Pub Date : 2009-04-24DOI: 10.1111/J.1600-0609.1981.TB01369.X
H. Büller, A. H. Weenink, P. Treffers, L. Kahlé, H. A. Otten, J. T. ten Cate
Severe acquired antithrombin III (AT III) deficiency was observed in a patient with severe pre-eclamptic toxaemia. Plasma AT III concentration of 0.25 U/ml was found in both functional and immunological assays. The patient was treated with human AT III concentrate as a result of the development of progressive disseminated intravascular coagulation (DIC), the further deterioration of renal function, the risk for thromboembolic complications and the possible adverse effects of heparin therapy. The selective correction of AT III activity resulted in a rapid disappearance of coagulation abnormalities. The patient underwent uncomplicated caesarian section. This observation indicates that acquired severe AT III deficiency may occur as an early feature of DIC in severe pre-eclamptic toxaemia.
{"title":"Severe antithrombin III deficiency in a patient with pre-eclampsia. Observations on the effect of human AT III concentrate transfusion.","authors":"H. Büller, A. H. Weenink, P. Treffers, L. Kahlé, H. A. Otten, J. T. ten Cate","doi":"10.1111/J.1600-0609.1981.TB01369.X","DOIUrl":"https://doi.org/10.1111/J.1600-0609.1981.TB01369.X","url":null,"abstract":"Severe acquired antithrombin III (AT III) deficiency was observed in a patient with severe pre-eclamptic toxaemia. Plasma AT III concentration of 0.25 U/ml was found in both functional and immunological assays. The patient was treated with human AT III concentrate as a result of the development of progressive disseminated intravascular coagulation (DIC), the further deterioration of renal function, the risk for thromboembolic complications and the possible adverse effects of heparin therapy. The selective correction of AT III activity resulted in a rapid disappearance of coagulation abnormalities. The patient underwent uncomplicated caesarian section. This observation indicates that acquired severe AT III deficiency may occur as an early feature of DIC in severe pre-eclamptic toxaemia.","PeriodicalId":21489,"journal":{"name":"Scandinavian journal of haematology","volume":"16 1","pages":"81-6"},"PeriodicalIF":0.0,"publicationDate":"2009-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81497748","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2009-04-24DOI: 10.1111/J.1600-0609.1983.TB02128.X
K. Bendix‐Hansen, Henning Kaspersen Nielsen
Myeloperoxidase (MPO) activity of blood granulocytes was estimated in 96 cases of acute myeloid leukaemia (AML), in 35 patients obtaining complete remission and in 14 of these patients during later relapse. As the pretreatment value of MPO activity was the same in patients who died before obtaining remission as in patients obtaining complete remission, determination of MPO-deficient PMN has no prognostic value with respect to the probability of obtaining complete remission. While half of the untreated patients had increased numbers of MPO-deficient PMN in the blood, all patients in complete remission had normal MPO activity (P = 0.0002). A normalization of the MPO activity after induction therapy, therefore suggests remission. Positive correlations could be demonstrated between an initially abnormal MPO activity and abnormal activity at relapse as well as between an initially normal MPO activity and normal activity at relapse (P = 0.0004). It is concluded that determination of the % of MPO-deficient PMN in the blood, may be a useful indicator of complete remission in AML, and in serial determinations during the remission phase also an indicator of threatening relapse.
{"title":"Myeloperoxidase-deficient polymorphonuclear leucocytes. (II) Longitudinal study in acute myeloid leukaemia, untreated, in remission and in relapse.","authors":"K. Bendix‐Hansen, Henning Kaspersen Nielsen","doi":"10.1111/J.1600-0609.1983.TB02128.X","DOIUrl":"https://doi.org/10.1111/J.1600-0609.1983.TB02128.X","url":null,"abstract":"Myeloperoxidase (MPO) activity of blood granulocytes was estimated in 96 cases of acute myeloid leukaemia (AML), in 35 patients obtaining complete remission and in 14 of these patients during later relapse. As the pretreatment value of MPO activity was the same in patients who died before obtaining remission as in patients obtaining complete remission, determination of MPO-deficient PMN has no prognostic value with respect to the probability of obtaining complete remission. While half of the untreated patients had increased numbers of MPO-deficient PMN in the blood, all patients in complete remission had normal MPO activity (P = 0.0002). A normalization of the MPO activity after induction therapy, therefore suggests remission. Positive correlations could be demonstrated between an initially abnormal MPO activity and abnormal activity at relapse as well as between an initially normal MPO activity and normal activity at relapse (P = 0.0004). \u0000 \u0000 \u0000 \u0000It is concluded that determination of the % of MPO-deficient PMN in the blood, may be a useful indicator of complete remission in AML, and in serial determinations during the remission phase also an indicator of threatening relapse.","PeriodicalId":21489,"journal":{"name":"Scandinavian journal of haematology","volume":"81 1","pages":"5-8"},"PeriodicalIF":0.0,"publicationDate":"2009-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84271023","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2009-04-24DOI: 10.1111/J.1600-0609.1969.TB01795.X
M. D. S. Özsoylu
A three-year-old Turkish boy with haemoglobin S-D disease and ventricular septal defect is reported in detail, mentioning his three siblings with the same disorder. The patient showed marked haemolytic anaemia and growth retardation. His father possibly had homozygous Hb D disease, and the mother had sickle cell trait. An aplastic crisis was found during his hospitalization.
