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Chronic hepatitis C treated with phlebotomy alone: biochemical and histological outcome.

BACKGROUND: In patients with chronic hepatitis C, the histological outcome of long term phlebotomy is unknown. AIM: To investigate biochemical and histological findings before and after phlebotomy in chronic hepatitis C. PATIENTS: Twenty-four non-haemochromatotic patients with chronic hepatitis C were treated with long-term phlebotomy alone. RESULTS: Hepatic iron concentration had decreased in all patients who underwent a second liver biopsy, two years after iron depletion was attained and maintained. Histological grading score decreased in four patients, was unchanged in two, and increased in five. Histological staging score decreased in two patients, was unchanged in five, and increased in four. Pretreatment high serum selenium level predicted the reduction of the inflammatory grading score in univariate analysis (p=0.008, while low serum aspartate aminotransferase (p=0.02) and low propeptide of procollagen III (p=0.08) levels predicted the lack of progression of liver fibrosis. Furthermore, when iron depletion was reached, significant reductions of serum levels of aminotransferase, gamma glutamyl transferase (-47%), propeptide of procollagen III, alpha foetoprotein, selenium were observed in 24 patients. No changes in serum hepatitis C virus-RNA levels were found. CONCLUSIONS: Phlebotomy alone seems to be efficacious in suppressing progression of chronic hepatitis C in some patients. Phlebotomy not only induces iron depletion, but it even modifies serum levels of other trace elements involved in the balance between oxidant and antioxidant processes.

Adult↗

Efficacy and safety of phlebotomy to reduce transfusional iron overload in adult, long-term survivors of acute leukemia.

BACKGROUND: Transfusional iron overload is a frequent finding in long-term survivors of acute leukemia (AL). Only a few studies have reported the results of iron depletion therapy in this category of patients. STUDY DESIGN AND METHODS: Between January 1996 and July 2003, 26 consecutive adult patients who achieved complete remission of AL and developed transfusional iron overload underwent a weekly phlebotomy program at our transfusion center. Serum ferritin levels and transferrin saturation were monitored during the iron depletion therapy and the follow-up period. These AL patients were also checked for the presence of 12 hereditary hemochromatosis (HH) gene mutations. RESULTS: After a mean follow-up of 57.8 months, therapeutic phlebotomy (mean number of units collected, 36.6) was effective in reducing mean ferritin concentration and transferrin saturation from 1726.9 to 93.0 mg per L and from 54.7 to 23.3 percent, respectively. The presence of a HH gene mutation did not influence initial iron status or response to treatment. The phlebotomy program was well tolerated and no adverse events were recorded during or after collection. In three cases the time between phlebotomies was increased because of patient's poor compliance or low Hb levels. CONCLUSION: Our study shows that phlebotomies are a safe and effective method for reducing iron over-load in multiply transfused long-term AL survivors with secondary hemochromatosis.

Adult↗

Prospective crossover study of the effect of phlebotomy and intravenous crystalloid on hematocrit.

OBJECTIVE: To compare the changes in hematocrit (Hct) between phlebotomized and nonphlebotomized individuals given IV crystalloid. METHODS: A prospective, crossover volunteer study was performed comparing Hct changes immediately and 30 minutes after IV crystalloid bolus in 20 healthy adults with and without prebolus phlebotomy. In the control portion, volunteers were given a 15-mL/kg bolus of normal saline over 30 minutes with Hct determination before (H1), immediately after (H2), and 30 minutes after (H3) crystalloid infusion. At least 7 days later, the same subjects were phlebotomized 1 unit of blood and then administered a 15-mL/kg IV bolus of normal saline 30 minutes later. Hcts were obtained before (H4) and 30 minutes after (H5) phlebotomy (immediately prior to crystalloid infusion). Hcts were also obtained immediately after (H6) and 30 minutes after (H7) crystalloid infusion. A post-hoc test performance analysis was then performed to determine the Hct drop thresholds that would yield the maximal sensitivity and specificity for 500 mL of blood loss (via phlebotomy) in this population. RESULTS: The Hct (%) drops in the nonphlebotomized individuals receiving IV fluids averaged 4.5 +/- 1.3 immediately and 3.2 +/- 1.3 30 minutes after infusion. These drops were different (p < 0.05) from the Hct drop in individuals receiving IV fluids after phlebotomy, which averaged 6.6 +/- 1.5 and 5.7 +/- 1.1, respectively. Post-hoc analysis revealed that Hct drops of 5.4 immediately, or 4.3 at 30 minutes after infusion, had a sensitivity of > 90% and a specificity of 75% for identification of patients in the phlebotomy group. CONCLUSIONS: The practice of measuring serial Hcts may be helpful to identify trauma patients with occult blood loss. A prospective clinical trial is needed to validate these Hct drop thresholds (immediate and 30 minutes postinfusion) in crystalloid-resuscitated trauma patients.

