BIOMAT Database Logo BIOMAT Database Logo

Acute splenic sequestration crisis in an adult with sickle beta-thalassemia. 2006

Prasad Rao Koduri, and Paula Kovarik

Acute splenic sequestration crisis (ASSC) is a major cause of morbidity and mortality in children with sickle cell disease. Reports of ASSC in adults with sickle beta-thalassemia (S-beta(thal)) are rare and consist of isolated case reports comprising a total of seven patients, three of whom died during the crisis. We report a 22-year-old man with S-beta(thal) who developed ASSC 1 day after suffering multiple blunt trauma. Systemic inflammatory response to severe blunt trauma may have precipitated ASSC in our patient. ASSC in adults with S-beta(thal) is a potentially life-threatening complication with a high risk of recurrence. Splenectomy is recommended after the first attack of ASSC in adults with S-beta(thal).

UI MeSH Term Description Entries
D006971 Hypersplenism Condition characterized by splenomegaly, some reduction in the number of circulating blood cells in the presence of a normal or hyperactive bone marrow, and the potential for reversal by splenectomy. Anemia, Splenic,Anemias, Splenic,Splenic Anemia,Splenic Anemias
D008297 Male Males
D006450 Hemoglobin SC Disease One of the sickle cell disorders characterized by the presence of both hemoglobin S and hemoglobin C. It is similar to, but less severe than sickle cell anemia. SC Disease,Sickle Cell Hemoglobin C Disease,Disease, Hemoglobin SC,Disease, SC,Diseases, Hemoglobin SC,Diseases, SC,Hemoglobin SC Diseases,SC Disease, Hemoglobin,SC Diseases,SC Diseases, Hemoglobin
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D013156 Splenectomy Surgical procedure involving either partial or entire removal of the spleen. Splenectomies
D013163 Splenomegaly Enlargement of the spleen. Enlarged Spleen,Spleen, Enlarged
D014947 Wounds and Injuries Damage inflicted on the body as the direct or indirect result of an external force, with or without disruption of structural continuity. Injuries,Physical Trauma,Trauma,Injuries and Wounds,Injuries, Wounds,Research-Related Injuries,Wounds,Wounds and Injury,Wounds, Injury,Injury,Injury and Wounds,Injury, Research-Related,Physical Traumas,Research Related Injuries,Research-Related Injury,Trauma, Physical,Traumas,Wound
D017086 beta-Thalassemia A disorder characterized by reduced synthesis of the beta chains of hemoglobin. There is retardation of hemoglobin A synthesis in the heterozygous form (thalassemia minor), which is asymptomatic, while in the homozygous form (thalassemia major, Cooley's anemia, Mediterranean anemia, erythroblastic anemia), which can result in severe complications and even death, hemoglobin A synthesis is absent. Anemia, Cooley's,Anemia, Erythroblastic,Anemia, Mediterranean,Hemoglobin F Disease,Thalassemia Major,Thalassemia Minor,Erythroblastic Anemia,Mediterranean Anemia,Microcytemia, beta Type,Thalassemia Intermedia,Thalassemia Major (beta-Thalassemia Major),Thalassemia Minor (beta-Thalassemia Minor),Thalassemia, beta Type,beta Thalassemia,Anemia, Cooley,Anemia, Cooleys,Anemias, Erythroblastic,Anemias, Mediterranean,Cooley's Anemia,Disease, Hemoglobin F,Intermedia, Thalassemia,Intermedias, Thalassemia,Major, Thalassemia (beta-Thalassemia Major),Majors, Thalassemia (beta-Thalassemia Major),Mediterranean Anemias,Microcytemias, beta Type,Minor, Thalassemia (beta-Thalassemia Minor),Minors, Thalassemia (beta-Thalassemia Minor),Thalassemia Intermedias,Thalassemia Major (beta Thalassemia Major),Thalassemia Majors (beta-Thalassemia Major),Thalassemia Minor (beta Thalassemia Minor),Thalassemia Minors (beta-Thalassemia Minor),Thalassemia, beta,Thalassemias, beta,Thalassemias, beta Type,Type Microcytemia, beta,Type Microcytemias, beta,Type Thalassemia, beta,Type Thalassemias, beta,beta Thalassemias,beta Type Microcytemia,beta Type Microcytemias,beta Type Thalassemia,beta Type Thalassemias
D018746 Systemic Inflammatory Response Syndrome A systemic inflammatory response to a variety of clinical insults, characterized by two or more of the following conditions: (1) fever >38 degrees C or HYPOTHERMIA <36 degrees C; (2) TACHYCARDIA >90 beat/minute; (3) tachypnea >24 breaths/minute; (4) LEUKOCYTOSIS >12,000 cells/cubic mm or 10% immature forms. While usually related to infection, SIRS can also be associated with noninfectious insults such as TRAUMA; BURNS; or PANCREATITIS. If infection is involved, a patient with SIRS is said to have SEPSIS. Inflammatory Response Syndrome, Systemic,Sepsis Syndrome,Sepsis Syndromes,Syndrome, Sepsis,Syndromes, Sepsis

Related Publications

Prasad Rao Koduri, and Paula Kovarik
Fatal splenic sequestration crisis with multiorgan failure in an adult woman with sickle cell-beta+ thalassemia.
March 2005, The American journal of the medical sciences,
Prasad Rao Koduri, and Paula Kovarik
Acute splenic sequestration crisis (ASSC) in an adult patient with beta-thalassemia sickle cell disease: a life-threatening complication.
June 2008, Annals of hematology,
Prasad Rao Koduri, and Paula Kovarik
Acute splenic sequestration crisis in an adult with sickle cell anaemia.
March 2012, British journal of hospital medicine (London, England : 2005),
Prasad Rao Koduri, and Paula Kovarik
Fatal splenic sequestration crisis in adult sickle cell-beta thalassaemia.
October 1991, Postgraduate medical journal,
Prasad Rao Koduri, and Paula Kovarik
Sickle cell disease: acute splenic sequestration crisis.
December 1983, Virginia medical,
Prasad Rao Koduri, and Paula Kovarik
[Acute splenic sequestration in an adult with sickle cell disease].
March 2008, Presse medicale (Paris, France : 1983),
Prasad Rao Koduri, and Paula Kovarik
Rescue splenic artery embolization in an adult patient of sickle cell disease presented with acute splenic sequestration crisis.
May 2024, Emergency radiology,
Prasad Rao Koduri, and Paula Kovarik
Acute Splenic Sequestration Crisis After Red Blood Cell Exchange for Acute Chest Syndrome in an Adult With Sickle β-Thalassemia: What Went Wrong?
April 2018, Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy,
Prasad Rao Koduri, and Paula Kovarik
Acute splenic sequestration crisis in adults with sickle cell anemia.
February 2007, American journal of hematology,
Prasad Rao Koduri, and Paula Kovarik
Acute splenic sequestration crisis in children with sickle cell disease.
December 2001, Saudi medical journal,
Copied contents to your clipboard!