Liver-spleen Scan Research Articles

Prognostic factors and evaluation of mycosis fungoides and Sézary syndrome

Background: Staging evaluations of patients with mycosis fungoides (MF) and Sézary syndrome (SS) are performed to individualize therapy and to predict survival. Objective: Our purpose was to determine the prognostic factors in patients with MF and SS. Methods: A retrospective study of 101 patients was performed. For inclusion in the study, patients had to have been evaluated for MF or SS within 6 months of the initial definitive histologic diagnosis. The evaluation included physical examination, chest radiograph, peripheral blood smear, lymph node biopsy, bone marrow biopsy, gallium 67 scan, liver-spleen scan and computed tomography (CT) of the chest, abdomen, and pelvis. Results: The type of skin disease present at initial diagnosis was a good prognostic indicator of survival and clinical outcome. Univariate adverse prognostic features included hepatosplenomegaly or adenopathy by CT scan, abnormal liver-spleen scan, abnormal gallium scan, adenopathy, and peripheral blood, bone marrow, and lymph node involvement. Independent prognostic factors in multivariate analysis were the type of skin involvement as well as peripheral blood and visceral involvement. Conclusion: Our study confirms previous reports that type of skin and peripheral blood and visceral involvement are important prognostic factors in patients with MF or SS. Our results support the finding that patients with T1 stage disease have an excellent survival outlook and clinical outcome. (J Am Acad Dermatol 1997; 37:58–67.)

54 INFECTIOUS MONONUCLEOSIS UNMASKING WILSON DISEASE.

Wilson disease is a rare disorder of copper metabolism with variable clinical presentations. Early diagnosis and treatment is imperative to prevent morbidity and mortality. A 15 year old white male presented with infectious mononucleosis cerebellitis and no stigmata of chronic liver disease nor Kayser-Fleischer rings. He had transient elevation of serum transaminases and a prolonged prothrombin time that responded to vitamin K therapy. Persistence of his ataxia and micrographia lead to further investigations which included a low ceruloplasmin and an elevated urinary copper only after penicillamine challenge. A liver-spleen scan showed impaired hepatic function but the percutaneous liver biopsy was histologically normal with absent copper staining. However, hepatic copper analysis was markedly increased at 1,337 mcg/gm with electron microscopy findings of electron dense deposition of copper in the mitochondria. A high index of suspicion for the early detection of Wilson disease should be maintained in any patient with deteriorating mentation and persistent neurologic impairment in spite of paucity of signs of chronic liver disease.

First Report of Reversal of Organ Dysfunction in Sickle Cell Anemia by the Use of Hydroxyurea: Splenic Regeneration

Much of the morbidity associated with sickle cell anemia (SCA) is due to ongoing infarction resulting in organ dysfunction. Because the spleen is often the first organ damaged in this illness, there is a significant impairment of the immune system in these patients. Hydroxyurea (HU) has been shown to increase fetal hemoglobin (HbF) and decrease painful episodes in patients with this disease. It is unclear whether HU can prevent organ damage. We treated two SCA patients with HU for several years and found evidence of reversal of previously documented splenic dysfunction. Patient no. 1 was treated for 30 months with an increase in HbF to 30%. HU was stopped because of cytopenia. She developed left upper quadrant pain. A splenectomy was performed due to the possibility of splenic abscesses. A pathologic review found no evidence of infection and an enlarged spleen that showed active germinal centers. Patient no. 2 was treated for 24 months with HU before developing splenomegaly. His HbF levels were 25% to 30%, his pit counts averaged 2%, and his liver spleen scans showed uptake. These two cases show that chronic HU therapy may reverse splenic dysfunction in certain patients and suggest that this drug may have efficacy beyond the elimination of pain in SCA.

Intrasplenic masses of ?preserved? functioning splenic tissue in sickle cell disease: correlation of imaging findings (CT, ultrasound, MRI, and nuclear scintigraphy)

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.

