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R Fonseca

Publications and source records attributed to R Fonseca.

At least 73 records · Page 4Linked to original sources

Plasmablastic morphology is an independent predictor of poor survival after autologous stem-cell transplantation for multiple myeloma.

PURPOSE: To study the prognostic value of plasmablastic morphology after autologous stem-cell transplantation for relapsed or primary refractory myeloma. PATIENTS AND METHODS: Seventy-five patients were studied. Investigators blinded to the clinical details of the individual cases reviewed bone marrow aspirate slides to determine plasmablastic classification. Plasmablasts were defined using strict, well-described criteria. Plasmablastic morphology was considered to be present (plasmablastic myeloma) when 2% or more plasmablasts were present in the plasma-cell population. RESULTS: Patients underwent transplantation 5 to 88 months (median, 20 months) after the initial diagnosis of myeloma. Twenty-eight percent of patients had plasmablostic morphology. A significantly greater proportion of patients with plasmablastic morphology had abnormal cytogenetics compared with those with nonplasmablastic classification (73% v 31%, respectively; P = .003). The overall survival rate measured from the time of transplantation was significantly worse in patients with plasmablastic morphology compared with those without (median survival time, 5 months v 24 months, respectively; P < .001). Progression-free survival time was shortened also, with a median time of 4 months compared with 12 months, respectively (P < .001). In the multivariate analysis, plasmablastic classification was the most powerful prognostic factor after transplantation for both overall (P = .001) and progression-free survival rates (P < .001). We also identified three risk groups based on plasmablastic morphology: plasma-cell labeling index, lactate dehydrogenase, and cytogenetics. The median overall survival time was 38 months when none of these factors was abnormal, 17 months with one abnormal factor, and 8 months with two or more abnormal factors (P < .001). CONCLUSION: Plasmablastic morphology is a powerful independent predictor of poor survival rate after autologous stem-cell transplantation for relapsed or primary refractory myeloma.

Adult↗

Cerebral toxicity in patients treated for small cell carcinoma of the lung.

OBJECTIVE: To present the clinical characteristics of patients enrolled in a trial of treatment of small cell carcinoma (SCC) of the lung and to describe the central nervous system toxicity associated with the chemotherapy and prophylactic cranial irradiation (PCI). MATERIAL AND METHODS: We performed a retrospective analysis of 60 patients with SCC who received chemotherapy and thoracic radiation therapy. PCI was administered to patients who had limited disease or who had extensive disease that was subsequently down-staged to only residual chest disease after initial treatment. The total PCI dose was 3,200 cGy administered in 16 fractions of 200 cGy, given concurrently with systemic chemotherapy. Diagnostic criteria for leukoencephalopathy were based on previously published guidelines. RESULTS: Of the 60 eligible and enrolled patients, 35 received PCI and 25 did not. Leukoencephalopathy developed in 5 of the 35 patients (14%) who received PCI. The median age of the patients in whom leukoencephalopathy developed was 64 years (range, 57 to 69), and the median follow-up time was 59 months. The most common signs and symptoms of leukoencephalopathy were intellectual changes, memory alterations, and motor abnormalities. The mean time to onset of symptoms after termination of irradiation was 357 days (range, 30 to 524). Of all 60 patients, 6 were still alive 4 years after enrollment, and 3 of them (50%) already had leukoencephalopathy. CONCLUSION: Small dosage fractions of PCI may still result in leukoencephalopathy. The routine use of PCI in the management of SCC should be reassessed because of increasing evidence of the toxicity associated with it.

Aged↗

Follicular dendritic cell sarcoma and interdigitating reticulum cell sarcoma: a review.

Neoplasms of reticular dendritic origin are extremely rare and include the follicular dendritic cell sarcoma (FDCS) and the interdigitating reticulum (or dendritic) cell sarcoma (IDCS). In this article, we review the literature pertaining to the two diseases and describe clinical observations and salient pathologic features, including information provided by authors of FDCS and IDDCS reports. We performed a computerized database search for published articles regarding FDCS and IDDCS. The articles were evaluated critically by the authors. Simple descriptive statistics were used to analyze the data. There are 51 cases of FDCS and 21 cases of IDDCS that are well documented in the literature. The pathologic diagnosis of FDCS and IDDCS is often challenging and requires morphologic, immunophenotypic, cytochemical, and electron-microscopic analysis. Patients with FDCS usually present with cervical or axillary lymphadenopathy, but extranodal disease has been described. In at least some patients, preexisting Castleman's disease has been recognized. Resected localized disease may be prevented from recurrence by consolidative radiotherapy. Chemotherapy regimens have shown nondurable antitumor activity in FDCS. Patients with IDDCS usually present with lymphadenopathy. The clinical course of IDDCS has been variable, but it seems to be more aggressive than that of FDCS. Variable degrees of remission may be achieved with chemotherapy. FDCS and IDDCS are rare neoplasms that may pose difficulty in pathologic diagnosis. IDDCS seems to display a more aggressive behavior than FDCS. Patients with IDDCS and FDCS can eventually die of disease progression. The role of chemotherapy and radiotherapy is not clearly defined.

