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Biomedical subjects

I Ben-Bassat

Publications and source records attributed to I Ben-Bassat.

At least 55 records · Page 3Linked to original sources
PubMed1995

A controlled trial of tranexamic acid therapy for the reduction of bleeding during treatment of acute myeloid leukemia.

In order to determine the efficacy of the antifibrinolytic agent tranexamic acid (TA) in reducing bleeding and platelet transfusions during the treatment of acute myeloid leukemia (AML), we conducted a randomized placebo-controlled double-blind study. Patients with AML undergoing induction or postremission consolidation chemotherapy were randomized into TA or placebo groups. Patients were not given platelet transfusions prophylactically but only when bleeding occurred. The severity of any bleeding event was scored. Thirty eight patients were randomized during induction. There were no significant differences between the two groups in the number of bleeding events and their severity or in the number of platelet transfusions given. Eighteen patients were studied during consolidation. In contrast, to the induction period, during consolidation there was a significantly less severe bleeding tendency in the TA group resulting in a lower platelet transfusion requirement [3.7 +/- 4.1 vs. 9.3 +/- 3.3 platelet units (p < .05)]. TA was well tolerated and no side effects were seen and no specific thromboembolic events were noticed. We conclude that giving TA during the thrombocytopenic period of AML patients undergoing consolidation chemotherapy is beneficial and safely reduces platelet transfusions.

Acute Disease↗
PubMed1994

The cyclophosphamide, vincristine, prednisone, bleomycin, doxorubicin, and procarbazine (COPBLAM-I) regimen for intermediate-grade non-Hodgkin's lymphoma. Long term follow-up in 51 patients.

BACKGROUND: Cyclophosphamide, vincristine, prednisone, bleomycin, doxorubicin, and procarbazine (COPBLAM-I) is a second generation combination chemotherapy for intermediate-grade non-Hodgkin's lymphoma (NHL). Since the first report by Laurence et al. in 1982, only a few series were reported on the long term results of this regimen. METHODS: In this prospective study, the clinical courses of 51 patients with intermediate-grade NHL (F, G, or H according to the International Working Formulation grading criteria) with a median age of 65 years (range, 55-81), who were diagnosed between 1983 and 1992, are reported. Eligibility criteria included at least one full cycle of the regimen and no previous chemotherapy. RESULTS: A mean of seven cycles (range, two to nine) with two escalations (range, zero to six) was administered. In a median follow-up of 2.4 years (range, 0.5-10), 33 patients (65%) experienced complete remission. The projected 3-year overall survival (OS) and progression free survival rates (PFS) were 58% and 77%, respectively, and the projected 5-year OS and PFS were 52% and 72%, respectively. In only 2 patients (4%) were treatment-related deaths observed, whereas nonfatal complications were more common. Patients in the low risk group, according to the international NHL prognostic index, had the best outcome. The mean percentage of projected dose intensity (DI) was 95% (range, 77-109), whereas no difference in DI was found between patients younger or older than 65 years. CONCLUSIONS: In a single center, COPBLAM-I combination chemotherapy was safe and effective for patients older than 55 years with intermediate-grade NHL.

Aged↗
PubMed1994

Acute leukemia relapse presenting as central diabetes insipidus.

BACKGROUND: Diabetes insipidus due to leukemic involvement of the central nervous system has been described previously in several patients. Although the timing of the diabetes insipidus onset in relation to the leukemia is variable, it has not been described previously as the first manifestation of leukemia relapse. METHODS: Two cases of leukemia relapse presenting as central diabetes insipidus are described and the literature is reviewed. RESULTS: Clinical data of 29 of the 39 reported cases were available. The median age was 46 years, with no gender predominance. Seventy-three percent had acute nonlymphocytic leukemia, and deletion of chromosome 7 or monosomy 7 was the predominant cytogenetic abnormality. No temporal relation between the onset of diabetes insipidus and the leukemia was found. In none of the previously reported cases was diabetes insipidus the first manifestation of the leukemia recurrence. CONCLUSIONS: The temporal relationship between diabetes insipidus and leukemia is inconsistent. The former may precede, follow, or present concomitantly with the latter. Two cases of diabetes insipidus as the presenting sign of leukemia relapse are reported.

Adult↗
PubMed1994

CD10+ cell population in the bone marrow of patients with advanced neuroblastoma.

