Separation of peripheral blood derived stem cells in children.

We performed 28 separations in 10 children from 14 to 44 kg (median: 24 kg) using either the Cobe Spectra or the Fresenius AS 104 cell separator. For children below 20 kg, human albumin solution was used for the last 200 ml of the priming procedure instead of NaCl. Blood flow was reduced to 30 - 50 ml/min depending on the childrens' size and weight to prevent citrate reactions. Within 149 - 337 minutes we processed 3 x the patients' total blood volume and collected 326.9 x 10(6) (76.5-1, 140.0) MNC and 2.03 x 10(6) (0.14-10.12) CD34+ cells per kg body weight. These results were comparable to the results we previously obtained in adults. We conclude from these initial results that PBSC separations with both devices can be adapted successfully to the needs for paediatric patients.

Cerebral vein thrombosis and prothrombin gene (G20210A) mutation.

Recently, prothrombin gene mutation G20210A has been associated with elevated thrombosis risk and rarely with cerebral vein thrombosis (CVT). Three patients are described who had this genetic predisposition and who developed CVT in an unusual constellation with other factors. In the first patient, the intake of valproic acid (VPA) may have played an aggravating role in the development of CVT; in the second patient diagnosis of coagulation disorder was made during pregnancy consultation 6 years after CVT; in the third patient the CVT occurred at the age of 78 years. In patients with CVT, coagulation-examinations should include tests for the prothrombin gene (G20210A) mutation.

The detrimental role of colloidal volume substitutes in severe ovarian hyperstimulation syndrome: a case report.

Although, the mechanisms for the development of ovarian hyperstimulation syndrome (OHSS) are still not clear, the symptoms usually correlate with the levels of serum estradiol and ovarian enlargement. We report a case, where the clinical course was unusually prolonged. When menstrual bleeding had already occurred, serum estradiol was less than 10 pg/ml and the ovaries were almost normal in size, the patient developed pleural effusion and a significant alteration in blood-coagulation. This was most likely caused by an over-infusion of hydroxyethyl starch (HES) over 10 days. The pleural effusion contained high levels of HES, reaching 74% of the plasma concentration as measured by a novel method after acidic hydrolysis of HES. Carbohydrates as dextran and HES are well known to interact with the blood-coagulation system. Increase capillary permeability, typical of OHSS, leads to loss of colloidal substances into the third space, where HES is slowly degraded and osmotic pressure is high. This might prolong and aggravate the urine of OHSS.

[Acute abdomen in immune complex vasculitis]. / Akutes Abdomen bei Immunkomplex-Vaskulitis

The case of a 51 year old male with 6 months history of loss of weight and abdominal pain is discussed. Laparotomy revealed gangrene of the gall bladder and perforation of the small intestine with consecutive peritonitis. Postmortem examination showed hypertensive intracerebral bleeding and disseminated infarctions of the abdominal organs and the kidneys. Histological findings including immune-fluorescence methods revealed the final diagnosis of immune complex vasculitis.

[Influence of cytostatic combination therapy with vincristine sulphate and iphosphamide on blood coagulation]. / Der Einfluss einer zytostatischen Kombinationsbehandlung mit Vincristinsulfat und Ifosfamid auf das Blutgerinnungssystem

Disorders of blood coagulation were investigated before and during a cytostatic combination therapy with vincristine sulphate and iphosphamide (Asta Z 4942) in 12 patients with malignant tumours or haemoblastoses. Thromboplastin time, partial thromboplastin time, thrombin time, heat-dependent fibrin, clot retraction, and clotting factors II, V, VIII, IX, X, and the platelet count were determined. A change in the plasmatic coagulation system attributable to the combination therapy could not be demonstrated in any patient. The influence of the cytostatic combination on the platelet-dependent haemostasis was small; a decrease in platelet count could be observed in only one patient, in whom an additional causative damage to thrombopoiesis due to the underlying disease could be assumed. Regardless of the cytostatic therapy there were indications of a hypercoagulability in 10 patients. This explains the increased susceptibility of such patients for thromboses or consumption coagulopathy.

[Clinical experiences with holoxan within the framework of sequential combination therapy (author's transl)]. / Klinische Erfahrungen mit Holoxan im Rahmen einer sequentiellen Kombinationstherapie.

153 patients with inoperable malignant tumors were treated by the so-called synchronization therapy with vincristine and ifosfamide. 13 complete remissions (8.5%) and 47 partial remissions (30.7%) were obtained. 5 times cessation of tumor growth occurred (3.3%). In 88 cases the therapy failed (57.5%), but in 11 cases a subjective improvement was noted. The most favourable results of treatment were obtained in cases of malignant lymphoma, small cell carcinoma of the lung and testicular carcinomas. Although the duration of remission was short in most cases, it could be demonstrated, that a targeted polychemotherapy is possible using the proliferation kinetic data in addition to the knowledge of the cell-phase-specific effect of cytostatic drugs. This therapy can succeed in causing a tumor regression even in metastatic carcinomas.

[Gastrectomy in congenital factor VII deficiency with substitution of factor VII concentrate]. / Gastrektomie bei kongenitalem Faktor-VII-Mangel unter Substitution von Faktor-VII-Konzentrat.

