1/2698. central nervous system T-cell lymphoproliferative disorder in a patient with chronic active Epstein-Barr virus infection.PURPOSE: central nervous system (CNS)-T cell lymphoproliferative disorder (T-LPD) developing during the course of chronic active Epstein-Barr virus (CAEBV) infection is reported. patients AND methods: CAEBV was diagnosed in a 14-month-old boy with fever, cytopenia, hepatosplenomegaly, and abnormal high titers of anti-Epstein-Barr virus (EBV) antibodies. At 8 years of age, he had a splenectomy because of progressive disease. RESULTS: After 27 months of clinical remission, muscle weakness and paresthesia developed. magnetic resonance imaging of his brain showed spotty T2 prolongation in left parietal, bilateral frontal, and temporal white matter with meningeal enhancement. brain biopsy revealed the cerebral infiltration of CD3 , CD4 , CD8-, CD45RO , CD56-, and EBV-encoded rna 1 cells. CONCLUSIONS: The CNS involvement of EBV-associated T-LPD is a rare but serious complication in CAEBV without known underlying immunodeficiency.- - - - - - - - - - ranking = 1keywords = disease (Clic here for more details about this article) |
2/2698. Acute leukemia with the phenotype of a natural killer/T cell bipotential precursor.An acute leukemia with an unusual immunophenotype developed in a 17-year-old girl. At the initial presentation, extramedullary involvement was not evident, but with advancing disease, massive splenomegaly and an osteolytic rib tumor developed. The disease was aggressive and refractory to intensive chemotherapeutic regimens for myeloid and lymphoid malignancies, and the patient died 3 months after the initial presentation. The leukemic cells were of irregular shape and variable size; they had deeply indented or bi-lobed nuclei and relatively fine, azurophilic granules in their cytoplasm. They were positive for acid phosphatase and beta-glucuronidase in granular staining, but they were negative for myeloperoxidase. The leukemic cells had a unique immunophenotype: it was positive for T-cell antigens (CD1a, CD2, cytoplasmic CD3, CD4), myeloid antigens (CD13 and CD33), NK-cell antigen (CD56), CD19 and CD30. dna analysis revealed no gene rearrangement in the T-cell receptor beta, gamma and delta, or immunoglobulin heavy chain genes. The leukemic cells of our patient are thought to have arisen from the transformation of a putative precursor cell common to both the T- and NK-cell lineage in the bone marrow. The current literature on precursor NK-cell malignancy is reviewed, and its clinicopathological feature is discussed.- - - - - - - - - - ranking = 2keywords = disease (Clic here for more details about this article) |
3/2698. Long-term follow-up of relapsed acute leukemia treated with immunotherapy after allogeneic transplantation: the inseparability of graft-versus-host disease and graft-versus-leukemia, and the problem of extramedullary relapse.Long-term outcome of 23 acute myeloid (AML, n=16) or lymphoblastic (ALL, n=7) leukemia patients who had received immunotherapy for treatment of persistent or recurrent disease 1.5-26 (median 4) months after allogeneic transplantation was studied to determine eventual survival. Immune manipulation comprised donor leukocyte infusion (n=18), interferon-alpha2b and/or interleukin-2 (n=15), and cyclosporine withdrawal (n=11) in various combinations. Graft-versus-host disease (GVHD) developed in 12 patients. Thirteen of 20 evaluable patients responded; 6 relapsing again. Eight patients died of toxicity, and 10 of progressive disease at 3-206 weeks (median 11). Five patients (3 AML, 2 ALL) are alive in remission with GVHD 2-46 months (median 23) after immunotherapy with Karnofsky scores of 70-100% (median 80). The overall survival of the whole group is 1-206 weeks (median 12), with an actuarial survival of 22% at 2 years. The development of GVHD was associated with superior survival in multivariate analysis (P=.007). Seven patients received immunosuppression because of the severity of GVHD (grade III/IV acute or extensive chronic): 3 died of GVHD, 3 improved but relapsed concomitantly, and 1 is alive in remission with extensive chronic GVHD. Four episodes of extramedullary relapse (granulocytic sarcomas) were seen in 3 patients with AML whose marrow remained in remission. We conclude that GVHD appears to be inseparable from graft-versus-leukemia in relapsed acute leukemia patients undergoing immunotherapy with a high proportion of patients dying due to toxicity or progressive disease, and isolated extramedullary relapse seems to be unusually common.- - - - - - - - - - ranking = 8keywords = disease (Clic here for more details about this article) |
4/2698. Thoracic disc herniation mimicking acute lumbar disc disease.STUDY DESIGN: Case report of a 49-year-old woman with a lower thoracic disc herniation mimicking acute lumbosacral radiculopathy. OBJECTIVE: To describe an unusual case of thoracic disc herniation mimicking acute lumbar disc disease. SUMMARY OF BACKGROUND DATA: Symptomatic thoracic disc herniation is rare and its clinical manifestations differ widely from those of cervical and lumbar disc herniations. Midline back pain and signs of spinal cord compression progressing over months or years are the predominant clinical features. Acute and subacute thoracic disc herniation occurs in less than 10% of patients, and isolated root pain is unusual. methods: A 49-year-old woman had acute low back pain radiation into the left buttock and the lateral aspect of the left leg and left foot. magnetic resonance imaging study showed a bulging disc and posterior osteophytes at T11-T12. RESULTS: Surgical removal of the herniated disc and osteophytes rapidly relieved her symptoms and neurologic deficits. A follow-up neurologic examination 3 years later showed normal motor and sensory functions, although low back soreness was noted occasionally. CONCLUSION: A case of thoracic disc herniation mimicking an acute lumbosacral radiculopathy is presented. Compression of the lumbosacral spinal nerve roots at the lower thoracic level after exit from the lumbar enlargement may be the mechanism for this unusual presentation.- - - - - - - - - - ranking = 5keywords = disease (Clic here for more details about this article) |
