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Biomedical subjects

H Kronenberg

Publications and source records attributed to H Kronenberg.

At least 91 records · Page 5Linked to original sources
PubMed1986

Early supradiaphragmatic Hodgkin's disease. High-dose gallium scanning obviates the need for staging laparotomy.

Experience with 16 sequential patients with Stage IA/IIA supradiaphragmatic Hodgkin's disease who had no evidence of intra-abdominal disease using high-dose gallium and computerized tomography scanning is reported. Subsequent staging laparotomy also was negative in all these patients and did not alter management decisions. It is suggested that high-dose, whole-body gallium scanning and other noninvasive staging procedures give reliable data for therapeutic decisions.

Adult↗
PubMed1986

Plasma lactoferrin in patients with neutropenia.

This study examines the role of plasma lactoferrin in the assessment of neutropenia. In particular, we have studied lactoferrin as an inhibitor of granulopoiesis and as an indicator of the size of the total blood granulocyte pool (TBGP). Plasma lactoferrin concentration was determined in a heterogeneous group of 30 patients with neutropenia. Serial plasma lactoferrin levels in a patient with cyclic neutropenia correlated with the cycles of the neutrophil count. Patients with splenomegaly had a grossly elevated lactoferrin:neutrophil ratio. Most chronic idiopathic neutropenia patients had no real clinical problems and a normal plasma lactoferrin level. The results provide further evidence to support the concept that plasma lactoferrin indicates the size of the TBGP and the lactoferrin: neutrophil ratio indicates the degree of granulocyte margination. There was no evidence to suggest that lactoferrin acting as a feedback inhibitor of granulopoiesis caused neutropenia in these patients.

Agranulocytosis↗
PubMed1986

Management of splenectomy failures in chronic immune thrombocytopenic purpura: role of accessory splenectomy.

Accessory splenic tissue was demonstrated in four of eight patients with chronic immune thrombocytopenic purpura investigated following post-splenectomy relapses. Time from initial splenectomy to relapse of thrombocytopenia ranged from immediately to eight years and post-splenectomy changes were present on the peripheral blood films of all patients at time of relapse. Three scanning techniques were employed to demonstrate and localise residual splenic tissue. Conventional 99mTc scans were positive in three of the four patients whilst 99mTc-heat damaged red cell scans and computerised tomographic scans were each positive in three out of three patients including the one patient in whom a conventional 99mTc scan was negative. Histological confirmation of splenic tissue was obtained in all cases with the weights of the accessory spleens ranging from 0.6 g to 2 g. Two patients responded to accessory splenectomy and, without immunosuppressive therapy, have remained well with normal platelet counts for over four years. There was no correlation between length of initial remission and the response to removal of the accessory spleen. The presence of a functioning accessory spleen should be considered in all patients with chronic ITP who fail to respond to, or relapse following, initial splenectomy.

Adult↗
PubMed1986

A phase I-II study of cytosine arabinoside, daunorubicin, and VP16-213 in adult patients with acute non-lymphocytic leukemia.

The combination cytosine arabinoside (ara-C), daunorubicin, and VP 16-213 was studied in 28 patients with acute non-lymphocytic leukemia to define the toxicity of the combination and assess its efficacy. Of 21 previously untreated patients, 16 (76%) achieved a complete response (CR) with the median remission duration not reached but exceeding 25 weeks. For CR patients, the median number of days with neutrophils less than 500/microliter was 19. The median survival for patients with CR is 60 weeks. Two of seven previously treated patients achieved CR for 11 weeks and in excess of 36 weeks, respectively. At the initial VP16-213 dose of 100 mg m-2 per day for seven days, severe stomatitis was seen in 38% of courses but was less with dose reduction to 75 mg m-2 per day for seven days. Other toxicity was similar to previous experience with ara-C and daunorubicin alone.

Acute Disease↗
PubMed1986

Complications associated with indwelling venous Hickman catheters in patients with hematological disorders.