Case report of a 57-year old man, who had a severe congenital factor VII deficiency (factor VII 2%), and who was substituted with factor VII concentrate, before undergoing a subtotal gastrectomy. The patient was previously asymptomatic. The operation and wound-healing was without complication. That the patient, despite his factor VII deficiency, showed no clinical signs of a bleeding diathesis, raises the question, whether in such a case a factor VII substitution is necessary. 3 children of the patient (2 sons aged 25 and 23 years, and an 18 year old daughter) were examined and found to be heterozygote carriers to a degree of 32%, 42% and 60% factor VII; none showed sure signs of a bleeding tendency related to factor VII deficiency.

[Disorders of hemostasis in liver cirrhosis. 2. Therapy]. / Hämostasestörungen bei Leberzirrhose. Teil 2: Therapie.

Hemostatic changes in liver cirrhosis regularly have complex causes. In addition to a quantitative deficiency of hemostatic factors, also qualitative changes in coagulation factors, disturbances in coagulation factor metabolism and possible iatrogenic disturbances in plasmatic and thrombocytic hemostatic mechanism are to be considered. To diagnose a deficit of hemostatic factors is no problem, but to answer the question which of the numerous pathogenetic factors dominates in an individual case at this moment is very difficult and often impossible.

[Postpartum hemophilia A with factor VIII inhibitor]. / Hemmkörperhämophilie A post partum.

An abnormal life-threatening haemorrhagic diathesis occurred 6 weeks after delivery in a 25-year-old female. The reason was a spontaneously acquired factor VIIIC inhibitor haemophilia. The clinical presentation was characterised by extensive deep-seated soft-tissue haemorrhages of the extremities, a retroperitoneal haemorrhage, haematuria and recurrent joint bleedings. The activity of factor VIIIC decreased to below 1% of normal. The factor VIII inhibitor reached a maximum of 122 Bethesda units. The recurrent knee joint haemorrhages responded well to treatment with an activated prothrombin complex concentrate (Feiba). Repeated Feiba administration did not lead to an increase of the factor VIII inhibitor. It disappeared completely within 16 months and did not recur during a second pregnancy. The pregnancy was without complications and delivery on time resulted in a healthy child. Six months after the second pregnancy both mother and child showed no evidence of a disorder of haemostasis.

[Treatment results of therapy resistant acute leukemias with 7 cystostatic agents (TRAMPCO-scheme)]. / Behandlungsergebnisse therapieresistenter akuter Leukosen mit 7 Zytostatika (TRAMPCO-Schema).

Treatment of therapy-resistant acute leukemia with 7 cytostatics (TRAMPCO) 13 adult patients with acute leukemia, primarily and secondarily resistant to other combined therapy, as well as 2 untreated patients were treated with 7 cytostatic drugs according to the so called TRAMPCO-regimen. 5 complete remissions and 5 partial remissions were obtained corresponding to a degree of response of 66%. The duration of remission was relatively short with 1 to 5 months, in one case more than 6 months. The toxicity of the combined therapy could be tolerated, the personal subjective tolerance was good. The Trampco-regiment therefore represents a realistic possibility in the treatment of acute leukemia resistant to other forms of treatment.

[What are the dangers of splenectomy in Hodgkin's disease?]. / Wie gefährlich ist die Splenektomie bei M. Hodgkin?

Splenectomy is a surgical procedure of medium severity, the mean lethality rate is 1%, the complication rate 10 to 20%. The surgical risk is dependent upon age and general condition of the patient, the severity of the disease, and the experience of the surgeon. The risk of late complications due to surgery is determined mainly by infections as well as ileus, requiring relaparatomy. The risk of infections is higher in children than in adults: one has to be aware of miningitis and sepsis in about 10% of the patients; half of those cases end lethal. An analysis of advantages versus risks of splenectomy must be made for each patient individually. For optimal treatment it is necessary to know the stage of the disease. Concerning M. Hodgkin, explorative laparatomy combined with splenectomy should be performed in stage I to III A. If, however, the surgical risk is rather high primarily and if there are no therapeutical consequences to be expected, splenectomy should not be performed because of the known risks and disadvantages.

[Autologous blood transfusion with deep frozen stored blood in cardiac surgery (author's transl)]. / Die autologe Bluttransfusion tiefkühlkonservierten Blutes in der Herzchirurgie.

Cardiac operations were made possible by deep freezing of autologous blood in 187 out of 262 cardiac patients (71%) using only the patients' own blood. In only 75 patients (29%) was additional homologous blood transfusion necessary. It should be possible to lower further the requirements for donor blood by using an even stricter indication for the transfusion of homologous blood and by further improvements in techniques and organization. Autologous blood donation and transfusion of deep frozen conserved own blood were both tolerated very well by patients with cardiac disease. The advantages of deep frozen conservation of autologous blood in cardiac surgery are so convincing that they fully compensate for disadvantages of high cost and work required.

[Heart surgery in a female patient with blood group Oh (Bombay phenotype)]. / Herzoperation bei einer Patientin mit der Blutgruppe Oh (Bombay-Phänotyp).

A 62-year-old woman with stenosing coronary artery disease had the rare blood group Oh (Bombay phenotype). After prophylactic deep-freeze conservation of autologous blood, direct myocardial revascularization was successfully accomplished under extracorporeal circulation. Three deep-freeze units of erythrocyte concentrates were used. Both operation and postoperative wound healing progressed without complication.