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F Zaja

Publications and source records attributed to F Zaja.

At least 37 records · Page 2Linked to original sources
PubMed1999

Nongastrointestinal low-grade mucosa-associated lymphoid tissue lymphoma: analysis of 75 patients.

PURPOSE: Nongastrointestinal locations represent about 30% to 40% of all low-grade mucosa-associated lymphoid tissue (MALT) lymphomas. We report a retrospective analysis of 75 patients with nongastrointestinal low-grade MALT lymphoma, presenting their clinical, therapeutic, and follow-up data with respect to the initial location of the lymphoma. PATIENTS AND METHODS: From January 1988 to October 1997, 75 patients with untreated nongastrointestinal low-grade MALT lymphoma were subjected to treatments ranging from local radiotherapy and local interferon alfa administration to chemotherapy. The lymphomas were located in the lung (19 patients), orbital soft tissue (16 patients), skin (seven patients), thyroid (seven patients), lachrymal gland (six patients), conjunctiva (six patients), salivary gland (six patients), breast (three patients), eyelid (two patients), larynx (one patient), bone marrow (one patient), and trachea (one patient). RESULTS: Complete and partial remissions were achieved in 59 (79%) and 16 (21%) of the 75 patients, respectively, with an overall response rate of 100%. All but two of the patients are still alive, with a median follow-up of 47 months; these two patients died from other causes. The estimated time to treatment failure rate is 30% at 5 years. In the thyroid and lachrymal gland sites, no relapses were reported. CONCLUSION: Our data regarding the largest reported series of nongastrointestinal MALT lymphomas confirm the good prognosis of this particular clinicopathologic entity and the significant efficacy of different therapeutic approaches to specific sites.

Adult↗
PubMed1999

Clinical features and outcome of multiple myeloma arising from the transformation of a monoclonal gammapathy of undetermined significance.

Patients with a monoclonal gammapathy of undetermined significance (MGUS) are usually submitted to a periodical clinical follow-up, but it is not known if this surveillance can ameliorate the prognosis of a plasma cell malignancy that will be eventually detected. We compared the clinical and laboratory characteristics at onset, the response to chemotherapy and the survival, of 21 cases of newly diagnosed multiple myeloma (MM) arising from the malignant transformation of MGUS and 41 cases without a previous history of MGUS, recruited to the same first-line treatments over a 3-years period. The former group showed a significant lower frequency of advanced stages as well as other several prognostic factors of high risk including anemia, renal failure, bone lesions and increase of beta2 microglobulin and C-reactive protein levels. Despite a similar response to treatment of the two groups, MM arising from MGUS showed a significantly longer median survival than MM without prior MGUS. This was particularly true for stage I, while stages II and III behaved similarly. We conclude that the regular clinical monitoring of MGUS patients allowed the identification of earlier malignant transformation, when tumor burden is lower, as indicated by lower beta2 microglobulin levels and marrow plasmacytosis of stage I MM arising from MGUS. Moreover, a slower proliferation rate of myeloma cells, as suggested by lower C-reactive protein levels, may be considered so as to explain the longer survival of these patients.

Aged↗
PubMed1999

The therapy of primary adult systemic CD30-positive anaplastic large cell lymphoma: results of 40 cases treated in a single center.

The outcome of a series of adult patients, affected by primary systemic CD30-positive anaplastic large cell lymphoma (ALCL), treated with a sequential intensive therapeutic program, has been analyzed and all data available in the literature have been reviewed. Forty consecutive, unselected patients with ALCL were treated with the F-MACHOP regimen, followed by radiotherapy (RT) for residual mediastinal disease (15 cases) and by autologous stem cell transplantation (ASCT) conditioned with BAVC (29 cases). Eighty-nine percent (32/36) of the patients younger than 60 years were eligible for completing the sequential treatment. Since then, 3 patients in CR refused ASCT, 1 was excluded for cardiac toxicity and 3 progressed and died of disease. Thus, 29 have been so far submitted to the transplant procedure. CR and PR rates were 40% and 45% respectively after CHT; 52.5% and 35% after RT; 80% and 5% after ASCT, with 78% of patients transplanted in PR convertin to a CR. Actuarial overall survival is 85% at 48.5 months (93% at 66 months for the 29 transplanted patients) and disease free survival is 100% at 54 and 64 months respectively, with no relapses observed among patients who reached a CR. Considering our data and those of the literature, it can be concluded that although the role of ASCT in the therapy of ALCL must not be considered as definitive, its efficacy in converting PR into CR and in preventing relapses, suggests that a randomized trial comparing CHT alone vs CHT+ASCT should be undertaken.