During a 28 month period, 77 Hickman catheters were inserted in 66 patients with hematological disorders. Complete follow-up was possible for 72 catheter insertions. Catheters remained in situ for a median period of 77 days (range 5-474 days). Fourteen (19%) catheters required removal because of complications which included sepsis, blockage, or displacement. Catheter-related infection was the major complication and Staphylococcus epidermidis and diphtheroids (Corynebacterium species) were the organisms most commonly isolated.

Adolescent↗
PubMed1986

Molecular defects in 2 examples of severe Hb H disease.

Severe Hb H disease presented in unexpected ways in 2 families of Greek origin. In 1, Hb H disease led to neonatal death. The underlying molecular defect was double-heterozygosity for the --Med/ alpha thalassaemia haplotype and a nondeletional alpha thalassaemia defect (alpha alpha T'Karditsa'/). The 2nd family requested antenatal diagnosis. The husband had mild nondeletional alpha thalassaemia. Initial investigations in the wife demonstrated unexpected gene mapping patterns. These have recently been shown to result from the (-alpha)Med 20.5/ haplotype.

Chromosome Mapping↗
PubMed1986

Alpha thalassemia British type (alpha alpha/--Brit) in an Australian family.

Alpha thalassemia is rarely diagnosed in Australian families of British or Northern European ancestry. In 1972, a third generation Australian was shown to have alpha thalassemia. In the absence of known Mediterranean or South East Asian ancestry it was reported as being the first example of alpha thalassemia in an Australian family. Further study of the proposita in 1985 using DNA mapping of the alpha globin gene complex, shows a distinctive molecular defect identical to the British type of alpha thalassemia. The latter is clearly different from the commonly encountered Mediterranean and South East Asian alpha zero haplotypes. Recognition that alpha zero thalassemia occurs in Australians is important since it may produce a microcytic hypochromic anemia. Its inheritance together with other forms of alpha thalassemia may lead to severe Hb H disease or Hb Bart's hydrops fetalis.

Adult↗
PubMed1986

(delta beta) zero thalassemia of the Southern Italian type. Its geographical origin and interaction with the sickle cell gene.

Hematological phenotypes and molecular defects were compared in 11 examples of heterozygous (delta beta) zero thalassemia. Despite differences in ethnic origins all cases had the gene deletion that is found in (delta beta) zero thalassemia of the Southern Italian type. HbF levels in these patients ranged from 3.6-14.6% with a mean +/- 1 SD of 8.9 +/- 3.1%. Variability in HbF output would suggest that additional factor(s) apart from deletions within the beta globin complex are involved in regulation of gamma gene expression. One individual, a compound-heterozygote for (delta beta) zero thalassemia and HbS, presented with a sickling disorder. Reduced HbF production in family members who are heterozygotes for (delta beta) zero thalassemia may explain the clinical picture in this instance.

Adult↗
PubMed1986

Functional activity of the triplicated alpha alpha alpha 4.2/gene rearrangement.

Function of the triplicated alpha alpha alpha 4.2/gene rearrangement was assessed by measurement of Hb Bart's and hematological phenotype including alpha/beta biosynthesis ratio. Increased output of alpha globin chains in alpha alpha alpha 4.2/-- compared to alpha alpha/-- was found in a cord blood. In contrast, hematological phenotypes in two family members with the alpha alpha alpha 4.2/-- genotype were consistent with that expected in alpha alpha/--. This would suggest that the additional alpha gene in the alpha alpha alpha 4.2/rearrangement has variable expression.

Chromosome Mapping↗
PubMed1986

Hemoglobin Woodville: alpha (2)6(A4) aspartic acid----tyrosine.

Hemoglobin Woodville was detected in a Vietnamese woman during antenatal and cord blood screening programs for families of South East Asian origin. The variant constitutes 9% of total hemoglobin and structural analysis demonstrated the substitution alpha 6 Asp----Tyr. Hematological data on the proposita were normal. No apparent clinical or pathological findings were associated with this abnormal hemoglobin.

Adult↗
PubMed1986

Stimulation of persisting colonies in agar cultures by sera from patients with CML and AML.

