Biomedical subjects
Publications and source records attributed to L Konopka.
Pseudothrombocytopenia in four patients has been described. It was due to platelet "++EDTA-dependent" antibodies. The number of platelets was described only in EDTA blood while the number was normal when platelet were evaluated in citrate blood. The importance of distinguishing pseudothrombocytopenia from thrombocytopenia is discussed, especially in view of unnecessary treatment, delayed operation etc.
In 6 women aged 38 to 68 years with thrombocythaemia during chronic myeloid leukaemia (4 cases), myelofibrosis (1 case), and idiopathic thrombocythaemia (1 case) the effects of recombinant human alpha-interferon (Intron A, rh IFN alpha -2b, Schering) were studied. The drug was given to all patients subcutaneously in one daily dose of 3 x 10(6) u, every day for 3 weeks, and then in the same doses twice weekly for 2 weeks (5 cases) and for 14 weeks (1 case). Intron A caused in all cases a fall of peripheral blood platelet count by 37% to 65.5% (mean 50%) in relation to the initial count (532 - 1,453 x 10(9)/l). The fall of the platelet count occurred usually after 7-10 days of this treatment, and the lowest count was noted usually after 24 days (10 to 42 days). During the treatment in 4 cases the peripheral leucocyte count dropped as well by 20-70%. In no cases exacerbation of chronic myeloid leukaemia was noted, and in the patient with myelofibrosis the enlarged spleen shrunk somewhat. These results of treatment and follow-up of patients with thrombocythaemia treated with Intron A indicate a significant although short-lasting effect of platelet count fall limited, however, to the time of the treatment. Side effects of the drug included mainly febrile conditions, myalgia and arthralgia.
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Measurement of delta-aminolaevulinic acid (ALA) synthetase has been performed in bone marrow of 24 normal subjects and 20 patients with sideroblastic anaemia (five congenital, two pyridoxine-responsive, 12 primary acquired and one secondary form due to isoniazid therapy). ALA-synthetase activity without added pyridoxal-phosphate in vitro was decreased in four of the congenital cases, and in all 12 primary acquired cases. In two of these 16 cases with low ALA-synthetase activity and in a case with sideroblastic change secondary to isoniazid therapy, the enzyme activity was corrected to normal by pyridoxal-phosphate in vitro. ALA-synthetase activity was normal in both cases of pyridoxine-responsive anaemia while they were receiving pyridoxine therapy but in one of these cases, studied at repeated intervals after withdrawal of pyridoxine therapy, the bone marrow ALA-synthetase activity fell to very low levels unless pyridoxal-phosphate was added in vitro. In this case the Km of the enzyme for pyridoxal-phosphate was substantially greater than that of a control sample. Haem synthetase activity was reduced in the bone marrow of two of six cases of primary acquired sideroblastic anaemia and in one congenital case tested but was normal in the pyridoxine-responsive patient studied on pyridoxine therapy. Nevertheless, this patient showed reduced incorporation of 14C-labelled delta-aminolaevulinic acid into bone marrow haem. It is concluded that both congenital and primary acquired sideroblastic anaemia are a heterogenous group of disorders, with different defects in haem synthesis, reduced activity of delta-aminolaevulinic acid synthetase being common but not invariable in both types. Pyridoxine-responsive (congenital) sideroblastic anaemia may be due to the presence of an abnormal ALA-synthetase apoenzyme which requires excessive amounts of the coenzyme, pyridoxal-phosphate, to achieve normal activity; other abnormalities of haem synthesis may also be present.
The purpose of this study was evaluation of the phagocytic ability of peripheral blood leucocytes and reticuloendothelial system of the lungs, liver and spleen after one infusion of plasma-substituting preparations: Fluigel and dextran 70 in volumes of 20 ml/kg of body weight. The experiments were done in rabbits. The response of the reticuloendothelial system was assessed on the basis of its ability of eliminating foreign erythrocytes labelled with 51Cr from the circulating blood and radioactivity accumulation in the liver and spleen. It was found that infusion of Fluigel and dextran produced transient changes in the function of the reticuloendothelial system with increased number of granulocytes and a fall of their radioactivity 3 and 6 hours after infusion, reduced ability of pulmonary 51Cr accumulation and increased storage in the spleen observed 24 hours after infusion.
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The authors carried out a clinical analysis of problems connected with splenectomy in elderly patients treated surgically at the Institute of Haematology in the years 1965-1972. Out of 24 patients aged above 60 years 12 were analysed clinically (8 females and 4 males). Splenectomy was done in these patients from haematological indications (haemolytic anaemia, thrombocytopenia, pancytopenia). In all these cases indications to splenectomy, the course of operation and postoperative period as well as late therapeutic results were assessed. The analysis of these elderly patients showed that the criteria of indications for splenectomy were the same as in other age groups. The choice of surgical method depended in these cases on the clinical and haematological findings, a lack of permanent improvement after conservative treatment, contraindications to immunosuppressive treatment and results of isotope investigations (particularly the values of the spleen-liver index). In most splenectomized patients there were no complications during the operation and in the postoperative period. In 2 cases only acute circulatory failure was observed immediately after the operation. Evaluation of late results showed that the effects of the operation were similar to those in other age groups.
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The level of cAMP was determined in isolated cells from bone marrow and peripheral blood of healthy subjects and patients with proliferative syndrome (acute and chronic myeloid leukaemia and chronic lymphatic leukaemia). In the investigations tritiated cAMP (3H-cAMP) was used and for binding of endogenous as well as exogenous cAMP protein isolated from bovine muscles was used. The mean cAMP level in peripheral blood granulocytes of healthy subjects was 27.90+/-3.82 pmol/10(7) cells and in normal lymphocytes it was from 11 to 18 pmol/10(7) cells. Much higher concentrations of cAMP: 56.4+/-16.25 and 52.7+/-11.02 pmol/10(7) cells were observed in myelocytes and metamyelocytes isolated from the bone marrow of healthy subjects. Lowering of cAMP concentration (below 4 pmol/10(7) cells) was observed in the lymphocytes of patients with chronic lymphatic leukaemia, while a higher cAMP concentration (above 90 pmol/10(7) cells) was found in the myeloblasts of patients with acute myeloid leukaemia.
A case of Hodgkin's disease was observed in the Warsaw Institute of Haematology. Involvement of the skin developed ++during uncontrolled and refractory pathological process, despite multiple changes of cytostatic programmes. The histological type of the lesions, nodular sclerosis, suggested a milder course of the disease.
On the ground of follow-up extended over many years the effectiveness was analysed of splenectomy in 93 children with idiopathic thrombocytopenia. Good and very good results of splenectomy were achieved in 84.95% of the cases treated surgically, in 15.05% no remission developed. In 27 splenectomized children recurrences appeared from 1 to 18 years after splenectomy. In 77.8% of cases the recurrences were transient, while in the remaining children qualified into the group with absent remission recurrences persisted throughout the whole follow-up period. In 3 children with unsatisfactory result of splenectomy accessory spleen was removed during repeated laparotomy, in two of them cure was obtained. No greater complications connected directly with the operation were noted. Severe viral and bacterial infections from 2 to 3 years after splenectomy developed in 3 children (3.23%) in 2 cases they were the cause of death (2.15%). Of decisive importance for good prognosis in children with this disease treated by splenectomy was normalization of platelet count immediately after the operation, preoperative disease duration not longer than 12 months, age below 10 years at the time of splenectomy, female sex, and spleen-liver index over 3.4.
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