technetium-tc-99m-sulfur-colloid and Anemia--Sickle-Cell

Bone marrow aspiration and biopsy are excellent techniques for evaluating bone marrow, but this evaluation is limited to a small part of the total blood-forming organ. With the introduction of radionuclide bone marrow imaging, a simple technique became available that overcomes marrow sampling errors by giving a total body view of functioning marrow. Furthermore, the procedure is noninvasive and provides an atraumatic method for evaluating a number of clinical problems including a discrepancy between bone marrow histology and clinical status (possible marrow sampling error), the determination of amount of active marrow after radiation and chemotherapy when further therapy is being considered, detection of sites of extramedullary hematopoiesis, location of the optimal sites for bone marrow biopsy, the diagnosis and staging of diffuse hematologic disorders, detection of metastases, the diagnosis of bone marrow infarcts in hemolytic anemias, and detecting avascular necrosis of the femoral heads. There are two major classes of bone marrow agents: (1) those that are incorporated into the erythroid precursors such as radioiron and (2) colloids that are taken up by the reticuloendothelial system (RES). Indium-111 chloride was originally considered to be an erythropoietic agent but appears to share some properties of RES labels. The best label to use is dependent on the disease being evaluated.

Topics: Anemia, Aplastic; Anemia, Hemolytic; Anemia, Sickle Cell; Bone Marrow; Bone Marrow Diseases; Bone Neoplasms; Erythropoiesis; Femur Head Necrosis; Gold Colloid, Radioactive; Hematopoiesis; Humans; Indium; Iron Radioisotopes; Leukemia; Lymphoma; Mononuclear Phagocyte System; Polycythemia; Primary Myelofibrosis; Radioisotopes; Radionuclide Imaging; Technetium Tc 99m Sulfur Colloid

Aseptic necrosis of the femoral head is a well-defined entity. The underlying diseases originate from very different types of pathological conditions. Alcoholism, cortisone therapy, gout or hyperuricemia, sickle cell anaemia and others all lead, through various pathways, to the impairment of the medullary blood flow. In many instances, a compartment syndrome can be demonstrated in the femoral head. Death of the osteocytes follows bone marrow necrosis. Revascularisation originates in the periphery of the necrotic segment. Vascular buds and fibroblasts invade the medullary space. New bone is laid over the necrotic trabeculae. Mechanical failure results from changes in the bony framework at three different levels. The subchondral boneplate may be weakened by the process of revascularisation, the necrotic trabeculae may fail because of diminished stiffness and strength, and overloading has been demonstrated at the junction between dead and living bone. Elevation of the intramedullary pressure is the first objective sign of impending or established bone necrosis. Scintigraphy with Technetium 99 m - Sulphur colloid can now show the early stages of marrow necrosis. Roentgenographic changes only appear in a later phase of the disease. Aseptic necrosis must be considered as involving both hips, unless proven otherwise. Attention given to the "silent hip" may allow salvage and prevent the occurrence of osteo-arthritic changes leaving merely unilateral disease. As long as the geometrical shape of the femoral head is maintained operation may well prove useful. The aim at this stage is to prevent collapse. It is impossible to know in the early stages whether mechanical failure will occur, but there is general agreement that the femoral head will eventually undergo deformation. A spherical epiphysis is therefore considered a success. All the conservative methods aim to decompress the medullary cavity. Core biopsy, curettage, bone grafting and intertrochanteric osteotomy all have their advocates. After fracture of the subchondral bone plate has occurred, there is evidence that grafts are unable to restore the strength of the necrotic area. Intertrochanteric osteotomy brings under the main load-bearing zone a vital part of the femoral head. Varus osteotomy can be successful if necrosis has spared sufficient of the lateral portion of the head. Rotation osteotomies, as proposed by Sugioka, are more radical and difficult operations. The published results are promising. Reva

Topics: Adult; Alcoholism; Anemia, Sickle Cell; Animals; Barotrauma; Cortisone; Dogs; Female; Femur Head; Femur Head Necrosis; Gout; Hip Prosthesis; Humans; Kidney Transplantation; Male; Methods; Osteotomy; Rabbits; Technetium Tc 99m Sulfur Colloid; Tomography, X-Ray Computed

