PubMed Health⌕ Search

Biomedical subjects

B Stark

Publications and source records attributed to B Stark.

At least 73 records · Page 4Linked to original sources
PubMed1988

Deferoxamine (Desferal)-induced ocular toxicity.

A 4-year-old girl with juvenile chronic myeloid leukemia relapsed after an allogeneic bone marrow transplantation (BMT) and became refractory to conventional chemotherapy. Treatment with two courses of high-dose deferoxamine, an iron chelator (130-180 mg/kg/day), along with low-dose ARA-c (5 mg/kg/day) caused a remarkable decrease of the WBC and fetal Hb. Three days following the last dose of deferoxamine, the patient experienced an acute visual loss, confirmed by electroretinogram (ERG) and visual evoked response (VER). Slight improvement occurred a few days later, but the patient developed severe pancytopenia and died of Klebsiella septic shock. The ocular manifestations were attributed to deferoxamine toxicity in light of the rapid onset after first exposure, the electrophysiological pattern of metabolic damage in the ERG and VER, and the long interval between the last chemotherapy and BMT. The pathogenesis of deferoxamine toxicity is discussed.

Blindness↗
PubMed1988

Hematology and serum chemistry values in the beluga (Delphinapterus leucas).

Normal values and ranges for 31 clinical hematology and serum chemistry tests are reported for the beluga or white whale (Delphinapterus leucas). The values were collected over a 6-yr period from eight belugas maintained for display at Sea World (San Diego, California, USA) facilities and represent long-term evaluations for each animal in a controlled environment. They represent the first report for a number of serum chemistry values for the beluga. Normal values such as these provide an important data base from which to detect diagnostically important changes in health status for belugas in a zoological setting. They also establish a baseline from which to evaluate differences in normal values in free-ranging belugas and from which to diagnose disease problems in wild populations.

Animals↗
PubMed1988

More than two immunoglobulin heavy chain J region genes in the majority of infant leukemia.

Congenital and infant leukemia are rare conditions associated with a very poor prognosis due to the high frequency of adverse clinical and laboratory parameters. As the occurrence of multiple immunoglobulin heavy chain hybridization band in childhood leukemia has been associated with poor prognosis, we studied whether it was present in this type of leukemia as well. Seven cases were examined, 4 of them less than 7 months of age. The immunophenotype was lymphoid in 5 and hybrid in 2. Most had abnormal karyotypes. In 5 of the 7, including all with congenital leukemia, an immunoglobulin heavy chain J region multiband pattern was found by Southern blot. The multiband pattern, whether primary or due to clonal evolution, seems to be associated with poor prognosis.

Adolescent↗
PubMed1988

Heterogeneity of childhood acute lymphoblastic leukemia (ALL): monoclonal antibodies phenotyping and induction of differentiation in vitro.

Fifty-eight pediatric patients with non-T acute lymphoblastic leukemia (ALL) were diagnosed and evaluated at the Sambur Center of Pediatric Hematology Oncology. At least six subtypes of non-T ALLs were identified, corresponding to the various stages of B-cell differentiation, by utilizing an extensive panel of monoclonal antibodies directed against T- B- and myeloid-cell differentiation antigens. Moreover, leukemic cells expressing the phenotype of early B cells could be driven to differentiate along the B- cell lineage to express CALLA and BL antigens and cytoplasmic and/or surface immunoglobulins (IgM). A unique phenotype of non-T ALL was also identified. These leukemic cells expressed B cell antigen exclusively, i.e., HLA/DR and B4 (CD19). Myeloid-cell antigens, however, were expressed on these cells spontaneously after a 24-hour incubation in culture medium in vitro. In addition, leukemic cells of four patients with a phenotype of HLA/DR, CD19, and CD10 expressed antigens of the T-cell lineage: CD7 (3AI) and CD2 (leu 5), and/or of the myeloid cell lineage (My7). These results provide confirming evidence for the wide scope of the heterogeneity of ALL. It stresses the validity of accurate classification of leukemia to identify biologically and clinically unique subtypes of ALL, which bears specific prognostic parameters; and designates therapeutic protocols.

Antibodies, Monoclonal↗
PubMed1987

Immunologic dysregulation in a patient with familial hemophagocytic lymphohistiocytosis.