Adolescent↗
PubMed1999

Splenectomy in hematology. Current practice and new perspectives.

BACKGROUND AND OBJECTIVE: Progress and changes in the management of blood diseases, in surgery and in video technology stimulate a critical reappraisal of splenectomy in hematology. DESIGN AND METHODS: We have collected information on the current practice of splenectomy in hematology in Italy and we have reviewed the results of a new technique of laparoscopic splenectomy (LS). RESULTS: Current splenectomy practice: the current practice in Italy is to offer splenectomy as front-line treatment for hereditary spherocytosis and as second-line for idiopathic thrombocytopenic purpura (ITP) and hemolytic anemia. Splenectomy is also offered in selected cases of leukemia and lymphoma but is going out of practice for hairy cell leukemia and Hodgkin's disease. The number of splenectomies that are performed every year is estimated to be higher than 10x10(6) persons (more than 500 cases per year). Laparoscopic splenectomy (LS): more than 700 cases of LS have been reported so far, for thrombocytopenia (470 cases) as well as for many other hematologic indications. The procedure carries a mortality of 0.8%, and a complication rate of 12%. Time spent in the operating theater ranges from 1.5 to 4 hours, blood transfusion requirement is minimal and the mean post-operative hospital stay is 3 days. INTERPRETATION AND CONCLUSIONS: Although a prospective comparison is not available, the results of LS compare favorably with the results of classic open splenectomy, so that LS is likely to become the technique of choice especially when the spleen is small, as in ITP. LS can also have some advantages in other cases of splenectomy, including splenomegaly for leukemia and lymphoma. These data and suggestions should stimulate and renew a discussion about splenectomy in hematology, with the purpose of establishing evidence-based guidelines.

Hematologic Diseases↗
PubMed1999

How do patients with aggressive non-Hodgkin's lymphoma treated with third-generation regimens (MACOP-B and F-MACHOP) fare in the long-term?

BACKGROUND AND OBJECTIVE: To examine the long-term clinical course and prognostic factors of patients with advanced aggressive non-Hodgkin's lymphoma (NHL) treated with third-generation regimens as front-line chemotherapy. DESIGN AND METHODS: A total of 348 patients aged <60 years received MACOP-B or F-MACHOP regimen between September 1988 and August 1993 for advanced stage aggressive NHL. RESULTS: Of these, 249 (71.5%) obtained a complete response (CR); 65/249 (26%) subsequently relapsed. The CR rates for MACOP-B and F-MACHOP were 70.5% and 72%, respectively, while the relapse-free survival rates (RFS) at 9 years were 66% and 74%, respectively. The overall survival rate at 9 years was 61% for MACOP-B and 67% for F-MACHOP patients. Of the relapses, 78.5% were early (<24 months) and 21.5% late. Eleven out of 65 (17%) patients are in continuous second CR with a median follow-up of 45 months; most of them have been salvaged with high-dose therapies. The validity of the International Prognostic Index was confirmed in long-term analysis. INTERPRETATION AND CONCLUSIONS: With a 9-year RFS, it is possible to consider cured 50% of the patient with aggressive NHL treated with a third-generation regimen. About a quarter of the CRs relapse and late relapse occurs in roughly 20% of all relapsed patients. In these patients high-dose chemotherapy followed by autologous bone marrow or hematopoietic stem cell transplantation seems to be a very reliable approach in terms of long-term second CR. Finally, the IPI score maintains its pivotal role in terms of stratifying patients according to different risk subsets also in long-term analysis.

Adolescent↗
PubMed1998

Impact of hepatitis C virus infection on clinical features, quality of life and survival of patients with lymphoplasmacytoid lymphoma/immunocytoma.

BACKGROUND: The non-Hodgkin's lymphoma (NHL) subgroup most frequently associated with hepatitis C virus (HCV) infection is the lymphoplasmacytoid lymphoma/immunocytoma (Lp-Ic). We have assessed the impact of the infection on the clinical features, quality of life and survival of HCV+ve Lp-Ic patients as compared to its impact in HCV-ve patients. PATIENTS AND METHODS: Seventy patients with Lp-Ic consecutively observed over a six-year period were studied. Clinical, virological and histopathological features were recorded at diagnosis. Quality of life was assessed using a scoring system including disease-related symptoms, performance status, working ability, hospital admissions and therapies required. RESULTS: Eighteen patients (26%) with HCV infection were identified. Significant differences between those patients and the HCV-ve group included number of symptomatic patients, Hb levels, serum protein levels, entity of the IgM monoclonal component, number of patients with cryoglobulins and with organ (liver, kidney) involvement, and entity and pattern of bone marrow infiltration. Survival rates were similar (P = 0.8383), but the quality-of-life score was significantly worse for the HCV+ve patients (P = 0.002). All anti-HCV Ab+ve patients tested positive for HCV RNA; genotype 2ac was detected in a significant proportion of cases. CONCLUSIONS: This study confirms that HCV infection is present in about one-third of patients with Lp-Ic. HCV infection does not seem to affect the overall survival of patients with Lp-Ic, but it affects the clinical expression of the disease, so that the overall quality of life of HCV+ve patients is significantly worse.