The purpose of this study is to perform and evaluate baseline abdominal ultrasound in infants with sickle cell anemia who participated in the BABY HUG multiinstitutional randomized placebo-controlled trial of hydroxyurea therapy and to examine the potential relationships among ultrasound results and clinical, nuclear medicine, and laboratory data.. After local institutional review board approval and with informed guardian consent, 116 girls and 87 boys (age range, 7.5-18 months) with sickle cell anemia underwent standardized abdominal sonography at 14 institutions. Imaging was centrally reviewed by one radiologist who assessed and measured the spleen, kidneys, gallbladder, and common bile duct. Baseline physical assessment of spleen size, serum alanine aminotransferase and bilirubin levels, (99m)Tc sulfur colloid liver-spleen scans, and (99m)Tc diethylenetriaminepentaacetic acid clearance glomerular filtration rates (GFRs) were obtained. Analysis of variance and the Student test were performed to compare sonographic findings to published results in healthy children and to clinical and laboratory findings.. The mean (± SD) spleen volume (108 ± 47 mL) was significantly greater than published normal control values (30 ± 14 mL; p < 0.0001). There was no correlation between spleen volume and function assessed by liver-spleen scan. The mean GFR (125 ± 34 mL/min/1.73 m(2)) was elevated compared with control GFRs (92 ± 18 mL/min/1.73 m(2)). Renal volumes (right kidney, 29 ± 8 mL; left kidney, 31 ± 9 mL) were significantly greater than control volumes (right kidney, 27 ± 3 mL; left kidney, 27 ± 3 mL; p < 0.0001) and were positively correlated with GFR (p = 0.0009). Five percent of patients had sonographic biliary abnormalities (sludge, n = 6; dilated common bile duct, n = 2; and cholelithiasis and thickened gallbladder wall, n = 1 each). There was no correlation between biliary sonographic findings and laboratory results.. In infants with sickle cell anemia, sonographic spleen volume does not reflect function, but increased renal volume correlates with GFR and is consistent with hyperfiltration. Sonographic biliary abnormalities can occur early in life, while remaining clinically silent.

Topics: Abdomen; Alanine Transaminase; Analysis of Variance; Anemia, Sickle Cell; Antisickling Agents; Bilirubin; Female; Glomerular Filtration Rate; Humans; Hydroxyurea; Infant; Kidney; Male; Placebos; Radionuclide Imaging; Spleen; Technetium Tc 99m Sulfur Colloid; Treatment Outcome; Ultrasonography

Patients with sickle cell disease (SCD) may develop functional asplenia as a chronic complication, secondary to repeated episodes of polymerisation of haemoglobin S. It is known that increased plasma concentrations of fetal haemoglobin (HbF) reduce the polymerisation of haemoglobin S. Hydroxyurea is a chemotherapeutic agent capable of increasing HbF levels in the red blood cells and its use has recently been proposed in the treatment of SCD. The objective of this study was to evaluate the effects of long-term therapy with hydroxyurea on recovery of splenic function. Twenty-one patients (aged 3-22 years; 14 with SS haemoglobinopathy, 7 with Sbeta(0) haemoglobinopathy) were studied with liver/spleen scintigraphy before and after 6 and 12 months of treatment. All studies were submitted to visual inspection and semi-quantitative analyses using spleen/liver ratios. Imaging prior to treatment demonstrated functional asplenia in nine SS patients and one Sbeta(0) patient and impaired splenic function in five SS patients and six Sbeta(0) patients. After treatment, splenic function improved in ten patients, remained unchanged in eight and worsened in three. Using liver/spleen imaging, it was possible to demonstrate that hydroxyurea is capable of improving splenic function in some SCD patients. Improvement is not always possible and frequently does not lead to a normal splenic function even after 1 year of treatment.

Topics: Adolescent; Adult; Anemia, Sickle Cell; Blood Transfusion; Child; Child, Preschool; Female; Fetal Hemoglobin; Follow-Up Studies; Humans; Hydroxyurea; Liver; Male; Radionuclide Imaging; Radiopharmaceuticals; Sensitivity and Specificity; Spleen; Technetium Tc 99m Sulfur Colloid; Treatment Outcome

Topics: Anemia, Sickle Cell; Bone and Bones; Child, Preschool; Diagnosis, Differential; Humans; Infarction; Male; Osteomyelitis; Radionuclide Imaging; Radiopharmaceuticals; Technetium Tc 99m Sulfur Colloid

