P. G. Frick

Familial disturbance of fibrin monomer aggregation.

The investigation of the cause of an accidentally observed prolonged prothrombin time in a patient without a haemorrhagic diathesis led to the discovery of a familial disturbance of the aggregation of fibrin monomers (von Felten, Duckert and Frick, 1965). The observed defect differs from previously reported anomalies in the last phase of coagulation such as congenital afibrinogenaemia or hypofibrinogenaemia (Frick and McQuarrie, 1954), congenital fibrin‐stabilizing‐factor (FSF) deficiency (Duckert, Jung and Shmerling, 1960), and congenital dysfibrinogenaemia (Beck, 1964). It may be similar to the fibrinogen anomaly described by Ménaché (1963).

Studies on Fibrin Monomer Aggregation in Congenital Dysfibrinogenaemia (Fibrinogen ‘Zürich’): Separation of a Pathological from a Normal Fibrin Fraction

Further characterization of the delay in aggregation of fibrinogen ‘Zürich’ showed a potentiation of the defect by increasing ionic strength and an acceleration by calcium ions or protamine. A new type of paracoagulation due to soluble abnormal fibrin monomers in fresh serum is described.

Recurrent Intravascular Coagulation with Renal Cortical Necrosis and Recovery

Excerpt Acquired hypofibrinogenemia is due either to a decreased rate of fibrinogen production in the liver or to an increased rate of disappearance from the circulating blood. The latter mechanism...

Autologous Hematologic Recovery from Aplastic Anemia following High Dose Cyclophosphamide and HLA-Matched Allogeneic Bone Marrow Transplantation

A 17-year-old woman with severe aplastic anemia was treated with high-dose cyclophosphamide followed by infusion of bone marrow cells from her HLA-identical, ABO-incompatible brother. The marrow graft failed to take. Subsequently, the patient revealed an autologous marrow reconstitution leading to a near-complete hematologic recovery which is now persisting for over 20 months.

Successful allogeneic bone marrow retransplantation with the same donor after graft rejection: Application of a modified conditioning regimen

ZusammenfassungEs wird über einen 26jährigen Patienten mit schwerer idiopathischer Panmyelopathie berichtet. Er erhielt nach Vorbehandlung mit Zyklophosphamid (200 mg/Kg K.G.) ein erstes Knochenmarkstransplantat seiner HLA-identischen Schwester, welches nach anfänglichem Angehen zwischen Tag 46 und 50 nach Transplantation abgestoßen wurde. Darauf folgte eine 4monatige vollständige Knochenmarkaplasie, welche der Patient unter strikter Isolation ohne Komplikationen überstand. Nach intensiverer Vorbehandlung (Zyklophosphamid 200 mg/kg K.G., Procarbazin 37.5 mg/Kg K.G.; Anti-Lymphozyten-Globulin „Pressimun“ 195 mg/kg K.G.) wurde eine zweite Knochenmarkstransplantation mit Zellen derselben Spenderin vorgenommen. Dies führte zu einer vollständigen hämatologischen Rekonstitution mit Zellen vom Spendertyp, welche jetzt bereits über 12 Monate anhält.SummaryA 26-year-old man with severe aplastic anemia was treated with high-dose Cyclophosphamide followed by the infusion of bone marrow cells from his HLA-identical sister. After initial take of the graft, rejection ensued by day 46 which was followed by a permanent complete aplasia. After 4 months, bone marrow retransplantation with the same donor was attempted after a more intensive conditioning regimen. This led to permanent engraftment with rapid normalization of the blood counts lasting now for over 12 months. The patient has since remained in excellent clinical condition without signs of graft-versus-host disease.

Clotting factor V activity in plasma cryoproteins

Plasma cryoprecipitates containing considerable quantities of clotting factor V were observed in five patients. The unusual factor V activity was not associated with significant amounts of cryofibrinogen. Clinically, the symptoms were not distinguishable from those of other cryopathies. The abnormality did not lead to a deficiency of factor V in the circulating blood nor to a haemorrhagic diathesis.