5/2698. Acute inflammatory neuropathy in charcot-marie-tooth disease.The authors report an association between acute inflammatory neuropathy and previously undiagnosed Charcot-Marie-Tooth 1A disease in a 15-year-old girl. sural nerve biopsy study showed hypertrophic neuropathy with endoneurial infiltrates of macrophages and lymphocytes. This association may be coincidental, but a particular susceptibility to damage of these peripheral nerves cannot be excluded. This report confirms the importance of pes cavus as a sign of long-standing sensorimotor neuropathy.- - - - - - - - - - ranking = 5keywords = disease (Clic here for more details about this article) |
6/2698. prognosis of acute poststreptococcal glomerulonephritis in childhood: prospective study and review of the literature.Serial, clinical, clinicopathologic and histologic studies performed simultaneously following onset of PS-AGN in children for a period of up to 144 months revealed no evidence of progression to chronic glomerulonephritis. Although acute morphologic changes were more severe in renal tissue obtained from patients with AGN following streptococcal upper respiratory infection than following pyoderma, the acute manifestations in both groups subsided 6 to 12 weeks after onset. Cumulative morphologic healing occurred in 20% of patients at 24 months, in 43% at 48 months after onset of PS-AGN; 1 patient who was unhealed at 49 months was lost to follow-up. In 2 patients (6%), acute histologic exacerbations without clinical signs occurred within 24 months after onset. Subsequent healing was documented histologically. Addis counts remained abnormal in a high percentage of patients throughout the 12 years of observation and did not correlate with the histologic findings of renal biopsy tissue. The occasional demonstration of renal vascular disease and/or hypertension may merely reflect the early development of spontaneous essential hypertension although the possibility of a relationship to the previous attack of PS-AGN is intriguing. This question cannot be answered at this time. Renal biopsy studies are more dependable than Addis counts in assessing the course of PS-AGN. The significance of persistence of immunofluorescent and/or electron microscopic changes (subepithelial dense deposits) many years after onset in 58% of 12 patients studied, at a time when a majority of patients (84%) revealed healing by light microscopy, remains to be assessed.- - - - - - - - - - ranking = 1keywords = disease (Clic here for more details about this article) |
7/2698. superior mesenteric artery syndrome simulating acute pancreatitis: a case report.A case of infrapapillary duodenal obstruction secondary to the superior mesenteric artery syndrome is reported. The clinical picture and laboratory data simulated acute pancreatitis but no evidence of pancreatic disease was noted at surgical exploration. A review of the causative factors and treatment of the superior mesenteric artery syndrome is presented along with the differential diagnosis of infrapapillary duodenal obstruction.- - - - - - - - - - ranking = 1keywords = disease (Clic here for more details about this article) |
8/2698. Postpartum onset of acute heart failure possibly due to postpartum autoimmune myocarditis. A report of three cases.autoimmune diseases, especially autoimmune thyroid disease, frequently develop after delivery due to the immune rebound mechanism. Most cases have transient dysfunction of affected organs. Cardiac dysfunction developed after delivery is called postpartum or peripartum cardiomyopathy. However, the aetiology of the disease is not clarified yet. Here we report three cases that developed acute heart failure in the postpartum period. One was complicated with an atrioventricular block and postpartum autoimmune thyroiditis. All patients recovered to normal cardiac function or pre-attack condition after 1 month of therapy with conventional drugs and bed rest. All three had positive antiheart antibody detected by indirect immunofluorescence assay, and one had antibody to heart myosin detected by enzyme-linked immunosorbent assay. Moreover, one of two patients examined revealed lymphocytic infiltration by endomyocardial biopsy. antibodies to 26 viruses were not elevated significantly during the first 2 weeks after admission in any case. It is strongly suggested that heart failure is induced by postpartum autoimmune myocarditis, and thus clinicians should be aware of this disease.- - - - - - - - - - ranking = 4keywords = disease (Clic here for more details about this article) |
9/2698. Case report: acute urinary retention secondary to Isaacs' syndrome.We report an unusual case of acute urinary retention secondary to Isaacs' syndrome due to external urethral sphincter spasm. The patient was able to resume spontaneous voiding after the treatment of the underlying disease. At 6-month follow-up, there were no voiding complaints.- - - - - - - - - - ranking = 1keywords = disease (Clic here for more details about this article) |
10/2698. Clinical outcome in three patients with myelodysplastic syndrome showing polyclonal hematopoiesis.The clinical outcome of 3 myelodysplastic syndrome (MDS) patients with polyclonal hematopoiesis is reported. All patients were heterozygous for the phosphoglycerate kinase (PGK) gene. The presence of polyclonal hematopoiesis was determined by the X-chromosome-linked restriction fragment length polymorphism-methylation method using the PGK gene as a marker. The patients were initially diagnosed as having refractory anemia (RA), RA with ring sideroblasts (RARS), and RA with an excess of blasts (RAEB), respectively. Their pancytopenia persisted during the follow-up period of 11.4 years for the RA patient, 19.5 years for the RARS patient and 0.8 years for the RAEB patient. Although the RARS patient continues to be in good health, leukemic transformation occurred in the other 2 patients. A karyotype change from 46,XX to 45,XX,t(3;21),-7 was observed at the time of disease progression in the RA patient. The coexistence of a monoclonal MDS clone and normal bone marrow cells is thought to be the most probable reason for the polyclonal hematopoiesis of these patients.- - - - - - - - - - ranking = 1keywords = disease (Clic here for more details about this article) |
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