Immunocytologic analyses of bone marrow can provide clinically useful prognostic information in neuroblastoma. While analyzing the bone marrow with a panel of monoclonal antibodies, which detect neuroblasts and other defining B-, T-, and myloid lineage, we identified two infants with stage IV-S neuroblastoma whose bone marrow contained a large population of common acute lymphoblastic leukemia (ALL)-like cells. This population expressed HLA-DR, CD19(B1), CD10(CALLA), and occasionally CD20(B1). Since 1988, 17 additional patients with advanced neuroblastoma (IV-S, III, and IV) were studied by us. In 10 of the 19 patients, the bone marrow revealed an expanded CD10 population (20-70%). It appears that this group of patients has a better prognosis. Out of 9 patients who did not have an expanded CD10 population, 8 died within 9 months from diagnosis, whereas out of 10 patients with an expanded CD10 population only one died and the others are alive, 6-30 months from diagnosis (P < 0.001). An expanded CD10 population in the bone marrow of disseminated neuroblastoma patients may therefore serve as a prognostic factor. Apart from the prognostic value of this particular population in the single patient, its presence may shed light on the interrelationship between the immune system and the neuroendocrine compartment.

Adolescent↗
PubMed1994

Hormonal treatment in pregnancy: a possible risk factor for neuroblastoma.

In the last 4 years, 24 cases of neuroblastoma were treated in the Pediatric Hematology-Oncology Unit at the Chaim Sheba Medical Center, 8 of whom were under 1 year of age. Four of them were the product of a pregnancy-induced or preserved by gonadotropins, clomiphene citrate, or progestational hormones. These drugs are known to produce a higher than normal level of estradiol or progesterone in the early stages of pregnancy. Our observation led to the hypothesis that high levels of progestational hormones given during pregnancy are a risk factor for neuroblastoma in infancy.

20-alpha-Dihydroprogesterone↗
PubMed1994

Congenital haemolytic anaemia associated with adenylate kinase deficiency.

Chronic haemolytic anaemia associated with adenylate kinase (AK) deficiency is very rare and only seven cases in five families have been described. We present six children of one family who are deficient of this enzyme and in three of them a combined G6PD deficiency was found. AK deficiency was transmitted by an autosomal recessive gene and heterozygous state was not accompanied by disease, whereas homozygously affected individuals present a congenital chronic non-spherocytic haemolytic anaemia with haemoglobin levels of 8-9 g/dl. Patients also deficient in G6PD suffer from a more severe haemolytic anaemia with haemoglobin levels around 6 g/dl. The AK-deficient children are also mentally retarded. Splenectomy performed in five of the six patients resulted in complete remission of the haemolytic process.

Adenylate Kinase↗
PubMed1994

Huge cutaneous plasmacytomas complicating multiple myeloma.

The case of a 46-year-old woman with multiple myeloma and extramedullary dissemination to the urinary bladder and skin is described. Cutaneous plasmacytomas were scattered as firm, violaceous-red, lesions of variable diameter (2-13 cm) which were extremely tender. Despite combined chemotherapy and half-body irradiation, the lesions progressed until the patient died 8 months following diagnosis.

Arm↗
PubMed1994

Familial leukemia: description of two kindreds and a review of the genetic aspects of the disease.

We describe two kindreds of Arab ancestry characterized by multiple cases of acute lymphoblastic leukemia. Consanguinity and intermarriages were prevalent in the two families. Age, mode of presentation, characteristics of the leukemic cells, response to treatment and prognosis were remarkably similar among the patients. A short review of the literature on familial leukemia is given.

Child↗
PubMed1994

Biased representation of immunoglobulin heavy chain variable region subgroups in chronic lymphocytic leukemia.

The distribution of three immunoglobulin heavy chain variable region gene subgroups (VH1, 3 and 5) was investigated, using the polymerase chain reaction technique, in 53 patients with chronic lymphocytic leukemia. Thirty-seven patients displayed rearrangements of one of the above three gene segments. Over-representation of the VH5 subgroup was observed relative to its small size.

Antigens, CD↗
PubMed1994

In vivo clonal evolution of pre-B to B-cell acute lymphoblastic leukemia in childhood.

Two cases are described that provide further evidence for clonal evolution in pre-B-cell acute lymphoblastic leukemia. Two infants, whose lymphoblasts at diagnosis were morphologically subtyped as L1 and immunophenotyped as HLA DR+, CD19+, CD10+/- and C mu-, were induced and maintained in remission. One child relapsed 6 months after initiation of therapy. This time his lymphoblasts had L3 morphology and immunophenotyping demonstrated the appearance of surface immunoglobulins. The second child relapsed 18 months after initiation of therapy with a lymphomatous picture. He also had peripheral and bone marrow blasts with L3 morphology and surface immunoglobulins. A lymph node biopsy showed diffuse small non-cleaved lymphoma with a 'starry sky' appearance compatible with Burkitt's lymphoma. Only one case with a similar clonal evolution has been reported in the literature, but no surface immunoglobulins were demonstrated. The significance of clonal evolution in these cases and its potential practical implications are discussed.