Cord plasma contains colony-stimulating activity (CSA) which stimulates the in vitro clonal growth of neutrophils, eosinophils, macrophages, erythrocytes, and persisting mast cells in semisolid cultures. Analysis of day 35 colonies in agar cultures was found to be a suitable means of demonstrating this activity and discriminating between it and granulocyte-macrophage colony-stimulating factor (GM-CSF). Serum (10%) from patients with acute and chronic myeloid leukemia (AML and CML) was added to normal human bone marrow cultures to search for similar activity in these patient's serum. Although the number of colonies on day 12 (predominantly neutrophils and macrophages) was not significantly different from the number of colonies in cultures containing normal serum, the number of colonies increased 500% in cultures containing CML serum on day 35. Serum from patients with AML during regeneration also stimulated an increased number of colonies on day 35. Although both eosinophil and mast cell colonies were still present on day 35, only mast cell colonies persisted for 150 days. On day 35, cultures containing 10% CML serum contained predominantly eosinophil colonies (84%), whereas cultures containing AML serum contained predominantly mast cell colonies (76%). Although serum contains various CSFs, the specific factor which stimulates persisting mast cell colonies may be the human equivalent of murine persisting (P) cell-stimulating factor (Multi-CSF).

Agar↗
PubMed1986

Human bone marrow progenitor cells stimulated by cord blood.

Agar cultures of human bone marrow cells stimulated by cord plasma or irradiated cord blood feeder layers demonstrated the presence of a multilineage hemopoietic growth factor in cord blood. When bone marrow cultures stimulated with giant cell tumor-conditioned medium (GCT-CM) were supplemented with this cord blood-derived growth factor, total colony numbers increased by more than 50% after day 23 and persisted in culture for approximately 40 days. Marrow cultures stimulated by the cord blood-derived growth factor formed colonies of neutrophils, monocyte-macrophages, eosinophils, mast cells, and a few colonies containing a mixed cell population. The results suggest that, while GCT-CM contains granulocyte-monocyte colony-stimulating factor (GM-CSF), cord blood contains a high concentration of a multilineage hemopoietic growth factor, which may be multi-CSF.

Bone Marrow Cells↗
PubMed1986

Globin genes in Polynesians have many rearrangements including a recently described gamma gamma gamma gamma/.

Rearrangements involving genes of the alpha- and beta-globin loci were frequently detected in DNA from Polynesians. A founder effect and genetic drift occurring 2,000-3,000 years ago as Polynesians migrated eastward across the Pacific is proposed as the likely mechanism for these genetic changes that include deletions or additions of alpha-, gamma-, and zeta-globin genes and an unusual restriction fragment length polymorphism (RFLP) associated with the zeta gene. Preliminary data show different frequencies for gene rearrangements between island groups. Further study of these differences should provide additional information on the prehistory of Polynesians.

Chromosome Mapping↗
PubMed1985

Some studies with a monoclonal antibody directed against human fibrinogen.

Some properties of a monoclonal antibody generated against the fibrinogen component of a factor VIII preparation were investigated. The antibody bound with equal affinity in solid phase radioimmunoassays to fibrinogens isolated from both normal patients and patients with von Willebrand disease. It reacted in a sensitive immunoassay of plasma fibrinogen. The specificity of the antibody was confined to the parent molecule with no significant inhibition of fibrinogen binding by the fibrinogen degradation products (FDPs) X, Y, D, or E. The antibody had no significant effect on the activated partial thromboplastin time and prothrombin time of normal plasma. However, it prolonged the thrombin time as determined by the Clauss chronometric fibrinogen method. During fibrinogen lysis by plasmin immunoreactivity of fibrinogen to the antibody was lost at the same rate as the clottable fibrinogen content determined by the Clauss assay. The lack of reactivity of the antibody with FDPs makes it a suitable reagent for investigating plasmin activity as well as studying fibrinogen and fibrin. These findings suggest that the epitope of the antibody lies within the polar protruberance of the carboxy terminal end of the A alpha chain of fibrinogen and is destroyed by plasmin cleavage.

Antibodies, Monoclonal↗