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Biomedical subjects

K A Deubelbeiss

Publications and source records attributed to K A Deubelbeiss.

At least 19 recordsLinked to original sources
PubMed1992

[Acquired hemoglobin H disease in the early stage of erythroleukemia].

At the onset of erythroleukemia, the patient, a 74-year-old Swiss male, was also found to have microcytic-hypochromic anemia (Hb: 82 g/l, MCV: 69 fl, MCH: 21 pg). Further laboratory examinations revealed reduced hemoglobin stability, a hemoglobin H fraction of 3.0% on cellulose acetate-electrophoresis, and an abundance of hemoglobin H inclusion bodies in red cells. These findings, as well as the Swiss origin of the patient and his age at the onset of the disease, were consistent with acquired hemoglobin H disease. In addition to genetic hemoglobin H disease, acquired hemoglobin H disease was reported to be associated with myelodysplastic and myeloproliferative syndrome, or erythroleukemia and acute myelogenous leukemia. The literature contains fewer than 50 cases. It is suggested that the molecular basis of this rare disease involves a gene in trans to the alpha-globin genes reducing the expression of all four alpha-globin genes to approximately 10% of normal activity.

Aged↗
PubMed1984

Autologous bone marrow transplantation in the treatment of children and adolescents with advanced malignant tumors.

Nineteen patients with advanced malignant tumors, less than 20 years old were treated with intensive chemotherapy (vincristine 2 mg/m2 i.v. and adriamycin 60 mg/m2 i.v. on day - 7; cyclophosphamide 45 mg/kg i.v. on days -6 to -3), total body irradiation (TBI, 600 rads on day -1) and autologous bone marrow transplantation (ABMT, day 0). Prior to this procedure induction of complete or partial remission by conventional therapy was attempted. Ten patients had intra-abdominal non-Hodgkin's lymphoma (NHL); three, yolk sac tumor; three, Ewing's sarcoma; and three, neuroblastoma. The supportive care included reverse isolation, immunoglobulin 400 mg/kg i.v. q 2 weeks, cotrimoxazole per os, and cell support as needed. No correlation between the bone marrow dose and the time of hematological reconstitution could be established. Five of seven patients with intra-abdominal NHL stage III (transplanted in first remission) are surviving disease-free for 5+, 5+, 20+, 23+, and 35+ months after ABMT. None of three patients with intra-abdominal NHL stage IV is surviving (two of them were transplanted in second remission). One of three patients with yolk sac tumor is surviving disease-free for 27+ months. There are no survivors among the patients with Ewing's sarcoma and neuroblastoma. Only one of 19 patients was lost due to therapeutic complications, while 12 died due to tumor. Regarding treatment results for advanced intra-abdominal NHL, the procedure described here is comparable to the best conventional regimens. In vitro methods for tumor cell eradication in the collected bone marrow might further improve the results of ABMT.

Adolescent↗
PubMed1983

Influence of the spleen on the distribution of blood neutrophils. Quantitative studies in the rat.

The influence of the spleen on the distribution of blood neutrophils was studied in rats with varying spleen sizes following transfusion of isologous 3H-dT-labelled neutrophils. The weight of the spleen correlated with the splenic neutrophil pool (r = 0.87, P less than 0.01). Alterations in spleen size did not significantly change the total blood neutrophil pool, but with increasing splenomegaly circulating and marginal neutrophils were shifted into the splenic pool. With massive splenomegaly, up to 50% of the total blood neutrophils were trapped in the spleen. These findings suggest splenic pooling of neutrophils and a shift of circulating and marginal neutrophils into the spleen proportionate to the splenic tissue present.

Animals↗
PubMed1979

[Selective aplasia of neutrophils: auto-immune origin].