Adult↗
PubMed1998

Adjuvant treatment with cyclosporin A increases the toxicity of chemotherapy for remission induction in acute non-lymphocytic leukemia.

P-glycoprotein (Pgp)-related multidrug resistance (MDR) is frequently observed in acute non-lymphocytic leukemia (ANLL) and is associated with a poor response to standard chemotherapy. Cyclosporin A (CsA) is an effective downmodulator of Pgp-related MDR in vitro and has already been tested for that purpose in vivo also. Since Pgp is expressed in several normal cells and tissues, the modulation of Pgp can also modify total body exposure to antileukemic drugs and can alter and increase the toxicity of the antileukemic treatment. We report here the results of a study where 46 consecutive adult patients with ANLL were assigned to receive the same standard chemotherapy regimen of arabinosyl cytosine and idarubicin (IDA) for remission induction or consolidation, without or with CsA. Twenty-eight patients received 36 courses of chemotherapy without CsA and 18 patients received 32 courses of chemotherapy with CsA. CsA dose was 10-12.5 mg/kg/day and was given as a continuous i.v. infusion for 72 h. Whole blood CsA steady-state concentration ranged between 0.61 and 1.14 microM. The IDA area-under-the-curve was about twice as high in the cases that received CsA than in the other cases. CsA had no detectable effects on renal function and fluid balance, but significantly increased systemic blood diastolic pressure and conjugated bilirubine concentration. Furthermore, CsA-treated patients had greater, and more severe, oral and intestinal mucosal toxicity, with more severe adverse events, including more cases of gram-negative bacteremia, and with a delayed hemopoietic recovery. In conclusion, this study showed that an attempt at an effective downmodulation of Pgp-mediated MDR would substantially increase the hemopoietic and mucosal toxicity of antileukemic treatment and that the increase is accounted for, at least in part, by an increase of total body exposure to IDA.

Adult↗
PubMed1998

BCL-2 immunohistochemical evaluation in B-cell chronic lymphocytic leukemia and hairy cell leukemia before treatment with fludarabine and 2-chloro-deoxy-adenosine.

Bcl-2 overexpression has been shown to be associated with several malignancies, including B-cell chronic lymphocytic leukemia (CLL) and non-Hodgkin's lymphomas (NHL), mainly low-grade and follicular in type. It has as yet not been described in hairy cell leukemia (HCL). In 30 patients with CLL and 14 with HCL who were consecutively selected for treatment with purine analogues (Fludarabine in CLL and 2-chloro-deoxy-adenosine in HCL), we evaluated bcl-2 oncoprotein expression in leukemic cells on marrow sections that were taken before treatment and stained immunohistochemically with a monoclonal antibody (Dakopatts 124 clone), by the avidin-biotin-peroxidase method. All samples were found to be bcl-2 positive, with a staining intensity that was moderate to strong in CLL and weak to moderate in HCL. 83% of CLL and 100% of HCL patients were responsive to purine analogues. These findings show that bcl-2 is overexpressed in almost all cases CLL and HCL and that bcl-2 overexpression does not predict a poor response to purine analogues, which are believed to induce apoptosis.

2-Chloroadenosine↗
PubMed1998

Fludarabine and cytosine-arabinoside for poor-risk acute myeloid leukemia.

Thirteen relapsed or refractory AML patients were treated with the FLA regimen. A complete remission was observed in 54% of cases but the median duration of remission was short (4 months). These results suggest that the FLA regimen is not able to induce a durable complete remission in the poor-risk AML patients.

Acute Disease↗
PubMed1997

The genotype of the hepatitis C virus in patients with HCV-related B cell non-Hodgkin's lymphoma.

