technetium-tc-99m-sulfur-colloid and Hemoglobin-SC-Disease

Reports of acute splenic sequestration crises in adults with sickle cell hemoglobin C disease or sickle cell thalassemia are rare, although an enlarged and distensible spleen persists in half of these patients. Seven episodes of acute splenic sequestration crises in four adults, two with sickle C disease and two with sickle thalassemia, are described. The crises were life-threatening and recurrent in all, but there were no fatalities. One patient had mild steady-state thrombocytopenia suggesting hypersplenism. Technetium 99m/sulfur colloid scanning of the spleen during the acute splenic sequestration crises in three patients showed almost total lack of splenic uptake or decreased uptake with intrasplenic filling defects thought to be splenic infarcts or hematomas on follow-up computed tomographic scanning. The scanning abnormalities resolved following recovery from the crises. Acute splenic sequestration crises probably are common in adults with sickle C disease and sickle thalassemia but may be underdiagnosed or misdiagnosed as splenic infarctions. The hematologic and splenic findings during acute splenic sequestration crises resemble those following splenic vein ligation in animals.

Topics: Acute Disease; Adult; Anemia, Sickle Cell; Female; Hemoglobin SC Disease; Humans; Male; Pregnancy; Radionuclide Imaging; Recurrence; Spleen; Splenomegaly; Technetium Tc 99m Sulfur Colloid; Thalassemia

Splenic function in sickle hemoglobinopathy syndromes was assessed to determine the developmental pattern of splenic dysfunction. Nonvisualization of the spleen using technetium-99 metastable (99mTc) spleen scans correlated strongly with pocked (vesiculated) RBCs greater than or equal to 3.5%. Cross-sectional analysis of pocked RBC data from 2,086 patients showed differences in the developmental pattern of splenic dysfunction between several disorders. In hemoglobin SS disease (sickle cell anemia) and hemoglobin S beta(0) thalassemia, splenic dysfunction (greater than or equal to 3.5% pocked RBCs) often occurred in the first 6 to 12 months of life. In hemoglobin S beta(+) thalassemia, splenic dysfunction occurred less frequently and later. Splenic dysfunction in hemoglobin SC disease (sickle cell-hemoglobin C) was intermediate. The level of pocked RBCs was inversely associated with fetal hemoglobin (P less than .007) and directly associated with age (P less than or equal to .001). These patterns of splenic dysfunction reflect the known severity of hemolysis and intravascular sickling and are consistent with the epidemiology of severe bacterial meningitis and sepsis in these diseases. Serial measurement of pocked RBCs permits determination of the onset of splenic dysfunction and the time of increased susceptibility to severe bacterial infections.

Topics: Age Factors; Anemia, Sickle Cell; Bacterial Infections; Erythrocytes, Abnormal; Fetal Hemoglobin; Hemoglobin SC Disease; Humans; Infant; Microscopy, Phase-Contrast; Radionuclide Imaging; Risk; Spleen; Technetium Tc 99m Sulfur Colloid; Thalassemia

To determine the clinical significance of splenic uptake in bone scintigraphy and functional asplenia on the radionuclide liver-spleen image, bone and spleen scintigrams of 38 patients with sickle cell disease were reviewed. Eighteen underwent bone and liver-spleen studies, 15 had only bone images, and five had only liver-spleen studies. Sixteen of 33 who had bone scintigraphy showed splenic uptake, but the frequency of homozygous sickle cell (SS) disease was not greater than heterozygous sickle cell disease (S-hetero) in this group. SS patients with splenic uptake of Tc-99m diphosphonate had significantly fewer painful crises of the abdomen and extremities, and fewer inpatient and outpatient hospital visits than SS patients whose bone imaging did not visualize the spleen. Functional asplenia on liver-spleen images (16 cases) was seen only in SS disease. One SS patient, age 8, still had a spleen capable of phagocytizing colloid.

Topics: Adolescent; Adult; Anemia, Sickle Cell; Child; Child, Preschool; Diphosphonates; Female; Hemoglobin SC Disease; Humans; Male; Middle Aged; Radionuclide Imaging; Spleen; Technetium; Technetium Compounds; Technetium Tc 99m Sulfur Colloid