The liver-spleen scan as a quantitative liver function test: Correlation with liver severity at peritoneoscopy

Sulfur colloid distribution on liver-spleen scan is determined by the perfused Kupffer cell mass. The perfused Kupffer cell mass is proportional to the perfused hepatocyte mass, but is less affected by acute changes in hepatocyte function. Thus, sulfur colloid distribution parameters (precisely measured by quantitative liver-spleen scan [QLSS]) may be an excellent test of the perfused hepatic mass. Although no gold standard exists for confirmation, a close correlation should exist between liver disease severity assessed at peritoneoscopy and sulfur colloid distribution. Peritoneoscopy severity (scored as total peritoneoscopy score [PS]; range, 0–5) was assessed in 76 patients who also had QLSS. Multivariate equation were generated to estimate liver disease severity from the QLSS. Multivariate equation were generated to estimate liver disease severity from the QLSS. These were then applied prospectively in 20 consecutive patients to validate these equations. In 76 patients, 62 were evaluated because of chronic liver disease (CLD) and included those with micronodular (20) and macronodular (20) cirrhosis with various degrees of severity (Child's A, 16, B, 29; C, 17). Multivariate analysis yielded a number of combinations of QLSS parameters that correlated with peritoneoscopic severity. These equations were used to estimate liver disease severity. Estimates of liver disease severity (estimated PS [EPS]) correlated well with the PS in these 76 patients ( r = .9064; r 2 = .8216; P < .0001). Adding histological fibrosis to the QLSS parameters yields an equation for estimating PS that was even more effective ( r =.9462; r 2 = .8953; P < .001). However, validation of multivariate equations requires confirmation of their value in a second population. Applying these equations to a prospective group of 20 patients who subsequently had peritoneoscopic evaluation produced a similar correlation for QLSS parameters alone for estimating severity ( r = .870; r 2 = .757; P < .0001), and this was improved when the equation including histological fibrosis was added ( r = .936; r 2 = .877; P < .001). We believe these data support the QLSS as a quantitative estimate of the perfused hepatic mass that correlates with liver disease severity at peritoneoscopy.

Primary Angiosarcoma of the Spleen: A Case Report and Review of the Literature

We describe a 47-year-old man with shoulder pain, multiple bony lesions, and a 1-cm lesion in the spleen. T-1 facetectomy revealed a poorly differentiated malignant neoplasm. Several months after chemotherapy, multiple splenic lesions were found by computed tomography and liver-spleen scan. A splenectomy showed a malignant spindle-cell neoplasm forming irregular vascular spaces. Tumor cells were positive for factor VIII-related antigen and vimentin. This patient died of extensive metastases from this primary angiosarcoma of the spleen. Splenic angiosarcoma is a rare neoplasm that often has a cryptic presentation and a dismal prognosis.

Functional Asplenia in Hemoglobin SC Disease

The incidence of functional asplenia in sickle-hemoglobin C (SC) disease has not been defined, and the use of prophylactic penicillin to prevent life-threatening septicemia in this disorder is controversial. The percentage of red blood cells with pits (pit count) is a reliable assay of splenic function in other disorders but has not been validated in hemoglobin SC disease. To address these issues, we conducted a prospective, multicenter study of splenic function in persons with hemoglobin SC disease. Baseline clinical data were recorded, and red blood cell pit counts were performed on 201 subjects, aged 6 months to 90 years, with hemoglobin SC; 43 subjects underwent radionuclide liver-spleen scanning. Pit counts greater than 20% were associated with functional asplenia as assessed by liver-spleen scan, whereas pit counts less than 20% were found in subjects with preserved splenic function. Pit counts greater than 20% were present in 0 of 59 subjects (0%) less than 4 years of age, in 19 of 86 subjects (22%) 4 to 12 years of age, and in 25 of 56 subjects (45%) greater than 12 years of age. Other subjects with hemoglobin SC, who had previously undergone surgical splenectomy, had higher pit counts (59.7% +/- 9.5%) than splenectomized subjects without hemoglobinopathy (38.5% +/- 8.8%) or with sickle cell anemia (20.5% +/- 1.9%; P < .001). Two subjects with hemoglobin SC disease (not splenectomized), ages 14 and 15 years, with pit counts of 40.3% and 41.7% died from pneumococcal septicemia. These data indicate that functional asplenia occurs in many patients with hemoglobin SC disease, but its development is usually delayed until after 4 years of age. The pit count is a reliable measure of splenic function in hemoglobin SC disease, but values indicative of functional asplenia (> 20% in our laboratory) are higher than in other disorders. The routine administration of prophylactic penicillin to infants and young children with hemoglobin SC disease may not be necessary.