Increasing evidence suggests that the hepatitis C virus (HCV) might be involved in the pathogenesis of B cell non-Hodgkin's lymphomas (NHL). Since several HCV genotypes are currently identifiable and might be involved in the pathogenesis of different diseases (with different severity and responsiveness to therapy), the aim of our study was to assess the prevalence of viral genotypes in a group of patients with HCV-related NHL. Among 470 consecutive patients, 42 HCV Ab-positive cases were identified. HCV RNA could be detected by reverse transcriptase-polymerase chain reaction and genotyping performed in 31 of these cases. As compared to our control group (211 healthy blood donors and patients with chronic liver disease), a striking high prevalence of genotype 2ac was detected among B cell NHL (48.4 vs 9.0%), with a relative risk of infection of 5.37 (P < 0.0001). No major differences were observed in the distribution of NHL histotypes and in the clinical features among patients with genotype 1b (the other most frequent genotype) or 2ac, a part from a trend towards a higher percentage of liver disease and a lower likelihood of response to interferon for patients with genotype 1b. The same high prevalence of genotype 2ac has been recently reported in patients with mixed cryoglobulinemia (MC), monoclonal gammopathies, B cell NHL complicating MC and autoimmune hepatitis. All these data taken together suggest that genotype 2ac might be involved in the pathogenesis of lymphoproliferative and autoimmune disorders.

Adult↗
PubMed1997

Sequential intensive treatment with the F-MACHOP regimen (+/- radiotherapy) and autologous stem cell transplantation for primary systemic CD30 (Ki-1)--positive anaplastic large cell lymphoma in adults.

Most of the adult patients with primary systemic CD30 (Ki-1)-positive anaplastic large cell lymphoma (ALCL) reported in the literature have been treated with combination chemotherapy (CHT), with only an occasional patient being autotransplanted, mostly after relapse. Here we report a series of 19 patients treated at our Institution with a sequential intensive therapeutic program including CHT (the F-MACHOP regimen given for 6 cycles), radiotherapy (RT), and autologous stem cell transplantation (ASCT) after conditioning with the BAVC regimen. Eleven of 19 (58%) patients achieved a complete remission (CR) after CHT; 3 after RT and 4 after ASCT (1 patient is still not evaluable for response to ASCT). The actuarial overall survival is 100% at a median of 49 months from diagnosis and the actuarial disease free survival is 100% at a median of 41 months from the time CR was reached. These data suggest that ALCL can be successfully managed with a sequential intensive treatment that prevents early relapses and projects these patients as long-term survivors.

Adolescent↗
PubMed1997

Myasthenia gravis after allogeneic bone marrow transplantation. A case report and a review of the literature.

A 26-year-old man, affected by acute non-lymphocytic leukemia, received a bone marrow transplantation from his HLA identical sister. A mild (grade 1) acute graft-versus-host disease (GVHD) developed during the first month after BMT. A moderate (grade 2) muco-cutaneous GVHD was present from the 4th month on, in association with recurrent oral herpes simplex infection. A typical clinical and electromyographic picture of myasthenia gravis (MG) developed 46 months after BMT, requiring continuous medication with pyridostigmine. A high titer of antibiotics against the acetylcholine receptor was found in the serum of the patient but not in the serum of the donor. Since the donor had no evidence of MG or other autoimmune disorders, this is likely to be an autoimmune complication of chronic GVHD. Other cases described in the literature are reviewed: a recurrent expression or coexpression of some HLA antigens was recorded, indicating that some genetic factors could predispose to this acquired disorder.

Adult↗
PubMed1996

Prevalence of hepatitis C virus infection in patients with lymphoproliferative disorders.

It has been recently hypothesized that the hepatitis C virus (HCV) might be involved in the pathogenesis of malignant B-cell non-Hodgkin's lymphomas (NHL). On the basis of this observation we sought to determine the prevalence of HCV infection in the patients affected by B-cell NHL and extended our analysis to all the patients affected by lymphoproliferation disorders seen at our institution in the last 30 months. Five hundred and thirty-seven unselected, consecutive patients were studied. HCV infection was investigated through detection of anti-HCV antibodies and HCV-RNA. HCV genotyping was performed on HCV-RNA positive specimens. The risk of being infected by HCV was compared with that of the general population of our area. Among all lymphoproliferative disorders, the prevalence and the relative risk (RR) of being infected by HCV were increased only among B-cell NHL (9%; RR 3.24; p < .0001). Among these, a strong prevalence of HCV was found only in the subgroup of immunocytomas (30%; RR 10.27; P < .0001), while other histotypes were associated with it only occasionally. Because HCV-positive lymphomas clinically behave as essential mixed cryoglobulinemia (EMC), the close association between HCV infection and EMC is confirmed, and evidence is provided that the pathological substrate of EMC corresponds to the immunocytoma. HCV genomic sequences were found in 84% of patients analyzed. Viral genotypes were those more frequent in our area.

Adult↗