{"title":"Haemoglobin S‐D Disease in a Turkish Family","authors":"M. D. S. Özsoylu","doi":"10.1111/J.1600-0609.1969.TB01795.X","DOIUrl":"https://doi.org/10.1111/J.1600-0609.1969.TB01795.X","url":null,"abstract":"A three-year-old Turkish boy with haemoglobin S-D disease and ventricular septal defect is reported in detail, mentioning his three siblings with the same disorder. The patient showed marked haemolytic anaemia and growth retardation. His father possibly had homozygous Hb D disease, and the mother had sickle cell trait. An aplastic crisis was found during his hospitalization.","PeriodicalId":21489,"journal":{"name":"Scandinavian journal of haematology","volume":"37 1","pages":"10-14"},"PeriodicalIF":0.0,"publicationDate":"2009-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81105760","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2009-04-24DOI: 10.1111/J.1600-0609.1983.TB01465.X
E. Øster-jørgensen, M. Rønne, N. Jensen, N. Grunnet
: A 38-year-old female suffered for several months from persistent fever without signs of infectious or immunologic disease. 57Co-bleomycin scintigraphy revealed abnormal concentration in the liver. Following a diagnostic laparotomy, the patient died from abdominal haemorrhage. Malignant cell infiltration was demonstrated in a biopsy from the liver. Histiocytic cells were demonstrated in a bone marrow smear obtained shortly before death. Investigation of cultured lymphocytes obtained from the patient the day before she died showed deficiency of T-lymphocytes. Investigations of chromosomes delineated several clones with polyploidi. The diagnosis of malignant histiocytosis was based on erythrophagocytosis, and immunologic and chromosomal aberrations. If malignant histiocytosis is the primary disease, a terminal stage of different diseases or a sign of complication secondary to immuno incompetence (e.g. virus infection) is an open question.
{"title":"Malignant histiocytosis. The diagnosis based on chromosomal, immunologic and histiopathologic analysis.","authors":"E. Øster-jørgensen, M. Rønne, N. Jensen, N. Grunnet","doi":"10.1111/J.1600-0609.1983.TB01465.X","DOIUrl":"https://doi.org/10.1111/J.1600-0609.1983.TB01465.X","url":null,"abstract":": A 38-year-old female suffered for several months from persistent fever without signs of infectious or immunologic disease. 57Co-bleomycin scintigraphy revealed abnormal concentration in the liver. Following a diagnostic laparotomy, the patient died from abdominal haemorrhage. Malignant cell infiltration was demonstrated in a biopsy from the liver. Histiocytic cells were demonstrated in a bone marrow smear obtained shortly before death. Investigation of cultured lymphocytes obtained from the patient the day before she died showed deficiency of T-lymphocytes. Investigations of chromosomes delineated several clones with polyploidi. The diagnosis of malignant histiocytosis was based on erythrophagocytosis, and immunologic and chromosomal aberrations. If malignant histiocytosis is the primary disease, a terminal stage of different diseases or a sign of complication secondary to immuno incompetence (e.g. virus infection) is an open question.","PeriodicalId":21489,"journal":{"name":"Scandinavian journal of haematology","volume":"129 4 1","pages":"167-173"},"PeriodicalIF":0.0,"publicationDate":"2009-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83647820","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2009-04-24DOI: 10.1111/J.1600-0609.1969.TB01860.X
v.m.d. Helge Stromorken
{"title":"The Release Reaction of Secretion: A General Basic Phenomenon Related to Phagocytosis/Pinocytosis","authors":"v.m.d. Helge Stromorken","doi":"10.1111/J.1600-0609.1969.TB01860.X","DOIUrl":"https://doi.org/10.1111/J.1600-0609.1969.TB01860.X","url":null,"abstract":"","PeriodicalId":21489,"journal":{"name":"Scandinavian journal of haematology","volume":"34 1","pages":"3-24"},"PeriodicalIF":0.0,"publicationDate":"2009-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77943520","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2009-04-24DOI: 10.1111/J.1600-0609.1977.TB02113.X
F. Cavalli, R. Sonntag, K. Brunner
{"title":"ACUTE LEUKAEMIA IN HODGKIN'S DISEASE","authors":"F. Cavalli, R. Sonntag, K. Brunner","doi":"10.1111/J.1600-0609.1977.TB02113.X","DOIUrl":"https://doi.org/10.1111/J.1600-0609.1977.TB02113.X","url":null,"abstract":"","PeriodicalId":21489,"journal":{"name":"Scandinavian journal of haematology","volume":"1 1","pages":"319-320"},"PeriodicalIF":0.0,"publicationDate":"2009-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79788452","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2009-04-24DOI: 10.1111/J.1600-0609.1969.TB02408.X
M. Norberg