Perfused Kupffer cell mass

The perfused Kupffer cell mass determines sulfur colloid distribution by liver spleen scan (LSS) and is proportional to the perfused hepatocyte mass. This accounts for the correlation of sulfur colloid distribution with tests of hepatic function and raises the question of whether the LSS can be used as a quantitative test of hepatic function. The recent ability to precisely measure sulfur colloid distribution by single-photon-emission computerized tomography (SPECT) prompted us to evaluate the clinical value in 329 consecutive patients with adequate LSS and clinical information, of which 27 apparent normals and 220 patients with chronic liver disease (CLD) were included in this study. The liver-bone marrow index (LBI) indicated the distribution of counts between the liver and bone marrow. The liver-spleen index (LSI) indicated the distribution between liver and spleen adjusted for spleen size. The LBI and LSI correlated with each other (r = 0.753; P < 0.001). The arithmetic mean of LBI and LSI was defined as the severity score. Detailed clinical evaluation was available in these patients and included 109 who had liver biopsy. A severity score in 27 normals was 102 +/- 5 (mean +/- SD) with all values > 85. The severity score correlated with hepatic fibrosis (r = -0.694; P < 0.001) in 109 patients with benign liver disease who had recent biopsies and with the Child-Pugh classification (r = 0.78; P < 0.001) in 220 patients with CLD.(ABSTRACT TRUNCATED AT 250 WORDS)

Role of nuclear medicine in liver transplantation

Orthotopic liver transplantation is now a very well-established technique for treating patients with end-stage liver disease. Since 1967, more than 26,000 liver transplants have been performed, including 15,000 in the United States. The overall 1-year survival rate is approximately 80% and 5-year survival is 70%. Nuclear imaging plays an important role in the management of liver transplant recipients before and after liver transplantation. The evaluation of candidates potentially includes liver-spleen scan for liver volume, multiple gated acquisition scan, adenosine or stress thallium study, bone scan, and quantitative ventilation perfusion scan for hepatopulmonary syndrome. In the post-transplant phase, the deconvolution analysis (which corrects for the problem of recirculation) is a promising tool for diagnosing rejection, although its role in the transplant population has to be established. A variety of nuclear medicine techniques are helpful in the postoperative diagnosis of biliary complications. By performing a semiquantitative analysis to discriminate hepatocyte dysfunction from biliary disease and measuring hepatocyte extraction fraction by deconvolution analysis and excretion, (T1/2 values measured by the nonlinear list squares technique) have been very promising.

Thoracic Splenosis

The authors present a case that demonstrates the continued high sensitivity of Tc-99m SC liver-spleen imaging for thoracic splenosis. A patient with profound weight loss, positive PPD and left upper lobe lung density on a chest radiograph was felt to have a strong clinical suspicion of a malignant pulmonary lesion. Two needle biopsies of the lesion were consistent with splenic tissue. Transbronchial biopsy of the right lower lobe lung revealed metastatic adenocarcinoma of an unknown origin. A Tc-99m SC liver-spleen scan of the abdomen and thorax indicated the lung density was consistent with thoracic splenosis.

Evaluation of the mechanisms of antiepileptic drug-related chronic leukopenia.

Antiepileptic drug (AED)-related chronic leukopenia [white blood cell (WBC) count < 4,000/microliters] is a dilemma, especially when the AED is effective in controlling seizures. We evaluated the possible mechanisms of leukopenia in 7 patients. Mean WBC count was 3,000/microliters with a mean of 42% polymorphonuclear leukocytes (PMN). The AEDs were carbamazepine (CBZ) alone in 1 patient or CBZ combined with phenytoin (PHT), primidone (PRM), phenobarbital (PB) and/or valproate (VPA) in 5 patients; one patient was receiving PHT only. Bone marrow (BM) aspirates and PMN antibody studies using chemiluminescence were normal. Two liver-spleen scans showed mild relative splenomegaly. After exercise, WBC count (n = 7) increased by 54% (SEM 12%), while the WBC counts in controls (n = 5) increased by 52 +/- 16%. Antinuclear antibodies (Hep-2) were absent in 6 patients and positive (1:160) in 1. PMN adhesion to nylon wool was decreased (54 +/- 10% in patients vs. 80 +/- 5% in controls: n = 13, p < 0.005). Our data, particularly the appropriate WBC response to the stress of exercise, and normal BM examinations suggest that continuation of AED therapy when leukopenia is stable and the percentage of PMN is normal is probably safe. Caution should be used if the absolute PMN count is consistently < 1,000/microliters. BM examinations need not be performed routinely for every patient with neutropenia due to AEDs, especially if the leukopenia fluctuates in the range of 2,000-4,000 cells/microliters.

