Biomedical subjects

M D Seftel

Publications and source records attributed to M D Seftel.

8 recordsLinked to original sources

PubMed2006

Leukocyte count as a predictor of death during remission induction in acute myeloid leukemia.

Acute myeloid leukemia (AML) presenting with a high leukocyte count has been associated with an increase in induction mortality and poor results in a number of other survival measures. However, the level at which an elevated leukocyte count has prognostic significance in AML remains unclear. In this report on a series of 375 adult (non-M3) AML patients undergoing induction chemotherapy at a single institution, leukocyte count analyzed as a continuous variable is shown to be a better predictor of induction death (ID) and overall survival (OS) than a leukocyte count of > or = 100 x 10(9)/L, a value characteristically associated with "hyperleukocytosis" (HL). In this patient cohort, a presenting leukocyte count of > or = 30 x 10(9)/L had high sensitivity and specificity for predicting ID, and both performance status (PS) and leukocyte count more accurately predicted for ID than age. Considering these parameters in newly-diagnosed AML patients may facilitate the development of strategies for reducing induction mortality.

Adolescent↗

PubMed2006

Myeloid sarcoma of the uterus presenting as vaginal bleeding.

Extramedullary relapse of acute myeloid leukaemia may occur in sites such as the central nervous system, testes, and skin. Presentations in the female genital tract are uncommon and usually asymptomatic. In contrast, symptomatic uterine myeloid sarcoma is very rare. Treatment of this is generally unsuccessful, but is improved when systemic therapies are used. We study a case of a uterine relapse of acute myeloid leukaemia presenting as vaginal bleeding and successfully managed by local irradiation. The mechanism of preferential infiltration of uterine tissue requires further study.

Diagnosis, Differential↗

PubMed2002

Fulminant tumour lysis syndrome in acute myelogenous leukaemia with inv(16)(p13;q22).

Tumour lysis syndrome (TLS) is caused by rapid breakdown of malignant cells resulting in electrolyte disturbances and acute renal failure. TLS has rarely been described in patients with acute myelogenous leukaemia (AML). Between November 1997 and July 2001, 114 consecutive adult AML patients aged <60 yr received induction chemotherapy consisting of cytosine arabinoside 1.5 g m(-2) q 12 h x 12 doses and daunorubicin 45 mg m(-2) d(-1) x 3 doses. During induction chemotherapy (CT), seven patients (6.1%, 95% CI 2.5-12.2) developed fulminant TLS, resulting in acute renal failure; five of these seven patients had inversion of chromosome 16 [inv(16)(p13;q22)], and one patient had a biological equivalent [t(16,16)(p13;q22)]. Four of the TLS patients underwent leukapheresis for a presenting white blood cell (WBC) count > 100 x 10(9) L(-1) prior to commencing chemotherapy, and six patients subsequently required haemodialysis for a median of 2 (range 1-8) wk. One TLS patient died of intracerebral hemorrhage on day 10 and another patient of multiorgan failure on day 17. Of the other five patients, all entered a complete remission (CR) and recovered normal renal function. Four patients remain in continuous CR [median follow-up 20 (range 12-25) months]. One patient relapsed at 12 months and again developed TLS on re-induction. In univariate analysis, TLS patients were more likely to have an elevated presentation and pre-chemotherapy WBC counts, elevated serum creatinine, and uric acid levels at presentation, as well as an inv(16). In multivariate analysis, only serum creatinine and inv(16) remained statistically significant (P < 0.001 for each). Patients with an inv(16) are a unique AML subgroup at high risk for fulminant TLS.

Adolescent↗

PubMed2002

Hematopoietic stem cell transplantation: the Vancouver experience.

Hematopoietic stem cell transplantation has been available as a therapeutic modality for selected adult patients in Vancouver, British Columbia since 1981. We report on the history, progress, and future prospects of the Leukemia/Bone Marrow Transplantation Program of British Columbia. The basic mechanisms and indications for hematopoietic stem cell transplantation are outlined. Limitations of this procedure are also examined, particularly that of associated toxicities such as graft-versus-host-disease.

British Columbia↗

PubMed2002

Treatment of lymphoid malignancies with non-myeloablative stem cell transplantation.

The traditional approach to allogeneic hematopoietic stem cell transplantation involves the administration of myeloablative preparative regimens. This form of conditioning is associated with a relatively high incidence of regimen-related toxicity. As a result, candidates for allogeneic stem cell transplantation may be excluded owing to advanced age or co-morbid medical illness. Recently, so-called "non-myeloablative" regimens have been introduced, where less intense conditioning therapy is used in an attempt to reduce regimen-related toxicity. In addition, non-myeloablative transplantation takes advantage of the graft-versus-tumour effect that is characteristic of allogeneic stem cell transplantation. We review the background, available clinical data, and future directions in non-myeloablative stem cell transplantation, and focus on its potential use in the treatment of lymphoid malignancies.

Graft vs Tumor Effect↗

PubMed2002

Intra-cerebral relapse following prolonged remission after autologous stem cell transplantation for multiple myeloma.

Central nervous system (CNS) myeloma is a rare phenomenon, especially so after high-dose therapy (HDT) and stem cell transplantation. We describe a case of isolated CNS relapse of myeloma post autologous transplantation that followed a prolonged progression-free interval. Issues regarding the pathophysiology and management of this unusual complication are discussed.

Brain Neoplasms↗

PubMed1997

Tetra-amelia and splenogonadal fusion in Roberts syndrome.

Roberts-SC phocomelia syndrome comprises limb deficiencies of variable severity, facial clefts, and other anomalies. Tetra-amelia may also be associated with facial clefts and similar anomalies. We report on a female infant with severe tetra-amelia, micrognathia, cleft palate, splenogonadal fusion, and premature centromere separation. We propose that this represents the severe expression of the Roberts-SC phocomelia syndrome.

Abnormalities, Multiple↗

PubMed1996

Lethal neonatal mandibuloacral dysplasia.

We report on a case of lethal neonatal mandibuloacral dysplasia. Large confluent fontanelles, sparse fine hair and eyebrows, pseudo-exophthalmos, micrognathia, bulbar digits, and short clavicles were present. In addition, we describe for the first time the presence of glandular hypospadias in this disorder. We propose that this neonatally lethal case represents severe expression of mandibuloacral dysplasia.

Clavicle↗