Dendritic Cells↗

Anemia after orchiectomy.

The decrease in testosterone production associated with bilateral orchiectomy may result in normocytic anemia in men. We sought to determine the effect of orchiectomy on hemoglobin concentration. Patients were evaluated at the Mayo Clinic in 1993 and 1994 and underwent bilateral orchiectomy for prostate carcinoma. All patients were seen by one of the staff urologists. Patients were included if they had a normal preoperative complete blood cell count and serum levels of creatinine, if they remained without disease progression (suppressed prostate-specific antigen level and no evidence of clinical progression on review), and if they had normal serum levels of creatinine and mean corpuscular volume during the follow-up period. The patients could have no other cause of anemia. The complete blood cell count, prostate-specific antigen level, and serum level of creatinine were determined preoperatively and at least once (>90 days) after orchiectomy. Sixty-four patients were included in the analysis (median age, 68 years). The median decrease in hemoglobin concentration was 1.2 g/dL after orchiectomy. There was a statistically significant difference in the hemoglobin concentration before orchiectomy compared with postoperative values at all the intervals studied, both by the paired group t-test and the Kruskal-Wallis test. There is a clinically and statistically significant decrease in hemoglobin concentration after orchiectomy. Knowledge of this phenomenon may prevent unnecessary diagnostic work-up in men with normocytic anemia after bilateral orchiectomy.

Aged↗

Surgical treatment of extensive skin necrosis secondary to purpura fulminans in a patient with meningococcal sepsis.

Meningococcal sepsis is associated with a high mortality rate. These patients may show severe disseminated intravascular coagulation (DIC) and skin necrosis. There is very little published experience regarding the surgical treatment of this complication. The similarity between skin necrosis secondary to DIC and full thickness cutaneous burns provides the rationale for its treatment as if it was a deep burn. We report the surgical treatment of extensive skin necrosis in a patient with meningococcal sepsis and DIC. This treatment is similar to that used in full thickness burns, including excision of necrotic tissue and coverage with autografts, as well as amputation of extremities if distal coverage is not possible.

Adult↗

Multiple myeloma and the translocation t(11;14)(q13;q32): a report on 13 cases.

Complex cytogenetic abnormalities have been described in patients with multiple myeloma (MM). To better understand the significance of the most frequent translocation observed in MM, we studied the clinical characteristics of patients with MM and the t(11;14)(q13;q32) abnormality. A search of the cytogenetic database at the Mayo Clinic identified patients with MM and t(11;14)(q13;q32). The medical records were reviewed for the clinical characteristics of these patients. We identified 13 patients with MM and t(11;14)(q13;q32) determined by standard cytogenetic analysis: in 10 patients the abnormality was detected at the time of relapse (three with previously normal results of cytogenetic examination). At the time the translocation was detected, plasma cell (PC) leukaemia was clinically diagnosed in two patients. The median number of circulating PCs, as determined by the cytoplasmic immunofluorescence of T-cell-depleted peripheral blood mononuclear cells, was 1.1x10(9)/l (mean 1.74; range 0.0017-6.26x10(9)/l). On linear regression analysis there was a strong correlation between the number of circulating PCs and the number of bone marrow PCs. The median survival after demonstration of the translocation was 8.1 months. Of all patients, 10 died of disease progression and three were alive. Patients with MM who have t(11;14)(q13;q32) seem to have an aggressive clinical course, even when the abnormality is detected at the time of diagnosis, with evidence of many circulating PCs.

Adult↗

Chromosomal abnormalities in systemic amyloidosis.

