technetium-tc-99m-sulfur-colloid and Primary-Myelofibrosis

Intracranial involvement in extramedullary hematopoiesis (EMH) is rare, but it should be suspected in patients with myelofibrosis presenting with chronic severe headache. We present a 9-year-old girl with known myelofibrosis whose headaches were unresponsive to routine treatment. CT and MRI studies of the brain showed diffuse pachymeningeal thickening. CT examinations of the chest and abdomen had demonstrated bilateral thoracic paraspinal masses caused by EMH, suggesting the possibility that the intracranial involvement might also be related to EMH. The diagnosis was confirmed by sulfur colloid isotope scan.

Topics: Brain Diseases; Child; Female; Headache; Hematopoiesis, Extramedullary; Humans; Magnetic Resonance Imaging; Primary Myelofibrosis; Radiopharmaceuticals; Technetium Tc 99m Sulfur Colloid; Tomography, X-Ray Computed

A 60-year-old man with a myeloproliferative syndrome and extramedullary hematopoiesis had progressive respiratory and cardiac insufficiency during the previous 18 months, with advancing interstitial pulmonary disease on chest x-ray. During analysis of his respiratory disease, results of a transbronchial biopsy showed interstitial involvement with increased numbers of megakaryocytes and other panhematopoietic staining elements. Results of a bone marrow scan demonstrated diffuse replacement of pulmonary interstitium with bone marrow, as a component of known ongoing extramedullary hematopoiesis.

Topics: Bone Marrow; Hematopoiesis, Extramedullary; Humans; Lung; Lung Diseases, Interstitial; Male; Middle Aged; Primary Myelofibrosis; Radionuclide Imaging; Technetium Tc 99m Sulfur Colloid

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

Single-agent thalidomide (THAL) at "conventional" doses (> 100 mg/d) has been evaluated in myelofibrosis with myeloid metaplasia (MMM) based on its antiangiogenic properties and the prominent neoangiogenesis that occurs in MMM. THAL monotherapy at such doses produces approximately a 20% response rate in anemia but is poorly tolerated (an adverse dropout rate of > 50% in 3 months). To improve efficacy and tolerability, we prospectively treated 21 symptomatic patients (hemoglobin level < 10 g/dL or symptomatic splenomegaly) with MMM with low-dose THAL (50 mg/d) along with a 3-month oral prednisone (PRED) taper (beginning at 0.5 mg/kg/d). THAL-PRED was well tolerated in all enrolled patients, with 20 patients (95%) able to complete 3 months of treatment. An objective clinical response was demonstrated in 13 (62%) patients, all improvements in anemia. Among 10 patients who were dependent on erythrocyte transfusions, 7 (70%) improved and 4 (40%) became transfusion independent. Among 8 patients with thrombocytopenia (platelet count < 100 x 10(9)/L), 6 (75%) experienced a 50% or higher increase in their platelet count. In 4 of 21 patients (19%), spleen size decreased by more than 50%. Responses observed were mostly durable after discontinuation of the PRED. The dose of THAL in this study (50 mg/d) was better tolerated than the higher doses used in previous studies. Adverse events associated with corticosteroid therapy were mild and transient. Clinical responses did not correlate with improvements in either intramedullary fibrosis or angiogenesis. THAL-PRED is well tolerated and preliminarily appears to be a promising drug regimen for treating cytopenias in patients with MMM.

Topics: Adrenal Cortex Hormones; Aged; Anemia; Angiogenesis Inhibitors; Antineoplastic Agents, Hormonal; Antineoplastic Combined Chemotherapy Protocols; Female; Hematopoiesis, Extramedullary; Humans; Male; Middle Aged; Pancytopenia; Prednisone; Primary Myelofibrosis; Splenomegaly; Technetium Tc 99m Sulfur Colloid; Thalidomide; Treatment Outcome

Pulmonary hypertension (PH) can complicate myelofibrosis with myeloid metaplasia (MMM), may arise in the absence of evidence for thromboembolic disease and carries a grim prognosis. Four patients with MMM and severe symptomatic PH were treated with whole-lung external beam radiotherapy in a single fraction of 100 cGy. Within 72 h, each patient noted marked symptomatic improvement and had relief of hypoxia and reduction of oedema and/or ascites. Three of the four patients enjoyed an objective improvement in pulmonary artery systolic pressure as measured by transthoracic Doppler echocardiography. Low-dose lung radiotherapy may be a useful palliative tool for patients with MMM complicated by PH.