Symptomatic Heterotopic Splenic Tissue in the Left Renal Fossa

Renal fossa heterotopic splenic tissue is characteristically asymptomatic and is usually an incidental finding that has been reported to mimic renal or adrenal tumors. However, symptoms related directly to the splenic tissue do occur. We present an unique case with simultaneous occurrence of an accessory spleen and splenosis which were diagnosed following intentional radiological investigations conducted to evaluate flank pain which was probably related to the presence of the heterotopic spleens. CT complemented by isotopic liver-spleen scan is the radiological modality of choice in the diagnosis of renal fossa splenosis. However, MRI may significantly contribute to the evaluation by accurate delineation of blood supply and surgical separation planes.

The quantitative liver spleen scan (QLSS) in ascitic patients

The quantitative liver spleen scan (QLSS) in ascitic patients

Grossly Abnormal Liver-Spleen Scan in a Patient with Veno-occlusive Disease of the Liver that Normalized Completely on Follow-up

A case of veno-occlusive disease of the liver of unknown etiology is reported. In the case, the gross abnormality of liver parenchyma, ascites, and colloid shift to spleen, bones, and lungs found on a Tc-99m SC liver-spleen scan, and confirmed on liver biopsy, resolved completely on follow-up. Histologic correlation and a brief review of the literature are presented.

Hepatic sinusoidal ectasia

Oral contraceptive-associated sinusoidal ectasia is a rare lesion of unknown pathogenesis. We describe the case of a 31-year-old woman who had used oral contraceptives for 10 years and was found to have abnormal liver function tests on “routine” examination. A single 9×5×4 cm hypervascular lesion was demonstrated radiographically. The hepatic immuno-diacetic acid and liver-spleen scans were normal. One subcapsular lesion was identified in the resected right hepatic lobe; it was characterized by focal marked dilatation of the sinusoidal spaces associated with rare hepatocyte necrosis and early intrasinusoidal fibrosis. The subcapsular location and the vascular nature of this wedge-shaped lesion suggest it may represent the telangiectatic precursor of a centrally scarred focal nodular hyperplasia.

Noninvasive Imaging of a Retrocardiac Spleen: Unusual Component of Paraesophageal Diaphragmatic Hernia

The findings on chest roentgenograms, cardiac ultrasound, computed tomography (CT), and radionuclide liver-spleen scan are described in a 54-year-old man with a paraesophageal diaphragmatic hernia. The CT revealed the spleen as a solid mass with a notched border adjacent to the intrathoracic retrocardiac stomach. The radionuclide scan showed splenic uptake above the diaphragm. The diagnosis in this case, which, to our knowledge, is the first such reported case, was confirmed at surgery.

Frequent occurrence of asplenism and cholelithiasis in patients with autoimmune polyglandular disease type I

purpose: This study assesses the occurrence of asplenism and gallstones in patients with autoimmune polyglandular disease type I (APG I). patients and methods: Nine patients with APG I (ages 14 to 48) were studied at the National Institutes of Health. Each patient received endocrine testing, a careful examination of his or her peripheral blood smear, lymphocyte immunophenotyping, a liver-spleen scan, and either an upper abdominal ultrasound or a computer-assisted tomogram to evaluate the spleen and gallbladder. results: We documented asplenism in four patients and cholelithiasis in four patients, with two patients having both conditions. The patients with asplenism had Howell-Jolly bodies on peripheral blood smears, lack of splenic uptake by liver-spleen scan, and absent spleens by abdominal computed tomographic scan or ultrasound evaluation. The clinical presentation of the patients with cholelithiasis ranged from acute symptoms requiring surgery to asymptomatic gallstones. Lymphocyte immunophenotyping did not reveal consistent changes in either B- or T-cell subpopulations in the patients studied. conclusion: Asplenism and gallstones occur frequently in patients with APG I. In addition to careful examination of the peripheral blood smear for Howell-Jolly bodies to screen for asplenism, we recommend an abdominal ultrasound to detect asplenism and/or gallstones in all patients with APG I. Appropriate immunizations and antibiotic coverage may be helpful in those patients with absent spleens.

