized by acute splenic enlargement and a rapid drop in hemoglobin (Hb) levels. Although ASSC is predominantly a disease of childhood, rare cases of ASSC have been reported in adults, frequently in association with heterozygous sickle cell disease. We describe a case of ASSC in a 29-year-old woman with homozygous Hb S (HBB: c.20A>T) disease and suspected hereditary persistence of fetal Hb (HPFH), which was accompanied by marked leukocytosis.

Acute Splenic Sequestration Crisis in Adult Sickle Cell Disease: A Report of 16 Cases.

Acute splenic sequestration crisis (ASSC), characterized by rapidly progressive anemia and circulatory compromise in the setting of sudden splenic enlargement, is an uncommon entity among adult sickle cell patients. We reviewed cases of adult ASSC encountered at our institution to generate insight into the recognition, diagnosis, and treatment of the condition. Cases of adult ASSC during a 10-year period were identified retrospectively. Patient charts were reviewed for laboratory and imaging results; demographic data and clinical course were collected and reviewed. Sixteen cases of adult ASSC were identified. Most patients presented with pain crisis; only four of 16 patients presented with abdominal pain. The maximum decreases in hemoglobin (Hb) (42.0%) and platelets (62.1%) occurred at day 2.9, delaying identification and treatment. Hemodynamic instability played a large role in dictating risk stratification. Therapy consisted of transfusion (14/16) and splenectomy (5/16). No recurrences were noted in a mean follow-up time of 5.3 years but review of patients' charts demonstrated that at least one of the patients had two prior episodes. Adult ASSC may present with non specific findings and patients may not deteriorate until several days into a previously uneventful hospital course. Changes in platelet counts may be more reliable markers than changes in Hb level since red cell transfusions may interfere with assessments of the sequestration process. This case series of adult ASSC, the largest reported in the literature to date, highlights common clinical, laboratory, radiological, and pathological features of this uncommon entity and helps to guide recognition, diagnosis, and treatment.

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(AU)

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(AU)

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