Autoimmunity is a recognized factor in pure red cell aplasia and in some cases of aplastic anemia, but not in agranulocytosis. The case reported here demonstrated that pure neutrophilic aplasia of autoimmune origin may exist in man. A 75-year-old male is described who presented with complete agranulocytosis and absence of neutrophilic precursors in the bone marrow without thrombocytopenia or anemia. After 6 weeks, remission was induced by immunosuppressive treatment and 22 months later the patient is doing well without therapy. The absence of known cases of agranulocytosis, a good response to immunosuppressive therapy, and the demonstrated fact that the patient's peripheral blood mononuclear cells inhibit the growth of granulocytic colonies of normal human marrow in vitro allow the conclusion that this patient had pure neutropenic aplasia, probably due to autoimmune phenomena.

Aged↗
PubMed1979

[Autologous bone marrow reimplantation in children with advanced tumor. First experiences of feasibility].

In three children with metastatic tumor uncontrollable by conventional chemo- and radiotherapy, bone marrow was obtained under general anesthesia and cryopreserved according to a carefully developed protocol. The autologous bone marrow cells were reinfused after intensive cytostatic therapy and total body irradiation (2 patients). After an aplastic phase of 7--14 days the peripheral blood leukocyte and thrombocyte count began to recover. The toxicity of the intensive treatment preceding the autologous bone marrow transfusion, and the autologous bone marrow cells themselves were well tolerated. The aplastic phase was easily controlled by the use of granulocytes, thrombocytes and erythrocytes. Except for fever and mucosal ulcerations observed during the phase of extreme leukopenia, the general condition of the patients during partial isolation lasting 26--34 days was astonishingly good. One child died 13 weeks after returning home due to a local relapse. The other two patients survived for 6 + and 11 + weeks and are in complete and partial remission respectively. A further evaluation of the clinical significance of autologous bone marrow reimplantations appears to be feasible in pediatric oncology.

Adolescent↗
PubMed1978

Postmitotic marrow neutrophils and neutrophil mobilization in man: role of the spleen.

The relationship between postmitotic marrow neutrophils (PMMN) and neutrophil increment in blood after an intravenous dose of 3 mg hydrocortisone/kg was studied in patients with normal-sized spleens and compared with splenectomized and splenomegalic patients. PMMN were quantified from the ferrokinetic measurement of the normoblast number and the PMMN/normoblast ratio in bone marrow biopsy sections. In 12 control patients with normal PMMN the increment was 3.50 +/- 1.13 X 10(9) neutrophils/liter. An excellent correlation was found between the number of PMMN and the maximal neutrophil increment (y = 826x - 1021, r = 0.93, p less than 0.001) among 24 patients with normal spleen size and a wide range of marrow cellularity. Significantly higher responses were observed in 10 splenectomized patients (y = 872x + 1429, r = 0.92, p less than 0.001). The two regression lines were shown to be parallel, indicating a diminution of the response by 2.5 X 10(9) neutrophils/liter in the presence of a normal spleen. In 11 hypersplenic patients the responses were further reduced and more variable. Peak neutrophilia occurred after median values of 2, 3, and 4 hr in the hypersplenic, the control, and the splenectomized group, respectively. These studies indicate that allowing for the different response curves neutrophil increments may be used as an index of PMMN in patients with normal spleen size and in splenectomized patients. They further suggest sequestration of the prematurely released cells by the spleen.

Bone Marrow Cells↗
PubMed1977

[The effect of various cryoprotective agents on the human bone marrow].

The following combinations were evaluated as cryoprotectors of human CFU-c enriched bone marrow frozen for one hour at -190 degrees C: 10% DMSO, 1% DMSO + 9% dextran (Dx), 1% DMSO + 9% HES, 15% glycerin (Gc), 1% Gc + 9% Dx, 1% Gc+ 9% HES, 5% Gc + 4% glucose. Cell counts and CFU-c content were compared with controls left at 4 degrees C. Highest values were observed with 10% DMSO and with 1% DMSO + 9% Dx. These studies confirm the superiority of DMSO to Gc as a cryoprotector. With the combination of 1% DMSO + 9% Dx the removal of DMSO prior to reinfusion of the marrow is obviated. This should facilitate the clinical use of autologous marrow transfusion.

Bone Marrow↗
PubMed1976

[Rise in neutrophils after hydrocortisone seen as bone marrow reserve mass. Dependency on spleen size].

