PubMed Health⌕ Search

Biomedical subjects

G Sokal

Publications and source records attributed to G Sokal.

At least 37 records · Page 2Linked to original sources

Addison's disease: immunological aspects.

The frequency of HLA DR3 in patients with Addison's disease is increased and the relative risk factor rises to 10 when adrenocortical failure is part of a polyendocrinopathy.

Addison Disease↗

Splenectomy in advanced chronic lymphocytic leukemia.

Forty chronic lymphocytic leukemia patients with splenomegaly were evaluated for splenectomy. Twenty were splenectomized. All but two normalized their hematocrit and all but one their platelet count. Satisfactory residual erythropoiesis as evaluated using ferrokinetics enabled prediction of a good response to splenectomy in 18 of 20 patients. The two patients who did not respond despite satisfactory residual erythropoiesis had a deteriorating blood count after splenectomy with progressive disease. The measurement of the splenic red cell volume predicted the minimal increase in hematocrit that could be expected after splenectomy. The transfusion need was abolished in 13 of 14 patients who needed red cell transfusions before splenectomy. Despite clinical improvement after the operation in all but two patients, splenectomized patients did not survive longer than nonsplenectomized patients, whether survival probability was calculated from the time of diagnosis or from the time of evaluation.

Actuarial Analysis↗

Quantitative assessment of erythropoiesis in bone marrow expansion areas using 52Fe.

Quantitative 52Fe scans were performed in 180 patients. Expansion of bone marrow was observed in 70. This bone marrow expansion was a nearly constant feature in haemolytic anaemia and in sideroblastic anaemia. It occurred in a third of the patients with myelofibrosis. In patients with polycythaemia rubra vera, expansion was noticed in only two out of seven. Erythropoiesis in expansion areas occurred despite persistence of fat in the iliac crest bone marrow biopsy. It could exist with a slight increase in erythropoiesis and might develop only after a long period of erythropoietic stimulation. Increased marrow activity can take place without erythropoietic expansion in long bones. The fraction of iron uptake in expansion areas did not exceed a third of total marrow iron uptake. With increasing erythropoiesis, the increase in iron uptake in expansion areas was less marked than the increase in the central areas. Erythropoiesis in expansion areas was usually not of major quantitative importance but could nevertheless reach the erythropoiesis of a normal adult.

Adult↗

Combined modality therapy for mediastinal Hodgkin's disease. Prognostic significance of constitutional symptoms and size of disease.

Combined modality therapy was used in an attempt to increase the complete remission rate and survival of previously untreated patients with Hodgkin's disease. MOPP (nitrogen mustard, vincristine, procabazine, prednisone) chemotherapy was followed by radiotherapy. The median duration of follow-up exceeded 5 years. Complete remissions were achieved in 63 of 71 patients (89%) with mediastinal masses. The actuarial survival of 10 years was 72% for patients with small mediastinal masses (mediastinal mass ratio less than 0.35) and 46% for patients with large mediastinal masses (P less than 0.05). The corresponding disease-free survival figures were 69% and 46%, respectively (P less than 0.05). A small mediastinal mass did not affect prognosis. Systemic symptoms especially affected the prognosis in patients with a large mediastinal mass, since in symptomatic patients the actuarial survival and freedom from relapse were 19%, whereas all asymptomatic patients survived without relapse. In patients with a small mediastinal mass, systemic symptoms had no significant effect on the actuarial survival or disease free survival. Age and stage did not affect the prognosis in patients with large mediastinal masses. It was concluded that MOPP chemotherapy followed by radiotherapy was an adequate treatment for asymptomatic patients with large mediastinal disease. However, in symptomatic patients with large mediastinal masses, this treatment was clearly inadequate.

Adolescent↗

Assessment of the sites of red cell destruction using quantitative measurements of splenic and hepatic red cell destruction.

Red cell survival, surface counting indices, the splenic and hepatic contribution to red cell destruction and the rate of splenic and hepatic red cell destruction were measured in 29 patients. Splenectomy was performed in 14. No correlation could be found between the splenic excess count index and both the amount and rate of red cell destruction in the spleen, but the rate of splenic and hepatic red cell destruction was related to the rate of disappearance of red cells from the circulation. The mean fractions of red cell destruction in spleen and liver were 46.1% +/- 20.5 (SD) and 11.7% +/- 4.2 (SD) respectively. After splenectomy, the haematocrit returned to normal in all patients despite fractions of red cell destruction in the spleen not exceeding 60%. Although the measurements of the splenic red cell destruction rate and of the fraction of red cell destruction in the spleen provide more precise information on the role of the spleen in red cell destruction, their prognostic value in patients who underwent splenectomy was not obvious.

Adult↗

In vivo stimulation and inhibition of granulopoiesis: the effect of an inflammatory reaction on murine diffusion chamber granulopoiesis.

Humoral factors influencing granulopoiesis have been evaluated using diffusion chambers (DC) implanted in the peritoneal cavity of mice challenged by an aseptic abscess produced by the subcutaneous implantation of copper rods. This resulted in an increase in peripheral blood neutrophils and an increase in tibial granulocytic elements. When DC loaded with bone-marrow cells were implanted into mice stimulated the day before by an aseptic abscess significantly more CFU-s, CFU-c, proliferative and non-proliferative granulocytes were produced, as compared to DC implanted into control hosts. When DC were implanted 4-6 d after the induction of inflammation in mice a significant depression of DC granulopoiesis was observed. Levels of serum and DC fluid CSF and serum inhibitors of in vitro colony growth showed no correlation with DC myelopoiesis. The data show that mice undergoing an inflammatory reaction elaborate first humoral substance(s) enhancing CFU-s and granulocytic growth in DC and next inhibitory factor(s) of DC granulopoiesis.