Primary systemic amyloidosis (AL) is a plasma cell disorder characterized by deposition of monoclonal light chains in different organ systems. Although multiple and complex numerical chromosomal abnormalities have been described in patients with multiple myeloma, it is currently unknown whether such changes occur in systemic amyloidosis. Bone marrow samples from 21 patients with AL were studied by standard cytogenetics and interphase fluorescence in situ hybridization (FISH) for the presence of numerical chromosomal abnormalities. We tested for six chromosomes (7, 11, 9, 15, 18 and X) using centromere-specific probes. The monoclonal plasma cells were identified by simultaneous fluorescent staining of the monotypic cytoplasmic immunoglobulin. We compared these results with those obtained from 19 patients with monoclonal gammopathy of undetermined significance (MGUS) and normal controls. Multiple numerical chromosomal abnormalities were detected in AL by interphase FISH, including trisomy of chromosomes 7 (42%), 9 (52%), 11 (47%), 15 (39%), 18 (33%) and X (13% in women and 54% in men). Monosomy of chromosome 18 was seen in 72% of cases. Previous exposure to alkylator therapy did not appear to correlate with these abnormalities. No significant difference was observed in the prevalence of these abnormalities between AL and MGUS. Multiple chromosomal numerical abnormalities were detected by interphase FISH analysis in patients with AL, especially monosomy of chromosome 18. Aneuploidy in the monotypic plasma supports a neoplastic nature for the disorder.

Adult↗

Pilot evaluation of venlafaxine hydrochloride for the therapy of hot flashes in cancer survivors.

PURPOSE: Hot flashes can be a prominent clinical problem for breast cancer survivors and men who undergo androgen-deprivation therapy. Anecdotal information suggested a low dose of a relatively new antidepressant, venlafaxine, could abrogate this clinical problem. MATERIALS AND METHODS: This study included 28 consecutive assessable patients entered onto a phase II clinical trial. Hot flash data were collected by daily diary questionnaires during a 1-week baseline period and then for 4 weeks, during which time patients received venlafaxine 12.5 mg orally twice daily. RESULTS: Fifty-eight percent of patients who completed the study had a greater than 50% reduction in hot flash scores (frequency times severity) during the fourth treatment week as compared with the baseline week. Median weekly hot flash scores were reduced by 55% from baseline during the fourth week of venlafaxine therapy. Therapy was generally well tolerated and appeared to alleviate fatigue, sweating, and trouble sleeping. CONCLUSION: Venlafoxine appears to represent an efficacious new method to alleviate hot flashes. Further evaluation of this compound for alleviating hot flashes is indicated.

Adult↗

Disseminated Kaposi's sarcoma after heart transplantation: association with Kaposi's sarcoma-associated herpesvirus.

BACKGROUND: Kaposi's sarcoma is an endothelial tumor rarely diagnosed after heart transplantation. We report on a patient originally from Africa in whom Kaposi's sarcoma developed in association with Kaposi's sarcoma-associated herpesvirus. METHODS: A man from Ghana had constitutional symptoms associated with multiple pulmonary nodules that developed 3 months after heart transplantation. Kaposi's sarcoma was diagnosed by thorascopic biopsy. Treatment consisted of reducing immunosuppression therapy and adding famciclovir treatment. Symptoms resolved within 1 month after treatment, and no disease progression was observed for 5 months. The patient died suddenly 8 months after heart transplantation; autopsy revealed occlusion of the left anterior descending coronary artery and grade 3A rejection. Extensive Kaposi's sarcoma was observed in the lungs and gastrointestinal tract at autopsy. RESULTS: Pathologic analysis of the tumor demonstrated features consistent with Kaposi's sarcoma. Polymerase chain reaction and in situ hybridization demonstrated the presence of Kaposi's sarcoma-associated herpesvirus. CONCLUSION: Kaposi's sarcoma rarely is diagnosed after transplantation. Patients infected with Kaposi's sarcoma-associated herpesvirus may be at increased risk. Screening for Kaposi's sarcoma-associated herpesvirus may be indicated in patients at risk. The role of antiviral medications and immunization in the treatment and prevention of the disorder is unknown.

2-Aminopurine↗

Ductal carcinoma in situ of the breast.

