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1/186. Long-term successful coronary artery angioplasty in polycythemia vera.

    In a 65-year-old man with polycythemia vera, invalidating angina pectoris was associated with severe narrowing of the right coronary artery. After percutaneous coronary angioplasty (PTCA) the patient became symptom free and remained so for 12 months, while receiving an antiplatelet agent, a calcium antagonist and nitrate. coronary angiography repeated after a year, because of reappearance of angina, documented good patency of the treated artery and some progression of a narrowing involving another coronary vessel. This is the first reported case of long-term success of PTCA in polycythemia vera, a disease exposed to a high risk of thrombosis and, possibly, of restenosis. It is undefined whether medical treatment contributed to the anatomical and clinical results. As far as a single case can say, polycythemia vera might not represent a prohibitive background for coronary PTCA.
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2/186. Development of a middle fossa arachnoid cyst. A theory on its pathogenesis.

    The progression of congenital arachnoid cysts has seldom been documented. We report the case of a child who was diagnosed with arrested hydrocephalus at the age of 13 months. neuroimaging studies performed when the girl was 22 months old showed the appearance of an arachnoid cyst in the right middle fossa, while the previously enlarged ventricles seemed to have decreased in size. To the best of our knowledge, the paradoxical expansion of an arachnoid pouch following a reduction in the size of the ventricular system has not previously been documented. We advance the hypothesis that the development of some cases of arachnoid cyst might be pathogenically related to impaired CSF dynamics associated with pre-existing hydrocephalus. We also briefly review the pertinent literature on the formation and evolution of congenital cerebral arachnoid cysts.
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3/186. Occurrence of acute nonlymphoblastic leukemia in two girls after treatment of recurrent, disseminated Langerhans cell histiocytosis.

    The occurrence of Langerhans cell histiocytosis (LCH) and acute leukemia in one individual has rarely been observed. Despite few exceptions, two distinct patterns of association appear evident: acute lymphoblastic leukemia preceding LCH and LCH preceding acute nonlymphoblastic leukemia (ANLL). The latency of ANLL after the diagnosis of LCH is suggestive of a therapy-related process. This report describes two new cases in whom ANLL was diagnosed 7 years 8 months and 5 years 8 months after the start of initial treatment of disseminated recurrent LCH. Morphology showed blasts from FAB-type M4/M5 in the first patient, who died due to progression of leukemia. The second patient showed myelodysplastic syndrome (refractory anemia with excess of blasts in transformation; RAEB-t) and is now in remission from leukemia 3 years 11 months after allogeneic bone marrow transplantation. The review of a total of 26 patients with ANLL after LCH suggests that the disease has a poor prognosis and allogeneic BMT seems to be the treatment of choice.
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4/186. cytogenetic analysis of a mature teratoma and a yolk sac tumor component of a late relapse of a disseminated testicular nonseminoma.

    We report on the cytogenetics of a primary testicular nonseminoma, a residual mature teratoma after remission-induction chemotherapy, and a late relapse after 9 years of follow-up, in one patient. The late relapse was composed of a mature teratoma and a yolk sac tumor component. Cytogenetic comparison of the different tumors shows that progression of primary testicular nonseminoma to residual mature teratoma and to a late-relapse lesion is accompanied by net loss of chromosomes. In addition, our findings may suggest that transformation to viable cancer in a late-relapse lesion is accompanied by further chromosomal losses.
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5/186. Extramedullary relapse despite graft-versus-leukemia effect after bone marrow transplantation in a girl with juvenile myelomonocytic leukemia.

    A 12 year-old girl with juvenile myelomonocytic leukemia (JMML) and monosomy 7 underwent allogeneic bone marrow transplantation (BMT) from her HLA-matched brother. To monitor the engraftment and the course of the disease we used fluorescence in situ hybridization (FISH) with probes specific for the centromeres of chromosomes X, Y and 7. Complete hematological remission was achieved and confirmed by the virtually exclusive presence of normal male cells in the bone marrow (BM). Acute graft-versus host disease (GvHD) was treated with prednisone and cyclosporine A (CSA) and female cells with monosomy 7 reoccurred in the peripheral blood (PB) and BM. After discontinuation of the immunosuppressive therapy, the leukemic cells with monosomy 7 disappeared again from these compartments. One year after transplantation, isolated extramedullary relapses occurred in lymph nodes and skin, followed by dissemination of blast cells into the BM, whereas the PB cells remained of donor origin. The fact that the leukemic cells fluctuated with the intensity of the immunosuppressive treatment provides evidence of a graft versus leukemia (GvL) effect in this unusually old girl with JMML with a unique extramedullary disease progression.
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ranking = 16.840608777042
keywords = disease progression, progression
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6/186. Percutaneous revascularization modalities in heart transplant recipients.