In 25 patients with a wide range of postmitotic marrow neutrophil mass a linear relationship was observed between the number of postmitotic marrow neutrophils and the rise of blood neutrophils after intravenous injection of 3 mg hydrocortisone/kg. Related to marrow neutrophil cellularity, the response curve was significantly higher in 10 splenectomized patients but greatly depressed in 10 patients with splenomegaly.

Bone Marrow↗
PubMed1976

Section preparation of human marrow for light microscopy.

Preparation of human marrow sections has been studied systematically in order to facilitate accurate identification of marrow cells. Both of the methods developed involve embedding marrow cores in methyl methacrylate. In one, acrolein fixation is followed by staining of deplasticized sections with eosine-y followed by azure II; in the other, neutrophilic forms are identified by their esterase-specific reactivity in marrow fixed with neutral-buffered formalin. These preparations are suitable for quantitative studies of marrow cellularity.

Acrolein↗
PubMed1976

Neutrophil kinetics in man.

A method has been developed for measuring neutrophil cellularity in normal human bone marrow, in which the neutrophil-erythroid ratio was determined from marrow sections and marrow normoblasts were estimated by the erythron iron turnover. Neutrophil maturational categories, defined by morphologic criteria, were supported by autoradiographs of marrow flashed-labeled with 3H-thymidine. Correction for multiple counting error was empirically derived by counting serial sections through cells of each maturational category. The normal neutrophil-erythroid ratio in 13 normal human subjects was 1.5 +/- 0.07. The mean number of normoblasts in the same subjects was estimated to be 5.07 +/- 0.84 X 10(9) cells/kg. Total marrow neutrophils (X 10(9) cells/kg) were 7.70 +/- 1.20, the postmitotic pool (metamyelocytes, bands, and segmented forms) was 5.59 +/- 0.90 and the mitotic pool (promyelocytes + myelocytes) was 2.11 +/- 0.36. Marrow neutrophil ("total") production has been determined from the number of neutrophils comprising the postmitotic marrow pool divided by their transit time Transit time was derived from the appearance in circulating neutrophils of injected 3H-thymidine. The postmitotic pool comprised 5.59 +/- 0.90 X 10(9) neutrophils/kg, and the transit time was 6.60 +/- 0.03 days. From these data marrow neutrophil production was calculated to be 0.85 X 10(9) cells/kg per day. Effective production, measured as the turnover of circulating neutrophils labeled with 3H-thymidine, was 0.87 +/- 0.13 X 10(9) cells/kg per day. This value correlated well with the calculation of marrow neutrophil production. A larger turnover of 1.62 +/- 0.46 X 10(9) cells/kg per day was obtained when diisopropylfluorophosphate-32P was used to label circulating neutrophils. Studies using isologous cells doubly labeled with 3H-thymidine and diisopropylfluorophosphate-32P demonstrated a lower recovery and shorter t1/2 of the 32P label.

Adolescent↗
PubMed1975

[Neutropenia in disorders of the neutrophil motility].

A 51-year-old patient exhibited severe neutropenia over a period of 12 months following lobectomy for carcinoma of the lung. Marrow neutrophil mass and marrow neutrophil production were measured quantitatively and were within normal limits. Mobilization into a skin window was absent. A defect in neutrophil motility could be demonstrated in vitro and was confirmed in vivo by poor neutrophil release from the marrow following injection of hydrocortisone; it thus represented a lazy leukocyte syndrome of the adult.

Agranulocytosis↗
PubMed1975

[Kinetics of neutrophil granulocytes].

The kinetics of neutrophilic granulocytes are briefly reviewed. The cell traverses 3 compartments during its life cycle. After a phase of proliferation and maturation in the bone marrow for a period of about 10 days, it enters the blood for an average period of 10-12 h and then migrates into tissues where it survives for an unknown length of time. Although a quantitative understanding of normal neutrophil kinetics has emerged, our knowledge of disorders of neutropoiesis is limited. Progress in the diagnosis and treatment of disorders of neutropoiesis is anticipated with improved methods of assessing quantitatively abnormal production and its regulation.

Bone Marrow Cells↗