A 6-year-old Jewish Iranian girl with familial hemophagocytic lymphohistiocytosis (FHLH) is described. The course of the disease fluctuated with partial initial response to antibiotics, steroids, and supportive treatment. Subsequent cytotoxic treatment, including VP-16, Velban (vinblastine sulfate), and methotrexate (MTX) controlled the disease for a few months but the child died with a clinical picture of meningocephalitis 1.5 years later. Benign-looking lymphohistiocytic infiltrates with varying degrees of hemophagocytosis were present in the bone marrow, pleural effusion, cerebrospinal fluid (CSF), liver, and brain. Clinical and laboratory evidence of immunologic dysregulation during the disease could be demonstrated. Frequent and intense viral and bacterial infectious diseases were encountered. The laboratory examination most consistently found was the absence of natural killer (NK) cell activity against K562 target cells. The impaired activity of NK cells persisted during all stages of the disease including remission, although NK cell numbers, determined morphologically and immunophenotypically (by Leu-11, Leu-7), were normal. Natural killer activity could not be restored by interferon. Moreover, the interferon system appeared to be intact. Impaired monokin interleukin 1 (IL-I) production by peripheral blood monocytes was found and could not be restored by indomethacin. Lymphopenia, a mild decrease in T4 numbers, and subsequently, decreased proliferative response to mitogens was noted. Elevated immunoglobulin levels were found during exacerbations and viral episodes, at times accompanied by the presence of auto-antibodies. The exaggerated fatal lymphohistiocytic response typical for FHLH could be attributed to a underlying genetic pathologic dysregulation of the various immunological response pathways.

Child↗
PubMed1987

Alpha 1-adrenergic stimulation of ketogenesis and fatty acid oxidation is associated with inhibition of lipogenesis in rat hepatocytes.

The effect of norepinephrine on fatty acid synthesis (3H2O incorporation into fatty acids), on fatty acid oxidation to CO2 and on ketogenesis was studied in isolated hepatocytes of fed rats. After incubation with norepinephrine (50 microM), lipogenesis was lower (5.7 +/- 1.1 nmoles 3H2O incorporated into fatty acids/mg dry weight/30 min) than in controls (7.5 +/- 1.7; n = 6, p less than 0.02). In contrast, (1-14C) palmitate conversion into total ketone bodies was increased to 10.9 +/- 1.8 nmoles/mg/30 min with norepinephrine, vs 8.5 +/- 1.6 in controls (p less than 0.05), and more (1-14C) palmitate was converted to 14CO2 with norepinephrine than in controls (1.48 +/- 0.10 nmoles/mg/30 min vs 1.06 +/- 0.11, p less than 0.05). The inhibitory effect of norepinephrine on lipogenesis was abolished by addition of the alpha 1-receptor blocker prazosin, but not by alpha 2 or beta-blockers. The results demonstrate that the ketogenic effect of norepinephrine is coupled with an inhibitory effect on lipogenesis which may be explained by diminished activity of acetyl-CoA carboxylase, diminished formation of malonyl-CoA and decreased activity of carnitine palmitoyl transferase I.

Animals↗
PubMed1987

Undifferentiated leukemia of infancy with t(11:17) chromosomal rearrangement. Coexpressing myeloid and B cell restricted antigens.

It has been suggested that the malignant transformation, in some of the acute leukemias, may involve totipotent stem cells resulting in a biphenotypic leukemia expressing both myeloid, and lymphoid characteristics. We describe here a hybrid cell acute leukemia, in a 16-day-old infant, in whom leukemic cells coexpressed myeloid and lymphoid B cell antigens. Blast cells in the bone marrow showed L2 morphology according to the French American British (FAB) classification, with positive periodic-acid Schiff, and nonspecific esterase staining. Sudan black, and specific esterase were negative. Terminal deoxynucleotidyl transferase, was strongly positive in 5% of blasts, and faintly reactive with the rest. Karyotypic analysis demonstrated a translocation of t(11:17);(q23;p13). Immunoglobulin gene analysis revealed rearrangement of the heavy chain genes. The blasts' phenotype was HLA/DR+ B4+ My7+ My9+ common acute lymphoblastic leukemia antigen (CALLA) B1- T11-. Dual immunofluorescence staining using anti My7, and My9 fluorescein isothiocyanate, and anti B4 pycoerythrin conjugated monoclonal antibodies, and flow cytofluorometry, revealed a labeling pattern of 25% B4+; 10% to 15% My7+; 17% My9+; and 50% of cells coexpressing B4 My7, and My9 antigens. These results provide evidence for a hybrid leukemia with lymphomyeloblasts being part of a single clone, which may indicate the origin of this leukemic clone from a pluripotent (lymphoid/myeloid) stem cell.

Antibodies, Monoclonal↗
PubMed1987

Room temperature ADP induced first stage hyperaggregation of human blood platelets: a previously undescribed phenomenon and its relationship to spontaneous cold induced platelet aggregation.