Animals↗

Assessment of bone marrow and splenic erythropoiesis in myelofibrosis.

The iron uptake in bone marrow and spleen was measured in 29 patients with myelofibrosis using 52Fe and quantitative scanning. In 10 patients, no iron uptake in the marrow could be observed and active erythropoiesis was extramedullary only. In the bone marrow of patients with myelofibrosis, the iron uptake per nucleated red cell was less than that observed in conditions without myelofibrosis or extramedullary erythropoiesis. Increasing splenic iron uptake was likely to be associated with a decreasing bone marrow iron uptake and was related to the size of the spleen. The data suggest that in myelofibrosis, the spleen dominates iron uptake through intense erythropoiesis and a high splenic blood flow, thus restraining iron supply to the bone marrow.

Adult↗

Myeloid stem cell kinetics in children hypertransfused during remission induction of acute lymphoblastic leukemia.

Experimental studies in animals and recent preliminary clinical evidence raised the possibility that hypertransfusion might be capable of producing a beneficial effect on granulopoiesis recovery following irradiation or chemotherapy. This prompted us to design a study to determine the effect of hypertransfusion on the blood and marrow CFU-c of leukemic children during remission induction. Nineteen children with acute lymphoblastic leukemia have been randomized in pairs to normotransfused (Hb: 12-14 g/dl) and hypertransfused (Hb: 16-18 g/dl) groups. Anti-leukemic chemotherapy (vincristine and adriamycin weekly during 4 weeks and prednisone daily) was identical in all children. As expected, suppression of erythropoiesis was observed in the hypertransfused group. During the first three courses of chemotherapy, the number of marrow CFU-c remained very low in both groups. One week after the third course of chemotherapy the number of bone marrow CFU-c began to increase in both groups. One week after course four the CFU-c value was significantly larger in the hypertransfused group. We also observed that circulating CFU-c were almost absent before induction chemotherapy, whereas their number increased after course three and was higher in the hypertransfused group and remained higher after course four. These results show the kinetics of bone marrow recovery after chemotherapy and suggest that hypertransfusion increases the rate of recovery of granulopoiesis.

Adolescent↗

HLA haplotypes and long survival in childhood acute lymphoblastic leukaemia treated with transfer factor.

Association between HLA haplotypes and long survival was investigated in 116 children with acute lymphoblastic leukaemia. It was found that patients with A2 B12 and/or A2 B40 haplotypes survived longer than patients wtihout these two haplotypes. Since all children were treated with transfer factor obtained from their relatives, it is suggested that children possessing A2 B12 or A2 B40 haplotypes may respond better to this type of immunotherapy.

Acute Disease↗

Measurement of erythropoiesis using 52Fe.

A method for determining erythropoiesis quantitatively with 52Fe has been applied to 25 patients with anaemia. The data were derived from quantitative scanning of the erythropoietic areas and the measurement of the plasma iron turnover. We have assessed this 52Fe measurement of erythropoiesis by comparing it with total marrow iron turnover studies. Both methods give similar estimates of erythropoiesis except in patients with severe ineffective erythropoiesis and in a patient with significant peripheral haemolysis, when erythropoiesis as measured with the 52Fe technique gave higher results. In these conditions, the estimate of total erythropoiesis might be more reliable using the 52Fe quantitative scanning technique. However, the measurement of erythropoiesis with 52Fe does not distinguish effective from ineffective erythropoiesis and cannot be applied to patients with extramedullary erythropoiesis.

Bone Marrow↗

Selective hypoplasia of pelvic bone marrow.

6 patients with thrombocytopenia and anaemia had fatty marrow replacement at the site of an iliac crest. 52Fe scans showed marked abnormalities of bone marrow distribution. Particularly, the uptake of radioiron into the pelvis was almost absent. The sternal marrow was cellular. Studies of the bone marrow which included cytology, histology and electron microscopy failed to reveal why the pelvic marrow was aplastic. Occasional vascular lesions could not seen in the iliac crest bone marrow sections. The cellular morphology showed slight maturation abnormalities. Ferrokinetics were consistent with erythroid marrow hypoplasia. The pelvic and sternal marrow cellularity in patients with pancytopenia may not be representative of the cellularity of the whole marrow, and the pelvic marrow especially may be prone to aplasia.

Adult↗

A phase II study of vindesine in patients with hematological malignancies.

A phase II protocol associating vindesine and prednisone was used in 47 hematological patients of whom 40 were evaluable. The best results were achieved in relapses of childhood's ALL, with a high proportion of complete remissions and in blastic crises of CML. The efficacy appeared less in relapses of ALL in adults and of malignant lymphoma. The other cases seemed not to respond favorably. The hematological toxicity consisted of leucopenia generally of short duration, but sometimes severe. The neurological toxicity was little and led to reduction of the dosage in only two adults with preexisting neuropathy.

Child↗

Chromosome abnormalities in acute promyelocytic leukemia (APL).

Sixteen patients, 15 adults and one child, with APL have been studied cytogenetically; 14 of these had an abnormal karyotype (87%). Eleven of these consistently showed a t(15;17)(q26;q22) structural anomaly, one patient showed a 47,+8 karyotype, one a rearrangement of chromosomes No. 15 and No. 17, apparently different from that in the other patients, and one a No. 17 deletion without a demonstrable translocation. as an additional chromosome change trisomy No. 8 was found in 5 cases and monosomy No. 7 in two. The t)15;17)(q26;q22) structural anomaly is highly characteristic of APL, is found in APL of children and adults, but it is apparently not associated with a clinically different form of APL.

Adult↗