Over a period of 11 y, 50 patients (22 males, 28 females; age range, 8 mo to 22 y) presenting with sickle cell-associated bone pain underwent 93 sequential examinations with 99mTc-sulfur colloid bone marrow scanning and 99mTc-diphosphonate bone scanning. Multiple examinations were performed on 21 patients. The number and distribution of total acute, healed, and nonhealed infarcts by location were recorded on a skeletal homunculus.. For this population, the total number of sites of bone and bone marrow infarction was 464. Of these, 175 were classified as acute by clinical and scintigraphic findings. There were a total of 61 nonhealed sites and 162 healed sites.. Knowledge of the distribution and natural history of sites of bone and bone marrow infarction is of considerable clinical and diagnostic import in the ongoing evaluation and treatment of sickle hemoglobinopathies.

Topics: Adolescent; Adult; Anemia, Sickle Cell; Bone and Bones; Bone Marrow; Child; Child, Preschool; Female; Humans; Infant; Infarction; Male; Radionuclide Imaging; Radiopharmaceuticals; Technetium Tc 99m Medronate; Technetium Tc 99m Sulfur Colloid; Wound Healing

We studied six patients with sickle cell disease (SSD), five homozygous for sickle cell anemia and one with sickle beta-thalassemia, in whom rounded intrasplenic masses proved to be preserved functioning splenic tissue.. Available images including computed tomography, ultrasonography, bone scans (Tc-99m MDP), liver spleen scans (Tc-99m sulfur colloid), and MRI were evaluated.. The masses were low density on CT (in an otherwise calcified spleen), hypoechoic relative to the echogenic spleen on US, and had the imaging characteristics of normal spleen on MRI. They failed to accumulate Tc-99m MDP but did demonstrate uptake of Tc-99m sulfur colloid.. In a patient with SSD and intrasplenic masses, proper correlation of multiple imaging modalities will establish the diagnosis of functioning splenic tissue and avoid mistaken diagnosis of splenic abscess or infarction.

Topics: Adolescent; Adult; Anemia, Sickle Cell; Bone and Bones; Female; Humans; Magnetic Resonance Imaging; Spleen; Technetium Tc 99m Medronate; Technetium Tc 99m Sulfur Colloid; Tomography, Emission-Computed; Tomography, X-Ray Computed; Ultrasonography

Splenomegaly in adult patients with homozygous sickle cell anemia (HbSS) is uncommon and splenic sequestration crises are rare. This paper describes a patient with HbSS who, at the age of 24, began to experience acute splenic sequestration crises. These episodes occurred with sufficient frequency and severity to warrant splenectomy. This case is presented to emphasize that, although rare, splenomegaly can persist in adults with homozygous HbSS and can be associated with severe and even life-threatening splenic sequestration. The incidence of splenomegaly in adults with HbSS and the factors linked to it will be discussed and the published reports of splenic sequestration crises in this patient population reviewed. It appears that high hemoglobin F (HbF) levels and alpha-thalassemia may be important etiologic factors in causing persistence of splenomegaly and predisposing patients to splenic sequestration crises.

Topics: Adult; Anemia, Sickle Cell; Fetal Hemoglobin; Hemoglobins; Homozygote; Humans; Male; Radiography; Splenectomy; Splenomegaly; Technetium Tc 99m Sulfur Colloid

During a prolonged hospital stay, left hip pain developed in a woman with sickle cell disease and bilateral hip prostheses. In-111 labeled WBC scintigraphy supplemented by Tc-99m SC bone marrow imaging demonstrated abnormal WBC accumulation surrounding the left greater trochanter. Results of surgical exploration showed an abscess involving the pseudocapsule, trochanteric bursa, and periprosthetic cement column. Cultures grew Clostridium difficile, an unusual pathogen in this site.

