Indicative morphological myelodysplastic alterations of bone marrow in overt AIDS

In HIV infection, numerous alterations of the hematopoietic system, with frequent cytopenias in peripheral blood and dysplasia of the bone marrow, have been observed. In order to assess the incidence of the myelodysplastic anomalies, 69 bone marrow aspirate smears from 47 patients with group IV HIV infection, as classified by CDC, have been studied. Various degrees of myelodysplastic alterations were found in all cases; however, dysgranulopoiesis was more frequent and more accentuated than other kinds of dyshematopoiesis. Intense vacuolization, especially in the granuloblastic series, was very frequent. It is felt that a bone marrow configuration that is highly indicative of overt AIDS can be sketched. The human immune deficiency virus may be either directly or indirectly responsible for myelodysplastic alterations.     

Human Herpesvirus-8 Serology in Pediatric Organ Transplantation

Human herpesvirus (HHV)-8 has been demonstrated to be involved in the pathogenesis of Kaposi's sarcoma, body cavity lymphomas, multicentric Castleman disease, and, more recently, acute bone marrow failure. In order to study the correlation between HHV-8 and immunosuppression, we performed a serological study in a group of 28 pediatric heart and heart-lung transplant recipients; of mean age 11.5 ± 2.5 years. We analyzed blood samples prior to, at 3 to 6 months, and at least 12 months after transplantation. All patients were negative pretransplantation. We observed 10 seroconversions: one patient at 6 months; two patients at 12 months; and seven within the third year after transplantation. Our preliminary data demonstrated that HHV-8 seroconversion is frequent among pediatric transplant recipients. It was probably related to the length of immunosuppression rather than the organ transplantation; Serological assays of all donor specimens of seroconverted patients were negative.     

Autologous bone marrow processing for autotransplantation using an automated cell processor and a semiautomated procedure

Twenty bone marrow aspirates harvested for autotransplantation from 20 patients suffering from several oncohematological diseases were processed using the automated Du Pont SteriCell processor. In 15 bone marrow harvests, the interface buffy coat cells were collected using the SteriCell processor in manual mode with a semiautomated procedure. The procedure yielded an average red cell removal of 84% and an average mononuclear cell (MNC) recovery of 86%. Cloning efficiencies of hematopoietic progenitor cells (CFU-GM and BFU-e) did not differ between processed and recovered MNCs. Four cryopreserved bone marrow buffy coats were thawed and reinfused into four patients who had undergone high dose chemotherapy. Stable engraftment was observed in all cases. In five bone marrow harvests, the SteriCell automated density gradient MNC isolation procedure was performed after buffy coat collection. The whole two-step procedure allowed an average MNC recovery of 69%. CFU-GM and BFU-e assays did not show a significant difference in cloning efficiency between processed and recovered bone marrow MNCs. We conclude that the SteriCell processor offers rapid, safe and feasible procedures for the semiautomated processing of human bone marrow for transplantation. The clinical efficacy of density gradient separated bone marrow employing the automated step and the opportunity to use fully automated processing must be investigated.     

Dermoid cyst in undescended testis in a 9-year-old boy.

Testicular dermoid cysts are rare in the pediatric age group: their discovery in an undescended testis is exceptional. The origin of this lesion is still unclear, but it should be considered as a monolayer expression of a mature teratoma. Though surgeons when confronted with these intratesticular cysts have traditionally opted for castration, in presence of small, compact masses, simple enucleation may be recommended, provided that the integrity of the neighboring parenchyma is not affected.     

Lymphocyte subset counting by immunoperoxidase using an automated routine hematologic analyzer

Using monoclonal antibodies against CD2, CD4, CD8 and CD19 antigens and an automated biotin-avidin immunoperoxidase technique on whole blood samples, we evaluated the technical performance and clinical usefulness of lymphocyte subset counting by the routine hematology analyzer Technicon H*1. Statistical evaluation demonstrated excellent precision and very good correlation with the immunofluorimetric flow cytometer Ortho Spectrum III. Correlation between manual immunofluorescence at the microscope and the H*1 method was much poorer, owing to the high intrinsic imprecision of the manual method. Reference ranges obtained with the H*1 immunoperoxidase method in 44 healthy subjects closely matched those obtained with the Spectrum III. In 46 subjects with or at risk for HIV infection, we found with the H*1 method a significant decrease in CD4+ cells and in the CD4+/CD8+ cell ratio, which was progressively more marked in HIV- negative patients with lymphadenopathic syndrome, AIDS-related complex, and in patients with full-blown AIDS.     