Topics: Aged; Female; Humans; Hypertension, Pulmonary; Male; Middle Aged; Palliative Care; Primary Myelofibrosis; Radionuclide Imaging; Radiopharmaceuticals; Technetium Tc 99m Sulfur Colloid

The occurrence of extramedullary hematopoiesis in the pleura is rare and is usually asymptomatic. It is generally diagnosed on postmortem examination. Herein we describe a 61-year-old woman with agnogenic myeloid metaplasia who sought medical assessment because of progressive dyspnea. Thoracentesis yielded sanguineous fluid with 10% myeloblasts. A 99mTc bone marrow scan demonstrated increased tracer activity throughout both lungs, an outcome consistent with extramedullary hematopoiesis. Fine-needle aspiration and video-assisted thoracoscopy were considered but deferred because of the potential risk of profuse bleeding. Treatment with low-dose external beam irradiation to the left posterior pleura was successful. To date, no hemothorax has recurred.

Topics: Female; Hematopoiesis, Extramedullary; Humans; Middle Aged; Pleura; Pleural Effusion; Primary Myelofibrosis; Technetium Tc 99m Sulfur Colloid; Tomography, X-Ray Computed

This case concerns a patient with intracranial extramedullary hematopoiesis (EH) suspected on a CT scan and subsequently confirmed with In-111 chloride and Tc-99m SC bone marrow scans. The bone marrow scans also provided additional information by demonstrating other sites of EH in the paravertebral tissues and bone marrow expansion into the distal extremities.

Topics: Adult; Bone Marrow; Brain; Hematopoiesis, Extramedullary; Humans; Indium Radioisotopes; Male; Primary Myelofibrosis; Technetium Tc 99m Sulfur Colloid; Tomography, Emission-Computed, Single-Photon; Tomography, X-Ray Computed

Topics: Aged; Bone and Bones; Bone Marrow; Female; Humans; Indium; Indium Radioisotopes; Primary Myelofibrosis; Radionuclide Imaging; Technetium Tc 99m Sulfur Colloid

A 66-year-old man has myelofibrosis with radiocolloid demonstrated splenomegaly and shift of activity to the sudden spleen. Four months later, he experienced sudden left-sided paid. A repeat radiocolloid study showed a marked change. The spleen was represented by only a few functional areas with several photopenic regions. Radioactivity was now principally present in the liver, with considerable uptake in the vertebral marrow and lungs. The splenic lesions were multiple acute infarcts. Sudden loss of the spleen's ability to extract radiocolloid was likely, followed by utilization of reticuloendothelial cells in the liver, bone marrow and lungs.

Topics: Aged; Humans; Male; Primary Myelofibrosis; Radionuclide Imaging; Spleen; Splenic Infarction; Splenomegaly; Sulfur; Technetium; Technetium Tc 99m Sulfur Colloid

A patient with postpolycythemic myeloid metaplasia developed an enlarging abdominal mass documented on TCT scanning. To distinguish between lymphoma and extramedullary hematopoiesis, marrow elements were imaged with indium-111 chloride and technetium-99m sulfur colloid. Because the mass failed to accumulate either tracer, a presumptive diagnosis of lymphoma was made and exploratory surgery was performed. The excised mass was found to consist of enlarged lymph nodes containing extramedullary hematopoiesis. Caution should be exercised in the use of In-111 or Tc-99m SC bone-marrow scans to diagnose sites of extramedullary hematopoiesis.

Topics: Bone Marrow; Diagnostic Errors; Hematopoiesis; Humans; Indium; Lymphoma; Male; Middle Aged; Primary Myelofibrosis; Radionuclide Imaging; Sulfur; Technetium; Technetium Tc 99m Sulfur Colloid

Bone marrow scintigraphy was performed in 29 patients with hemopoietic depletion states of various etiology. Two tracers were used for visualization of the marrow: 99mTc-sulphur-colloid and 111InCl3, some patients being examined by both indicators. 111InCl3 is bound on transferrin and is adsorbed on the surface of reticulocytes and erythroblasts). The scintillation camera PHO GAMMA SEARLE IV fitted with moving table and computer CLINCOM was used, so that whole body images were obtained. The comparison of all scans and marrow puncture smears was done. In patients with aplastic anemia with both hyperplastic or hypoplastic marrow good correlation of bone marrow scans and sternal puncture smears was found. In several cases the scintigraphic examination helped to establish a diagnosis of marrow depletion state. A peculiar disadvantage of the imaging method with either 99mTc-sulphur-colloid or 111InCl3 is that it shows the disorders in erythropoietic and reticuloendothelial cells, whereas the defects in myelopoietic cell series and platelet precursors are not provable. According to literature data, great attention is paid to the prognostic value of scintigraphic examination in aplastic anemia.

Topics: Adult; Anemia, Aplastic; Bone Marrow; Bone Marrow Diseases; Humans; Indium; Male; Middle Aged; Primary Myelofibrosis; Radioisotopes; Radionuclide Imaging; Sulfur; Technetium; Technetium Tc 99m Sulfur Colloid