    Accelerated allograft vasculopathy significantly limits the survival of heart transplant recipients. The prevalence of allograft coronary artery disease is as high as 18% by 1 year and 50% by 5 years following heart transplant. heart failure and sudden cardiac death are the two most common clinical presentations. In heart transplant recipients with severe, discrete focal allograft vascular disease, percutaneous balloon angioplasty is a viable palliative option. However, its application is limited by a significant restenosis rate and progression of allograft disease in nontreated segments. Diffuse disease with tapering of vessels may be approached by debulking devices. Emerging revascularization modalities for focal stenoses and some of the diffuse tapering vessels include coronary stents, rotational atherectomy, various wavelength lasers, and, to a lesser extent, directional atherectomy. Conceivably, stents will reduce restenosis rates related to focal, discrete plaques; yet it is unknown whether they will be efficacious in short- and long-term treatment of diffusely diseased segments affected by allograft disease. Accurate assessment of clinical outcomes and long-term evaluation is imperative prior to acceptance of these devices as fundamental interventional tools for treatment of allograft coronary artery disease.
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7/186. Massive extramedullary disease progression in a patient with stable multiple myeloma during G-CSF priming for peripheral blood progenitor mobilization.

    High dose therapy followed by autologous peripheral blood progenitor cell (PBPC) transplantation has recently become an encouraging treatment option for younger patients with multiple myeloma (MM). The influence of the growth factors used for progenitor mobilization on myeloma cells is not known. We report on a patient suffering from IgG kappa myeloma who had been in stable, very good partial remission for seven months after standard therapy until PBPC mobilization with granulocyte-colony stimulating factor (G-CSF, Filgrastim) was initiated. Massive extramedullary disease progression occured coincidentally with the administration of G-CSF. The case suggest the possibility of myeloma stimulation by G-CSF during PBPC mobilization.
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ranking = 84.203043885211
keywords = disease progression, progression
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8/186. Development of focal segmental glomerulosclerosis in the renal allograft of a patient with lupus.

    Nephritis has been a recognized complication of systemic lupus erythematosus since the early 1900s. Almost all lupus patients have some degree of renal involvement related to their condition, but a considerably smaller proportion of these patients actually progress to end-stage renal disease (ESRD). However, lupus patients are also susceptible to other primary renal insults that may significantly contribute to the deterioration in their renal function. We present a case of a patient with clinical and pathological evidence of lupus nephritis that progressed to ESRD and subsequently developed "recurrent" focal segmental glomerulosclerosis in her transplant kidney. Retrospective clinicopathologic correlation suggested the possibility of more than 1 primary renal process that eventually led to her dialysis-dependent state. This case illustrates the importance of meticulously examining both clinical and renal biopsy data in patients with lupus nephritis and considering the presence of co-existing renal pathologies to resolve an otherwise discordant picture of disease progression. These considerations may have important therapeutic and prognostic implications.
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ranking = 16.840608777042
keywords = disease progression, progression
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9/186. Coronary artery aneurysm after stent implantation.

    A coronary artery aneurysm that developed 6 months after coronary stent implantation in the left anterior descending artery, was treated by double coronary artery bypass grafting due to restenosis of the affected vessel and progression of right coronary artery stenosis. Although the poststenting aneurysm initially remained postoperative angiography showed that it had disappeared. In addition to thrombotic occlusion, another mechanism behind its disappearance may be that geometric changes of the implanted stent caused by heart retraction during surgery closed the entrance to the aneurysm.
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10/186. trisomy 10 in a child with acute nonlymphocytic leukemia followed by relapse with a different clone.

    We report a 2-year-old Japanese boy with acute nonlymphocytic leukemia (ANLL) having trisomy 10 as the sole chromosomal abnormality. The majority of the marrow blasts had lobulated nuclei and Auer rods in their cytoplasm. The blasts were positive for peroxidase, CD13, CD15, and CD33, but negative for esterase, CD3, CD7, CD34, and HLA-DR, indicating a diagnosis of ANLL, atypical M2 in French-American-British (FAB) classification. He was treated with combination chemotherapy, including anthracyclines, etoposide, and cytosine arabinoside. Four months after achieving the first remission, the disease relapsed during the consolidation therapy, and he died 9 months later. trisomy 10 was not detected at relapse, and blasts showed phenotypes different from those at initial diagnosis. The present case suggests that the prognosis of acute leukemia with trisomy 10 in the pediatric age group may not be good, and that further studies are required to clarify the association of trisomy 10 with leukemogenesis and disease progression.
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ranking = 16.840608777042
keywords = disease progression, progression
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