Depletion lymphocytapheresis in chronic lymphocytic leukemias: criteria for predicting which patients will respond to treatment

Depletion lymphocytapheresis therapy using the continuous-flow separator was used in twenty-two cases of chronic lymphocytic leukemia. Applying the Montserrat scoring system, a prediction can be made, to a good approximation, whether the patient will be a "responder" or a "non-responder" to leukapheresis. Criteria have been formulated for decisions on when depletion lymphocytapheresis is absolutely or only relatively indicated, on the basis of total scores from 2 through 6 using the Montserrat criterion.     

Eponym

Rasmussen’s syndrome (RS) is a rare acquired progressive inflammatory encephalopathy characterized by drug-resistant partial seizures and cognitive deterioration resulting from a gradual impairment and a subsequent atrophy of a single brain hemisphere. It was firstly described by Theodore Rasmussen in 1958. The original etiopathogenic hypothesis of a chronic viral infection no longer holds. Today, the presumed mechanism is a complex autoimmune process comprising an active role of both cytotoxic T lymphocytes and circulating autoantibodies, activating the subunit 3 of the ionotropic glutamate receptor (GluR3Ab). Several medical options, such as antiepileptic drugs and immunomodulatory therapies, deserve consideration to face epileptic manifestations and to slow neurologic regression. Yet, all these therapies turn out to be almost always just temporizing measures. Surgical disconnection of the affected hemisphere (“functional hemispherectomy”) is the best therapeutic choice to achieve the arrest of the disease, a good seizure control, the block of neurologic decline, and the improvement of patient’s quality of life.     

High-dose chemotherapy as a consolidation approach in advanced ovarian cancer: long-term results.

The aim of this study was to assess the long-term impact of high-dose chemotherapy (HDC) as consolidation in a large series (n = 55) of advanced chemosensitive ovarian cancer patients who were optimally cytoreduced at time of first surgery or at interval debulking surgery (IDS). HDC consisted of carboplatin (600 mg/m(2) days 1 and 2), etoposide (450 mg/m(2) days 1 and 2) and melphalan (50 mg/m(2), days 3 and 4). The primary endpoint of the study was the assessment of time to progression (TTP) and overall survival (OS). In September 2000 the overall population had a median follow-up of 55 months (range 17--137) and a TTP of 35 months with a 5-year TTP rate of 35% (CI 95%: 21--49) whereas OS averaged 75 months with a 5-year OS of 59% (CI 95%: 45--73). In patients achieving optimal primary cytoreduction the median TTP was 44 months with a 5-year rate of 43% (CI 95%: 26--60). In the same series the 5-year OS rate was 62% (CI 95%: 45--79) (median OS = 75 months). In patients who were optimally cytoreduced at the time of IDS the median TTP was 25 months and the 5-year TTP rate was 22% (CI 95%: 3--41) and median OS was 46 months with a 5-year OS rate of 50% (CI 95%: 27--73). HDC with hematopoietic support could represent an effective approach for the treatment of advanced optimally cytoreduced ovarian cancer patients with chemosensitive disease. Patients who underwent IDS because of unresectable tumors at the time of first surgery had the greater survival benefit from HDC.     

Protein Zero Is Necessary for E-Cadherin-Mediated Adherens Junction Formation in Schwann Cells

Protein Zero (P0), the major structural protein in the peripheral nervous system (PNS) myelin, acts as a homotypic adhesion molecule and is thought to mediate compaction of adjacent wraps of myelin membrane. E-Cadherin, a calcium-dependent adhesion molecule, is also expressed in myelinating Schwann cells in the PNS and is involved in forming adherens junctions between adjacent loops of membrane at the paranode. To determine the relationship, if any, between P0-mediated and cadherin-mediated adhesion during myelination, we investigated the expression of E-cadherin and its binding partner, beta-catenin, in sciatic nerve of mice lacking P0 (P0(-/-)). We find that in P0(-/-) peripheral myelin neither E-cadherin nor beta-catenin are localized to paranodes, but are instead found in small puncta throughout the Schwann cell. In addition, only occasional, often rudimentary, adherens junctions are formed. Analysis of E-cadherin and beta-catenin expression during nerve development demonstrates that E-cadherin and beta-catenin are localized to the paranodal region after the onset of myelin compaction. Interestingly, axoglial junction formation is normal in P0(-/-) nerve. Taken together, these data demonstrate that P0 is necessary for the formation of adherens junctions but not axoglial junctions in myelinating Schwann cells.