Acute splenic sequestration crisis in adults with sickle cell anemia.
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Biomedical subjects
Publications and source records attributed to Prasad Rao Koduri.
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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).
Acute splenic sequestration crisis (ASSC) is a potentially life-threatening complication and one of the leading causes of death in children with sickle cell disease. It is rarely reported in adults with hemoglobin S-C disease and its natural history in these patients has not been well studied. We describe here the clinicopathological features of ASSC in nine adults with hemoglobin S-C disease treated between 1972 and 2000 and followed for a mean period of 9 years (range 0-21 years). ASSC was characterized by acute left upper quadrant abdominal pain, splenomegaly, fever, and a rapid decrease in hematocrit with active erythropoiesis. The hemoglobin decreased by a mean of 4.8 g/dl from the steady state value (range 3.0 to 6.7 g/dl) during ASSC. Two patients failed to respond to transfusion of packed erythrocytes and required urgent splenectomy. There was one fatality-a 76-year-old woman, who died 36 h after admission. There was no recurrence of ASSC in five patients followed for 2, 3, 16, 18, and 21 years, respectively. ASSC in adults is a serious and occasionally, fatal complication of hemoglobin S-C disease. Patients with hemoglobin S-C disease may remain at risk of ASSC into their eighth decade.
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Sickle cell anemia (SCA) is an inherited disorder of hemoglobin synthesis that is characterized by life-long severe hemolytic anemia, attacks of pain crisis, and chronic organ system damage. A third of the hemolysis in SCA is intravascular and the resulting urinary losses of iron may lead to iron deficiency. There is no evidence of iron overload in SCA and iron deficiency may be more common than suspected, especially in men. Absence of bone marrow iron remains a gold standard for the diagnosis of iron deficiency in these patients. Although low serum ferritin is highly specific for the diagnosis of iron deficiency, its sensitivity is quite low in SCA because of non-specific elevation due to increased red cell turnover. The kinetics of sickling is strongly concentration dependent such that small decreases in the mean corpuscular deoxyhemoglobin-S concentration (MCHC-S) cause a substantial delay in sickle hemoglobin polymerization. Prolongation of the "delay time of gelation" in excess of the capillary transit time may allow the erythrocyte to traverse the capillary bed to escape to the arterial side before there is rheologic impairment of the erythrocyte from polymerization of sickle hemoglobin. Overt iron deficiency lowers the MCHC-S and thereby decreases the sickling tendency and the severity of hemolysis. The clinical improvement in SCA following the induction of iron deficient erythropoiesis by repeated phlebotomies or by erythrocytapheresis has been reported. Prospective controlled studies are needed to evaluate further, the therapeutic strategy of inducing controlled iron-deficient erythropoiesis in selected patients with SCA.
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Four men were diagnosed with human immunodeficiency virus infection (AIDS) and autoimmune hemolytic anemia (HIV-AIHA) during the years 1997-2000 at Cook County Hospital, Chicago. All patients presented with the acute onset of severe hemolytic anemia, fever, and splenomegaly. The direct and indirect antiglobulin tests were positive in all, and three patients had mixed warm and cold autoantibody hemolytic anemia. Two patients responded to prednisone therapy and remain in remission from AIHA for 15 and 30 months, respectively.
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