The oxalate-induced nuclear hypersegmentation in neutrophils may be recorded by counting the segments. In heparinized blood, the number of segments remains fairly constant during incubation at 37° C. for at least 6 hours. Temperature depression and demecolcine decrease the number of segments recorded. Under identical experimental conditions, the formation of radial-segmented (RS) nuclei in mononucleated blood cells is promoted by incubation at room temperature and inhibited by treatment with demecolcine. Cold (+4° C.) and demecolcine at room temperature induce segmental clumping in the nuclei of neutrophils. The possible role of cytoplasmic microtubules in neutrophil segmentation is discussed.
{"title":"Neutrophil Segmentation and Radial Segmentation","authors":"M. Norberg","doi":"10.1111/J.1600-0609.1969.TB02408.X","DOIUrl":"https://doi.org/10.1111/J.1600-0609.1969.TB02408.X","url":null,"abstract":"The oxalate-induced nuclear hypersegmentation in neutrophils may be recorded by counting the segments. In heparinized blood, the number of segments remains fairly constant during incubation at 37° C. for at least 6 hours. Temperature depression and demecolcine decrease the number of segments recorded. Under identical experimental conditions, the formation of radial-segmented (RS) nuclei in mononucleated blood cells is promoted by incubation at room temperature and inhibited by treatment with demecolcine. Cold (+4° C.) and demecolcine at room temperature induce segmental clumping in the nuclei of neutrophils. The possible role of cytoplasmic microtubules in neutrophil segmentation is discussed.","PeriodicalId":21489,"journal":{"name":"Scandinavian journal of haematology","volume":"75 1","pages":"274-279"},"PeriodicalIF":0.0,"publicationDate":"2009-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83850606","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2009-04-24DOI: 10.1111/J.1600-0609.1983.TB01523.X
Pierguido Sala, E. Tonutti, S. Mazzolini, G. Antonutto, M. Bramezza
The occurrence of alpha-heavy chain disease in an Italian adult, working in Libya, is reported, characterized by a spontaneous clinical and immunological recovery. It is suggested that the patient could have acquired the disease during his stay in an endemic area of alpha-heavy chain disease, and that the observed spontaneous remission could be related to the removal of environmental pathogenic factors, following his departure from Libya.
{"title":"Alpha-heavy chain disease. Report of a case with spontaneous regression.","authors":"Pierguido Sala, E. Tonutti, S. Mazzolini, G. Antonutto, M. Bramezza","doi":"10.1111/J.1600-0609.1983.TB01523.X","DOIUrl":"https://doi.org/10.1111/J.1600-0609.1983.TB01523.X","url":null,"abstract":"The occurrence of alpha-heavy chain disease in an Italian adult, working in Libya, is reported, characterized by a spontaneous clinical and immunological recovery. It is suggested that the patient could have acquired the disease during his stay in an endemic area of alpha-heavy chain disease, and that the observed spontaneous remission could be related to the removal of environmental pathogenic factors, following his departure from Libya.","PeriodicalId":21489,"journal":{"name":"Scandinavian journal of haematology","volume":"151 1","pages":"149-154"},"PeriodicalIF":0.0,"publicationDate":"2009-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77789997","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2009-04-24DOI: 10.1111/J.1600-0609.1981.TB01405.X
C. Caranobe, P. Sié, C. Nouvel, G. Laurent, J. Pris, B. Boneu
Platelet serotonin (5-HT) uptake and storage in the presence and absence of reserpine were studied simultaneously with platelet volume, density and dense bodies content (mepacrine test) in 33 patients affected with myeloproliferative disorders (MD): 12 chronic myeloid leukaemia (CML), 9 polycythaemia vera (PV), 6 essential thrombocythaemia (ET) and 9 agnogenic myeloid metaplasia (AMM). Observations were (1) a dramatic reduction of the initial velocity (Vi) uptake and of the granular pool of 5-HT; (2) a slight reduction of the number of platelet dense bodies which, in many cases, were less fluorescent than in controls; (3) an increase of the percentage of light platelets while platelet volume was mostly normal; (4) a significant correlation between the number of dense bodies per platelet volume unit and either the percentage of light platelets (r = 0.76) or the size of the granular pool of 5-HT (r = 0.81). These results support evidence of a quantitative and qualitative acquired storage pool syndrome in these patients. In addition, the Vi studies demonstrate that the serotonin uptake across the plasmatic membrane is abnormal.
