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  LETTERS TO THE EDITOR 259 Letter to the ditor A RETROSPECTIVE STUDY OF ACUTE PLASMA EXCHANGE IN SEVERE INTRAVASCULAR HEMOLYSIS To the Editor Plasma exchange (PE) has been used as a form of treatment in conjunction with various diseases and with varying results (1). In the treatment of intravascular hemolysis, PE has been described as an adjuvant to conventional therapy (2). The etiology of intravascular hemolysis varies and, in the present retrospective study, 13 cases of acute severe intravascular hemolysis are described that were triggered-off by various mecha- nisms. All patients suffering from severe intravascular hemolysis and treated by PE therapy at Orebro Med- ical Center Hospital between 1980 and 1986 have been included in this retrospective study. PE was, already in 1980, an established form of therapy in the treatment of various diseases at our hospital and therefore prob- ably all patients suffering from excessive hemolysis have been considered for PE treatment. The imminent risk of death, despite intensive conventional therapy, was the primary indication for PE treatment in cases no 1 8. In cases no 9-11, no response to conventional treatment was achieved and PE therapy was therefore instigated. In cases no 12-13, surgical intervention was considered inadvisable due to gross hemolysis in the patients. In all cases, acute PE therapy was employed to rapidly eliminate toxic breakdown products and eventual hemolysins (Table 1) from the circulation. PE was performed by a centrifugation technique using Hemonetic Cell Separator@ (Hemonetics Corporation Inc., Braintree, Mass., USA . A typical PE treatment comprized the removal of approx. 2.5 liters of patient plasma. Replacement was accomplished by intermittent infusion of 0.5-1.0 liters of 4 or 5 albumin in 0.9 saline solution with 2750 mg Calcium glubionate (4.46 mmol Ca ) additive and with 1.5-2.0 liters of donor plasma until full substitution. Donor plasma was selected according to each patient's essential require- ments; however, stored non-frozen plasma was pre- dominantly used. Routine laboratory investigations were regularly monitored before, during and after each series of PE treatments. In all, 12 severely ill patients (13 cases with 1 patient treated twice) were treated with PE therapy in conjunction with other forms of conventional treat- ment. The number of plasma exchanges performed on each patient varied considerably, but in 9 of 13 cases only five PE's or less were required for the temporary obviation of hemolysis. Clinical evaluation of the pa- TABLE 1 erological findings in 3 cases treated by plasma exchange Direct anti- Case no globulin test Polyspeci fic Monospecific anti- Serum antibodies anti-IgG+C3d IgM IgG C4 c3 C3d 1 2 3 4 5 6 8 9 10 11 12 13 (+) nd t +I nd nd nd nd 9 t (+) (+) Alloantibody anti-c,K Anti-Jk, Autoantibody, unidentified No antibody detected Autoantibody anti-I Autoantibody anti-I nd nd Autoantibody, unidentified Autoantibody, unidentified No antibody detected nd nd Agglutinin reactions: - negative. (+) 5 cells agglutinated. 6 10 cells agglutinated. small macroscopic agglutination. large macroscopic agglutination. complete agglutination. nd = not done.    m  m  Sum acad   4  4  TA 2 g v   Clncaaaodan   crebphma eh C No oSuoHmoon g  dou Sum bunumoSum cenn umo cncfoowu 4  no  P o hmoys S FAbBoePAePBoePAePBoePAePBoePAeP Bloramfuoro 1 1  6 1 nnnn 76 Ce 22 87 9 25 2 11 Ce 36 +   36353133 Ce 43 4 1 5 11  7527 Ce   Ineo 52   5 9 5 4 6611 Cebdewmoh 69 58188118 Ce 7 1  612 1 817 nDewhnod 82 79172133 Celaeaeaso  uommu 95 48193185 Ce 1 1   783 1 52 1 1  8 Ce 18   69341   81 Impobdeaefodn a scmia Otheoo 14   78144244 Ceareae 1   91 n 4 n 3 nImporeaebdeaefvdnDC$N =  soenopamaF =  ehfopamaAb =  abminsouo 4  o 5 . n =  n d  LETTERS TO THE EDITOR 261 tient immediately after each series of PE was good in all cases. However, the general condition of cases no 7, and 13 rapidly deteriorated and all 3 patients died within d after the last PE treatment. In the remaining 10 cases there were no signs of recurrence of the hemolysis and therefore treatment was considered suc- cessful. None of the 12 patients showed any detri- mental effects which could be related to the PE. The clinical and laboratory data of all 13 cases before and after PE therapy is shown in Table 2. The serological findings regarding the detection of erythrocyte-bound and circulating serum antibodies on commencement of PE is shown in Table 1. The treatment approach to acute intravascular hemo- lysis is two-fold. n he one hand, treatment is focused on neutralizing the triggering mechanism that induces the hemolysis and, in severe cases, compensating even for the resulting red cells destruction; and, on he other hand, reducing the risk of complications that can result from the hemolysis itself. The choice of replacement fluids for PE is under discussion (3). If an intensive and prolonged series of PE is conducted and albumin is used as the sole source of substitution, the coagula- tion-, immunoglobin- and complement factor levels are reduced (4, 5). In this study, donor plasma was used for substitution in all patients and during the study period more than 340 patients have been treated by PE with donor plasma at our center. No case of clinical hepatitis or HIV infection has been diagnosed in any of these patients. Some of the patients have also been specifically tested for hepatitis B and HIV markers and found to be negative. The risk for plasma-transmitted diseases has yet to be considered in the recently treated patients 6). The choice of replacement fluid must, however, be carefully considered in each individual patient according to the possible risks involved and the advantages gained. In conclusion, PE was found to have a positive effect in most of the 13 cases presented in this study. Due to the severity of the patients’ underlying diseases, the outcome with 3 mortalities is still regarded as being successful and fully justifying the medical risk and costs of the PE treatment. We do not state that PE by itself can cure the patient, but PE temporarily ‘normal- izes’ the condition in the patient. PE can also improve the condition by removing some of the circulating trigger substances and the harmful breakdown prod- ucts resulting from the trigger substances. Thus PE treatment provides a better biochemical support for the conventional therapy to have effect, and if PE is per- formed in severe intravascular hemolysis according to the guidelines presented here, then this may result in a better survival rate and a shorter treatment period. eferences 1. Shumak KH, Rock GA. Therapeutic plasma exchange. N Engl J Med 1984;12:762-71. 2. Sokol RJ, Hewitt S Stamps BK. Autoimmune hemolysis: Mixed warm and cold antibody type. Acta Haemat 3. Nydegger VE. Choice of the replacement fluid during large volume plasma exchange. Res Clin Lab 1983;13:103-10. 4. Keller AJ, Urbaniak SJ. Intensive plasma exchange on the cell separator: Effects on serum immunoglobulins and com- plement components. Br J Haematol 1978;38:531-5. 5. Beyer JH, Klee M, Kosterling H, Nagel GA. Coagulation studies before, during and after repeated plasma exchanges with a 5 albumin/saline solution in normal donors. In: Siegberth HG, ed. Plasma Exchange. Stuttgart, New York FK Schattaur Verlag, 1980;87. 6. Kiprov D, Simpson D, Romaninck-Schmiedl S, Lipert R, Spira T Busch D. Risk of AIDS-related virus (Human Immunodeficiency Vis transmission through apheresis procedures. J Clin Apheresis 1987;3:143-6. 1983;69:266-74. Correspondence to: Hans Fredlund, Olle Berskus, Elisabeth Bjorsell-bstlilng Derek Filbey The Blood Transfusion Center Orebro Medical Center Hospital S-701 85 Orebro Sweden
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