No Malabsorption of Inorganic Ferrous Iron in Patients with Achylia gastrica

No Malabsorption of Inorganic Ferrous Iron in Patients with Achylia gastrica Celada et al. [1] recently described in this journal a considerable malabsorption of 59pe2+ for subjects with achylia gastrica. The authors have used the same test dose of 0.56 mg 59Fe2+ and the technique of whole body counting of absorbed 5βFe as proposed by us [7] but their results are in contradiction with published work from our [3-6] and another laboratory [8]. Neither normal gastric juice nor an intrinsic factor is required for the intestinal absorption of 59Fe2+ or hemoglobin-39Fe in humans [3-6]. Subjects with histamine-re-fractive achylia gastrica and absolute intrinsic factor deficiency (pernicious anemia in remission) absorbed 27 ± 15% [3, 5, 6] and subjects with a partial gastrectomy (Billroth I and II) 32 ± 15% [6], which was identical with the absorption of 31 ± 12% as observed for the 10 μrnoi (= 0.56 mg) sθpe2+ test dose m normal subjects [2-6]. Magnusson did confirm our results since he found no indications for iron malabsorption from the 0.56 mg 59Fe2+-dose in patients with Billroth ∏-partial gastrectomy or an-trectomy and gastroduodenostomy with or without vagotomy [8]. 59Fe2+ malabsorption (5.1 ± 3.3%) was observed only in patients with a total gastrectomy [6]. Hemoglobin 59Fe was even better absorbed (2-fold) by subjects with achylia gastrica and absolute intrinsic factor deficiency (15 ± 5.6% from a 5 mg hemoglobin-Fe dose versus 7.5 ± 2.4% in normal subjects) [3, 5], since the acidity of normal gastric juice does reduce the bioavailability of hemoglobin-iron probably by heme-polymeri-zation and precipitation [4]. A 3-fold reduction of intrinsically 59Fe-labeled meat-(and liver-)iτon bioavailability was, however, demonstrated for subjects with gastric mucosa atrophy or Billroth II partial gastrectomy [4] and confirmed with an extrinsically 59Fe-labeled test meal for subjects after antrectomy or partial gastrectomy [8]. This reduced bioavailability is however caused by, e.g., meat iron mal-digestion and not by malabsorption since it can be corrected by an in vitro peptic pre-digestion of the 59Fe-labeled pork [4]. The normal absorption of ferrous iron, the doubled hemiglobin-iron absorption and the considerable reduction of meatand liver-iron favour the assumption that dietary iron maldigestion rather than malabsorption causes the development of iron deficiency in patients with achylia gastrica or partial gastrectomy [4]. Patients with achylia gastrica No Malabsorption of Inorganic Ferrous Iron in Patients with Achylia gastrica 57 and iron deficiency anemia do absorb iron from therapeutic oral ferrous sulphate iron preparations like normal subjects and are not resistant to oral iron therapy and do not require parenteral iron. References Celada, A.; Rudolf, H.; Herreros, V., and Donath, A.: Inorganic iron absorption in subjects with iron deficiency anemia, achylia gastrica and alcoholic cirrhosis using a whole body counter. Acta haemat. 60: 182-192 (1978).

Fibrinogen “Zurich” Separation of a Pathological from a Normal Fibrin Fraction

Publisher Summary This chapter describes unstable hemoglobin with a modified affinity for oxygen in a French patient. This hemoglobin has been found in three members of the same family and is accompanied by identical symptoms that include a moderate hemolytic anemia, slight reticulocytosis, and the presence of red cell inclusion bodies. Electrophoresis of this hemoglobin at pH 8.6 shows a somewhat diffuse band representing 10–15% of the total, migrating a little in front of hemoglobin A 2 . There is also a very slow constituent to be seen, which could be free α-chain. This hemoglobin is unstable. Different stability tests have been studied after the initial elimination of nonhem proteins. This hemoglobin is thermolabile and a seizable precipitate is also obtained by incubation in the presence of para-hydroxy-mercuribenzoate. Purification of the supplementary peak shows a peptide similar to βT5 in its composition but modified specifically by the absence of an aspartic residue and a proline residue