Clone Cells↗
PubMed1993

[Biclonal gammopathies: clinical and theoretical aspects].

Monoclonal gammopathies are disorders in which a clone of lymphocytes arising from a common ancestor cell proliferates and secretes a monoclonal antibody. Electrophoresis of plasma proteins is used as a diagnostic test for these disorders. It produces a narrow peak within the zone of gammaglobulins, corresponding to the monoclonal antibody. On rare occasions electrophoresis produces 2 narrow peaks simultaneously, suggesting the existence of 2 monoclonal antibodies, establishing a biclonal gammopathy. We present 4 such patients with biclonal gammopathy. The prevalence of mono- and biclonal gammopathies in the general population ranges between 1-3%, but only 1% of these are biclonal. Patients with biclonal gammopathy do not differ clinically from those with monoclonal gammopathy. Theoretically, pathogenesis of biclonal gammopathies might involve true biclonicity: 2 separate transformations in 2 precursor cells. Alternatively, the 2 transformed clones might have originated from a common ancestor cell early in differentiation, prior to immunoglobulin gene rearrangement; in this case pseudobiclonicity could be implied. The question of true biclonicity versus pseudobiclonicity has been examined by others using statistical analysis of isotypic combination distributions of heavy and light chains. The incidence of identity of the light chain isotype in both monoclonal antibodies (44%) was higher than would be expected in a random distribution (27%). In a study of the 2 monoclonal immunoglobulins, the identity of the amino acid sequences of the variable site was demonstrated, suggesting a common ancestor. In another research, anti-idiotypic antibodies suggested a common idiotype in the 2 proteins. Immunofluorescent labeling of bone marrow cells produced conflicting evidence of biclonicity in several studies.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1993

Massive hematuria due to extramedullary plasmacytoma invading the bladder.

Bladder involvement by extramedullary plasmacytoma is a rare condition. A 46-year-old woman with known IgA-lambda multiple myeloma who developed this condition is described. The patient suffered from massive hematuria, which led to hypovolemic shock. Prompt supportive treatment by blood transfusions, fulguration of the bleeding mucosa and continuous bladder irrigation stopped the bleeding. Subsequent bladder irradiation lead only to partial response of the tumor.

Female↗
PubMed1993

Favorable response of pediatric AIDS-related Burkitt's lymphoma treated by aggressive chemotherapy.

We describe 4 male children infected by the human immunodeficiency virus (HIV) who developed Burkitt's lymphoma during their disease. The clinical picture was characterized by an insidious appearance of symptoms. All the children suffered for several months from abdominal discomfort and a gradual elevation of their blood lactic dehydrogenase (LDH) level prior to diagnosis. Bone marrow involvement was found in 2 of the patients and jaw involvement in the other 2. After confirming the diagnosis of Burkitt's lymphoma, they were treated according to conventional protocols, with no need to reduce the dose intensity. They all went into complete remission and did not suffer from major opportunistic infections during chemotherapy. None of them relapsed. Two patients died from opportunistic infections 1 and 3 years after diagnosis. The other 2 are alive, 7 years and 6 months after diagnosis. The various characteristics of this unique pediatric group are described and the comparison of the clinical picture in adults is made, together with a review of the relevant literature.

Adolescent↗
PubMed1993

Analysis of rearranged immunoglobulin genes indicating a process of clonal evolution in chronic lymphocytic leukaemia.

Chronic lymphocytic leukaemia (CLL) is known to be a stable monoclonal neoplasm. In contrast to early studies demonstrating no more than two hybridizing immunoglobulin heavy chain bands corresponding to the two expected alleles, we have demonstrated an unexpected multiband pattern when the HindIII-digested DNA samples from 38 CLL patients were analysed by Southern blot hybridization using JH and C mu gene probes. In order to characterize the genetic basis for the multiband pattern, we molecularly cloned the immunoglobulin heavy chain genes of one of the patients whose leukaemic DNA sample demonstrated three hybridizing JH bands and a loss of the germline band. The cloned rearranged immunoglobulin genes could be divided, based on the restriction mapping and the hybridization with the various probes, into two basic patterns representing two alleles. In one of the cloned rearranged immunoglobulin genes a secondary rearrangement occurred that resulted in the addition of 300 base-pair long sequence into the switch region, and the creation of a HindIII restriction site. The results of the study suggest that clonal evolution occurs in some CLL, and that many of these neoplasms are indeed oligoclonal due to the accumulation of secondary genetic changes.

Antibodies, Neoplasm↗