Topics: Abscess; Adult; Anemia, Sickle Cell; Bone Marrow; Clostridioides difficile; Clostridium Infections; Female; Hip Prosthesis; Humans; Indium Radioisotopes; Leukocytes; Prosthesis-Related Infections; Radionuclide Imaging; Technetium Tc 99m Sulfur Colloid

To determine the effects of blood transfusions on splenic function in older patients with sickle cell anemia, we investigated splenic function in 12 patients who had had cerebrovascular accidents and who were being treated at two collaborating centers using different transfusion protocols. Splenic function was assessed by radionuclide scan and pocked erythrocyte count. Patients were 6 to 18 years of age and had been receiving transfusions for 7 months to 10 years (median 4.2 years). Of the 12 children, five had normal or increased splenic size and function (normal scan and normal or minimally elevated pocked erythrocyte count). All were receiving intensive transfusion therapy, with the aim of maintaining the hemoglobin S level at less than 20%. The other seven patients had abnormal splenic function (absent radionuclide uptake and elevated pocked erythrocyte count); each was receiving less intensive transfusion therapy, with the pretransfusion hemoglobin S level usually at 30% to 40%. No patient developed bacterial septicemia while receiving hypertransfusion therapy. We conclude that splenic function during a long-term transfusion program is variable, depending in part on the "intensity" of transfusion therapy. Apparent splenic involution and fibrosis may be a reversible event in some patients.

Topics: Adolescent; Anemia, Sickle Cell; Blood Transfusion; Child; Erythrocyte Transfusion; Erythrocytes, Abnormal; Female; Hemoglobin, Sickle; Humans; Male; Radionuclide Imaging; Spleen; Splenic Infarction; Technetium Tc 99m Sulfur Colloid

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

The clinical records and scintigrams of patients with sickle hemoglobinopathy who underwent combined Tc-99m bone marrow imaging and Ga-67 imaging to differentiate osteomyelitis from bony infarction were reviewed. Of 18 paired examinations in 15 patients, osteomyelitis was diagnosed correctly in six cases; in all six, gallium uptake at the symptomatic site was incongruently increased relative to the bone marrow activity. Of the 12 episodes of infarction, 11 showed congruent activity on both Tc-99m and Ga-67 images. The remaining study was interpreted incorrectly as osteomyelitis due to incongruent Tc-99m and Ga-67 uptake. The use of sequential Tc-99m bone marrow and Ga-67 imaging is an effective means of distinguishing osteomyelitis from bony infarction in patients with sickle hemoglobinopathy.

Topics: Adolescent; Adult; Anemia, Sickle Cell; Bone and Bones; Bone Marrow; Child; Child, Preschool; Diagnosis, Differential; Gallium Radioisotopes; Humans; Infant; Infarction; Osteomyelitis; Radionuclide Imaging; Technetium Tc 99m Aggregated Albumin; Technetium Tc 99m Sulfur Colloid

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

Bone scans or bone marrow scans or both were obtained during 42 episodes of bone pain in 40 children with sickle cell disease, and the usefulness of these procedures was compared. On the basis of the subsequent clinical course, a diagnosis of bone infarction was made in 34 episodes, and osteomyelitis in eight. Among 22 patients with bone infarction, uptake on bone scan was increased in 14, decreased in three, and normal in five. Seven of eight patients with osteomyelitis had increased uptake on bone scan; one had normal uptake. In contrast, marrow scan uptake was markedly decreased in 15 of 16 patients with bone infarction, and was normal in five of five patients with osteomyelitis. Thus, decreased uptake on bone marrow scan in a patient with sickle cell disease and bone pain almost invariably indicates infarction, whereas normal uptake strongly suggests the diagnosis of osteomyelitis. We found marrow scans more useful than bone scans for this differential diagnosis.

Topics: Adolescent; Anemia, Sickle Cell; Bone and Bones; Bone Marrow; Child; Child, Preschool; Diagnosis, Differential; Female; Humans; Infant; Infarction; Male; Osteomyelitis; Radionuclide Imaging; Technetium Tc 99m Medronate; Technetium Tc 99m Sulfur Colloid

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

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

Topics: Adult; Anemia, Sickle Cell; Biopsy; Bone Marrow; Female; Fever; Humans; Mononuclear Phagocyte System; Necrosis; Pancytopenia; Radionuclide Imaging; Sulfur; Technetium; Technetium Tc 99m Sulfur Colloid

Three cases of sickle cell disease associated with functional asplenia are described. The spleen was not visualized on routine Tc-99m-sulfur colloid scan. The bone scan performed with Tc-99m-phosphate compounds revealed abnormal splenic activity in all three cases. The previous case reports and the literature on this subject are reviewed.

Topics: Adult; Anemia, Sickle Cell; Bone and Bones; Etidronic Acid; Female; Humans; Male; Organotechnetium Compounds; Radionuclide Imaging; Spleen; Sulfur; Technetium; Technetium Tc 99m Sulfur Colloid