technetium-tc-99m-sulfur-colloid and Thalassemia

Topics: Adult; Bone Marrow; Diagnosis, Differential; Hematopoiesis, Extramedullary; Humans; Male; Radionuclide Imaging; Spinal Neoplasms; Technetium Tc 99m Sulfur Colloid; Thalassemia; Tomography, X-Ray Computed

A 19-year-old black woman with sickle cell beta(0) thalassemia had experienced more than 100 hospital admissions for sickle cell crisis and aseptic necrosis of both femoral heads. Her spleen was enlarged threefold and accumulated both radiocolloid and bone-seeking agent on two occasions, demonstrating an exception to the rule in sickle cell anemia that spleens that take up bone-seeking agents demonstrate functional asplenia. In the context of fever, left upper quadrant pain, and splenomegaly, the pattern of calcification in the patient's spleen as revealed in ultrasound and CT studies suggested possible abscess and led to unnecessary splenectomy. The nuclear medicine studies did not support this diagnosis. Nuclear medicine physicians should not be misled by splenic findings of sickle cell thalassemia (and possibly of other heterozygous sickle cell disorders) that differ from those of the more familiar homozygous sickle cell anemia.

Topics: Adult; Anemia, Sickle Cell; Diphosphonates; Female; Humans; Radionuclide Imaging; Spleen; Technetium; Technetium Compounds; Technetium Tc 99m Sulfur Colloid; Thalassemia; Tomography, X-Ray Computed

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

Extramedullary hematopoiesis is an infrequent cause of thoracic masses. Extrathoracic locations are even less common. We have studied two patients, one with suspected pelvic neoplasm, with technetium-99m sulfur colloid marrow image and transmission computerized tomography. This noninvasive evaluation established the diagnosis of pelvic extramedullary hematopoiesis in both. We recommend a similar diagnostic approach in patients with chronic anemia or other predisposing disease, presenting with a pelvic or abdominal mass.

Topics: Adult; Anemia, Sickle Cell; Bone Marrow; Female; Hematopoiesis; Humans; Mediastinal Neoplasms; Mediastinum; Middle Aged; Pelvic Neoplasms; Pelvis; Sulfur; Technetium; Technetium Tc 99m Sulfur Colloid; Thalassemia; Tomography, Emission-Computed

18 thalassaemic children, aged 3.5 to 13 years comprise our clinical material. In 14 of them clinically elicited spleen markings, haematocrit, blood platelet count and red cell morphology were studied daily for the whole period between transfusions. In 10 patients considerable changes in spleen size were noticed. According to out clinical observations the spleen size starts decreasing 1 to 3 d after blood transfusion up to the 10th posttransfusion day fluctuating thereafter to reach its maximum size again prior to the next blood transfusion. The decrease of spleen size was followed by an increase of haematocrit and blood platelet count and vice versa. 4 additional children were studied clinically only twice: prior to and 7 to 10 d after blood transfusion. A definite decrease of the spleen size following blood transfusion was observed. Spleen and liver 99mTc-sulfur colloid uptake was studied in 10 of the above children prior to and 7 to 10 d after blood transfusion. Statistically significant post-transfusion increase of the spleen uptake was demonstrated. Our findings suggest that (a) splenic size is relevant to blood volume sequestrated int this organ, (b) splenic radioactive uptake increases with its post-transfusion reduction in size.

Topics: Adolescent; Blood Transfusion; Child; Child, Preschool; Female; Hematocrit; Homozygote; Humans; Liver; Male; Organ Size; Platelet Count; Radionuclide Imaging; Spleen; Sulfur; Technetium; Technetium Tc 99m Sulfur Colloid; Thalassemia