ADP induced human platelet aggregation was shown to be accentuated when tested at 20-30 degrees C as increased sensitivity and as a greater change of optical density although second stage aggregation and the release reaction did not occur. This previously undescribed phenomenon is defined as room temperature ADP induced first stage hyperaggregation. Aggregation, which occurs under the above mentioned conditions with a quantity of ADP insufficient to maintain the aggregation (usually less than 1.5 micron), is reversible when the temperature is raised to 37 degrees C. After rewarming to these temperatures, second stage aggregation appeared in the presence of larger quantities of ADP (usually more than 2 microns) and could be blocked by aspirin. The absence of the release reaction was demonstrated with a lumi-aggregometer. Spontaneous cold induced platelet aggregation seen after chilling platelets to 0-4 degrees C is shown to be a distinct phenomenon.

Adenosine Diphosphate↗
PubMed1987

Intraocular involvement of Burkitt's lymphoma in a Bedouin child.

A child with a B-cell lymphoma with extensive abdominal disease responded to high doses of chemotherapy. One month later, eye and central nervous system recurrence developed. Intraocular involvement was documented by diagnostic vitrectomy. Differential diagnosis and therapy are discussed.

Abdominal Neoplasms↗
PubMed1986

Leukemia of early infancy. Early B-cell lineage associated with t(4:11).

A case of infantile acute leukemia associated with translocation t(4:11)(q21:q23) is reported. This leukemia has a very poor prognosis, and this patient survived for only 9 months. The blast cell morphology was L1/L2 according to the FAB classification and showed a lymphoid appearance on transmission electron microscopy. The histochemical stains showed a pattern of periodic acid-Schiff positivity and variable alpha-naphtyl acetate staining. The cells were TdT-positive and surface-marker phenotyping was positive for Ia-like and B4 antigens but negative for CALLA, T-cell markers, myelocyte and monocyte markers. The leukemic cells represent a frozen state of a very early precursor, corresponding to the earliest recognizable stage of the B-cell lineage. This observation may contribute to the controversion regarding the cell origin of this unique leukemia associated with t(4:11), lymphatic versus null cell, early myeloid, or mixed, and points to the possibility of a very early B-cell lineage leukemia.

Antigens, Neoplasm↗
PubMed1984

Familial hemophagocytic lymphohistiocytosis in Israel. II. Pathologic findings.

Presented are the pathologic findings in familial hemophagocytic lymphohistiocytosis (FHLH), based on observation of 11 cases from four affected families. The outstanding morphologic feature was a multiorgan involvement with lymphohistiocytic cellular infiltrates. The organs most frequently affected were the bone marrow and lymph nodes and, less frequently, the liver and the brain. The histiocytes in the infiltrates were usually bland and displayed prominent hemophagocytosis. The differential diagnosis of FHLH and the difficulty in distinguishing it from malignant histiocytosis is discussed. In view of the focal distribution of lesions, it is suggested that histopathologic diagnosis of FHLH be made only after evaluation of combined findings in several organs and consideration of the patient's family history as well as the result of the clinical and laboratory investigations.

Bone Marrow↗
PubMed1984

Familial hemophagocytic lymphohistiocytosis (FHLH) in Israel. I. Description of 11 patients of Iranian-Iraqi origin and review of the literature.

Eleven patients with familial hemophagocytic lymphohistiocytosis (FHLH) are described. They all belonged to four Jewish families of Iranian and Iraqi origin. Parental consanguinity was found in three families. The age of onset of disease ranged from 6 weeks to 36 months. All patients had fever, wasting, and enlargement of the liver and spleen. In addition, lymph-node enlargement and neurologic complications were common. The most consistent laboratory findings were pancytopenia, atypical lymphomonocytoid cells in the peripheral blood, abnormal liver function test results, and increased cerebrospinal fluid protein. The course was fatal in all patients. Nine of the 11 patients died within 2 weeks to 3 months of presentation, and 2 patients achieved temporary remissions but died of disease within 8 and 24 months, respectively. Response to antibiotic therapy or to the administration of corticosteroids and cytotoxic drugs was unimpressive. Pancytopenia complicated by sepsis or bleeding, hepatic failure, or encephalopathy were the terminal events. This report draws attention to the existence of FHLH in Jews of Iranian-Iraqi origin in whom parental consanguinity is very common.

Child, Preschool↗
PubMed1983

Cord blood polymorphonuclear leukocyte chemotaxis. Differential response to formyl peptide and endotoxin-activated serum.

The chemotactic responses of cord blood polymorphonuclear leukocytes (PMN) to two different chemotactic factors, N-formyl-Nle-Leu-Phe-Nle-Tyr-Lys (F-peptide) and the endotoxin-activated serum (EAS), were compared with that obtained with adult PMN. Cord blood PMN chemotactic response to F-peptide was normal, while the response to EAS was decreased (57%). The number of receptor sites for F-peptide on the membranes of cord blood PMN was also found to be normal (120,000 receptors/cell). The differential chemotactic response of cord blood PMN may suggest a poorly developed receptor system for the EAS, in which complement-derived peptides are the major chemoattractants.

Adult↗