{"title":"Platelets in myeloproliferative disorders. II. Serotonin uptake and storage: correlations with mepacrine labelled dense bodies and with platelet density.","authors":"C. Caranobe, P. Sié, C. Nouvel, G. Laurent, J. Pris, B. Boneu","doi":"10.1111/J.1600-0609.1981.TB01405.X","DOIUrl":"https://doi.org/10.1111/J.1600-0609.1981.TB01405.X","url":null,"abstract":"Platelet serotonin (5-HT) uptake and storage in the presence and absence of reserpine were studied simultaneously with platelet volume, density and dense bodies content (mepacrine test) in 33 patients affected with myeloproliferative disorders (MD): 12 chronic myeloid leukaemia (CML), 9 polycythaemia vera (PV), 6 essential thrombocythaemia (ET) and 9 agnogenic myeloid metaplasia (AMM). Observations were (1) a dramatic reduction of the initial velocity (Vi) uptake and of the granular pool of 5-HT; (2) a slight reduction of the number of platelet dense bodies which, in many cases, were less fluorescent than in controls; (3) an increase of the percentage of light platelets while platelet volume was mostly normal; (4) a significant correlation between the number of dense bodies per platelet volume unit and either the percentage of light platelets (r = 0.76) or the size of the granular pool of 5-HT (r = 0.81). These results support evidence of a quantitative and qualitative acquired storage pool syndrome in these patients. In addition, the Vi studies demonstrate that the serotonin uptake across the plasmatic membrane is abnormal.","PeriodicalId":21489,"journal":{"name":"Scandinavian journal of haematology","volume":"46 1","pages":"289-95"},"PeriodicalIF":0.0,"publicationDate":"2009-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90490711","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2009-04-24DOI: 10.1111/J.1600-0609.1981.TB01679.X
R. Manthorpe, B. Kofod, A. Wiik, O. Saxtrup, Svehag Se
In a prospective 9 month study at a medical department with special interest in immunology and rheumatology, 10 patients with spuriously low thrombocyte counts - pseudothrombocytopenia - were registered. The phenomenon was observed when the counting of platelets was performed on a Thrombocounter C apparatus or by manual counting using K2EDTA stabilized blood since platelet agglutination was considerably less pronounced when solution citratis glucosi fortis (ACD) was used as the anticoagulant. Results of our in vitro studies indicate that the mechanisms contributing to pseudothrombocytopenia involve the interaction of circulating immune complexes with platelet membrane Fc receptors causing agglutination. Fab'2-fragments of isolated patient IgG did not react with normal donor thrombocytes. This finding argues against agglutination due to true antiplatelet antibody activity.
{"title":"Pseudothrombocytopenia. In vitro studies on the underlying mechanism.","authors":"R. Manthorpe, B. Kofod, A. Wiik, O. Saxtrup, Svehag Se","doi":"10.1111/J.1600-0609.1981.TB01679.X","DOIUrl":"https://doi.org/10.1111/J.1600-0609.1981.TB01679.X","url":null,"abstract":"In a prospective 9 month study at a medical department with special interest in immunology and rheumatology, 10 patients with spuriously low thrombocyte counts - pseudothrombocytopenia - were registered. The phenomenon was observed when the counting of platelets was performed on a Thrombocounter C apparatus or by manual counting using K2EDTA stabilized blood since platelet agglutination was considerably less pronounced when solution citratis glucosi fortis (ACD) was used as the anticoagulant. Results of our in vitro studies indicate that the mechanisms contributing to pseudothrombocytopenia involve the interaction of circulating immune complexes with platelet membrane Fc receptors causing agglutination. Fab'2-fragments of isolated patient IgG did not react with normal donor thrombocytes. This finding argues against agglutination due to true antiplatelet antibody activity.","PeriodicalId":21489,"journal":{"name":"Scandinavian journal of haematology","volume":"8 1","pages":"385-92"},"PeriodicalIF":0.0,"publicationDate":"2009-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86525066","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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