PURPOSE: The increasing incidence and biological heterogeneity of ductal carcinoma in situ (DCIS) of the breast have made the management of this entity challenging and controversial. This paper reviews data on the natural history of the disease and results obtained with various management approaches. DATA SOURCES: Computerized MEDLINE search of articles related to DCIS published since 1966. STUDY SELECTION: Randomized trials were given higher value; however, because these were relatively scarce, retrospective studies and data published in abstract form were also included. DATA EXTRACTION: The authors reviewed all sources critically. No formal statistical calculations were made. DATA SYNTHESIS: The incidence of DCIS is increasing, and a greater proportion of diagnoses are being made in asymptomatic patients. No data from randomized trials compare mastectomy and breast-conserving therapy for the treatment of DCIS. A large randomized trial comparing lumpectomy with lumpectomy plus radiotherapy showed lumpectomy plus radiotherapy to be effective for management of this disease. The presence of comedo necrosis and surgical margin status are frequently used as predictors of subsequent recurrence, although this practice is controversial. The risk for in-breast recurrence at 5 years after lumpectomy and radiotherapy is approximately 8%. With more refined molecular analysis, the relation of DCIS to invasive breast cancer will be better defined. CONCLUSIONS: Treatment strategies for DCIS have evolved, and lumpectomy followed by radiotherapy is an appropriate alternative for most patients. The use of lumpectomy alone in selected patients remains controversial.

Breast Neoplasms↗

Follicular dendritic cell sarcoma mimicking diffuse large cell lymphoma: a case report.

Follicular dendritic cell sarcomas (FDCSs) are rare tumors arising from follicular dendritic cells in lymphoid tissue. Fewer than 20 cases have been described in the English-language literature. We describe the second case of an FDCS with primary liver involvement. The initial diagnosis was lymphoma, and appropriate treatment was prescribed. After the initial treatment failed, additional biopsy samples were obtained. Standard pathologic analysis and immunophenotyping for a panel of monoclonal antibodies were performed on formalin-fixed paraffin-embedded tissue and frozen sections. The pathologic findings were consistent with FDCS, and the specimens showed some of the characteristic pathologic features suggestive of this tumor, including multinucleation and a spindle pleomorphic morphology. The tumor cells were positive for S-100 protein, CD45, CD14, and vimentin. Because of its morphological characteristics, FDCS can be confused with other neoplastic entities, such as lymphomas and other solid tumors.

Antigens, CD↗

Practical aspects in the diagnosis and management of aplastic anemia.

Aplastic anemia may result from several pathogenic mechanisms, the most common is idiopathic. The current definitive treatments for aplastic anemia are bone marrow transplantation (BMT) or immunosuppressive (IS) therapy. The benefits of each are comparable. However, certain subsets of patients derive superior benefit from one or the other. Bone marrow transplantation is the initial treatment of choice for young patients (< 20 years old). It results in the complete reconstitution of hematopoiesis, whereas autologous hematopoietic remissions after IS therapy are more susceptible to relapse. Survival rates after BMT, in patients between the ages of 20 and 40, are comparable to those reported for IS therapy. Better survival rates after BMT have been achieved with improved conditioning regimens and graft-versus-host disease prophylaxis. For patients older than 40, the treatment of choice is IS. Long-term complications of IS therapy include recurrence and development of clonal myeloid disorders. Long-term complications after BMT include graft-versus-host disease and secondary neoplasms. The IS regimen includes the combination of antithymocyte globulin and cyclosporin A. The addition of growth factor to the IS regimen seems promising; however, their use on their own is not recommended. Androgens have been shown to be inferior in the treatment of aplastic anemia. The role of BMT from an unrelated donor is being investigated.

Anemia, Aplastic↗

[Validity of routine serum calcium measurements].

BACKGROUND: The frequency of diagnosis of primary hyperparathyroidism increased dramatically with the advent of routine serum calcium measurements. The reliability of colorimetric serum calcium measurements is not well known. AIM: To know the frequency of hyper and hypocalcemia in a population of healthy people and the reliability of serum calcium measurements. SUBJECTS AND METHODS: Serum calcium was measured in 731 subjects participating in a preventive medical examination, using an automated colorimetric method. Serum albumin was also measured. In 31 randomly chosen serum samples, calcium was also measured by atomic absorption spectrometry. Normal serum calcium ranges were established as the mean +/- 1.34 SD of the sample. RESULTS: Mean serum calcium was 9 +/- 0.7 mg/dl, serum albumin was 4.3 +/- 0.5 g/dl and albumin corrected calcium levels were 8.7 +/- 0.7 mg/dl. Excluding lipemic sera, colorimetric calcium measurement had a correlation of 0.6 with atomic absorption spectrometry and a reliability of 17.9%. Using the proposed normal ranges (7.3-10.6 mg/dl), the frequency of hypercalcemia and hypocalcemia was 0.14 and 0.4% respectively. CONCLUSIONS: Most subjects found to have hyper or hypocalcemia in this study were asymptomatic.

Adolescent↗