Splenic function in persons with sickle cell trait at moderately high altitude

We investigated the possibility that persons with sickle cell trait who reside chronically at moderately high altitude might develop impaired splenic reticuloendothelial function. Seventeen healthy young black men with sickle trait who had lived at greater than or equal to 1,609 m for greater than or equal to 10 years participated in the study along with 25 matched control subjects with normal hemoglobin. Splenic function was assessed by radionuclide liver-spleen scanning and by red cell pit counts. No evidence of impaired splenic function was found in the sickle trait group. The data suggest that long-term residence at moderately high altitude does not place persons with sickle cell trait at risk for splenic dysfunction.

Urticaria pigmentosa. Systemic evaluation and successful treatment with topical steroids

Nine patients with adult-onset urticaria pigmentosa were studied for the incidence of extracutaneous mast cell involvement and the efficacy of potent topical corticosteroid therapy for cutaneous lesions. Seven of the nine patients had increased mast cells in the marrow biopsy specimens, and five patients had focal aggregates of mast cells. The bone scan was abnormal in one patient. Liver-spleen scans revealed a shift of colloid uptake from liver to spleen in four patients. No abnormal gastrointestinal tract roentgenograms were obtained. Urinary histamine metabolites correlated with nodular bone marrow involvement, but not with other parameters. Results of the psychoneurologic testing revealed significant deviation from the norm with a verbal memory deficit in all nine patients and abnormalities on the Minnesota Multiphasic Personality Inventory in four patients. All nine patients were treated with 0.05% betamethasone dipropionate ointment under occlusion over half of the body nightly for 6 weeks. Seven of nine patients treated responded with almost complete resolution of their lesions. Hypothalamic pituitary adrenal axis suppression was evaluated with intramuscular cosyntropin stimulation and metyrapone administration during treatment. Only two patients, both of whom used the medication improperly, developed transient abnormalities. Slow return of lesions was noted 6 months after completion of therapy. Remissions could be lengthened with single weekly applications of topical steroids. Systemic involvement is frequent in patients with cutaneous mast cell disease and it is best demonstrated by bone marrow biopsy. Mast cell lesions can be safely and effectively treated with topical steroids in motivated patients.

Urticaria Pigmentosa

† Nine patients with adult-onset urticaria pigmentosa were studied for the incidence of extracutaneous mast cell involvement and the efficacy of potent topical corticosteroid therapy for cutaneous lesions. Seven of the nine patients had increased mast cells in the marrow biopsy specimens, and five patients had focal aggregates of mast cells. The bone scan was abnormal in one patient. Liver-spleen scans revealed a shift of colloid uptake from liver to spleen in four patients. No abnormal gastrointestinal tract roentgenograms were obtained. Urinary histamine metabolites correlated with nodular bone marrow involvement, but not with other parameters. Results of the psychoneurologic testing revealed significant deviation from the norm with a verbal memory deficit in all nine patients and abnormalities on the Minnesota Multiphasic Personality Inventory in four patients. All nine patients were treated with 0.05% betamethasone dipropionate ointment under occlusion over half of the body nightly for 6 weeks. Seven of nine patients treated responded with almost complete resolution of their lesions. Hypothalamic pituitary adrenal axis suppression was evaluated with intramuscular cosyntropin stimulation and metyrapone administration during treatment. Only two patients, both of whom used the medication improperly, developed transient abnormalities. Slow return of lesions was noted 6 months after completion of therapy. Remissions could be lengthened with single weekly applications of topical steroids. Systemic involvement is frequent in patients with cutaneous mast cell disease and it is best demonstrated by bone marrow biopsy. Mast cell lesions can be safely and effectively treated with topical steroids in motivated patients. (<i>Arch Dermatol.</i>1991;127:191-196)