Adult↗

Patient compliance with phlebotomy therapy for iron overload associated with hemochromatosis.

OBJECTIVE: The aim of this study was to evaluate patient compliance with phlebotomy therapy of hemochromatosis-associated iron overload. METHODS: We reviewed medical records of white adults with hemochromatosis and iron overload diagnosed during medical care. We defined three elements of compliance: 1) achieving iron depletion (serum ferritin </= 20 ng/ml); 2) timeliness of phlebotomies on a weekly or biweekly schedule to achieve iron depletion; and 3) participation in therapy to maintain serum ferritin </= 50 ng/ml. We evaluated associations of timeliness of phlebotomy and participation in maintenance therapy with these variables: age at diagnosis, sex, pretreatment serum ferritin concentration, HFE genotype, units of blood removed to achieve iron depletion, and presence or absence of cirrhosis at diagnosis. RESULTS: A total of 118 patients were evaluable for iron depletion and 142 for maintenance therapy; 96.6% achieved iron depletion, and 33.1% and 43.2% followed weekly and biweekly schedules, respectively. Timeliness was not significantly associated with the variables we evaluated. In the first year of maintenance therapy, 84.0% of patients complied; the percentage of C282Y homozygotes complying was significantly greater than that of other patients. Average compliance with maintenance therapy decreased 6.8% annually; the mean follow-up after achieving iron depletion was 4.1 +/- 2.8 yr (range 0.6-9.7 yr). CONCLUSIONS: Most patients with hemochromatosis diagnosed in medical care achieve iron depletion with phlebotomy; one-third tolerate and adhere to weekly phlebotomy. There is a constant rate of decline in the percentage of patients who comply with maintenance therapy.

Adolescent↗

[Comparison between phlebotomy and erythrocytapheresis of iron overload in patients with HFE gene mutations].

BACKGROUND AND OBJECTIVE: Large-volume erythrocytapheresis (EA) is an useful and speedy method to treat iron overload (IO). We assesed the efficacy of EA in patients with HFE gene mutations and IO compared to the classical phlebotomies. PATIENTS AND METHOD: Data from 9 patients with IO treated with EA, using a discontinuous flow cell separator as a single needle procedure, for a period of 2 years, were compared to 9 matched patients who underwent conventional phlebotomies. RESULTS: The mean volume of red blood cells removed in each EA was 275 ml, with a median reduction of 23 g/l for haemoglobin and 55 microg/l for serum ferritin levels (vs. 17 microg/l between phlebotomies). The liver function test returned to normal values in 4 out of 5 patients undergoing EA, but none of the phlebotomies. The time required to achieve iron depletion was 3 times shorter in EA group. CONCLUSIONS: EA is an effective and safe procedure that achieves iron depletion more quickly than manual phlebotomies. Nevertheless, to determine its cost-effectiveness, economical, prospective, randomized studies are warranted.

Adult↗

Phlebotomy overdraw in the neonatal intensive care nursery.

OBJECTIVE: Because blood loss attributable to laboratory testing is the primary cause of anemia among preterm infants during the first weeks of life, we quantified blood lost attributable to phlebotomy overdraw, ie, excess that might be avoided. We hypothesized that phlebotomy overdraw in excess of that requested by the hospital laboratory was a common occurrence, that clinical factors associated with excessive phlebotomy loss would be identified, and that some of these factors are potentially correctable. DESIGN, OUTCOME MEASURES, AND ANALYSIS: Blood samples drawn for clinical purposes from neonates cared for in our 2 neonatal special care units were weighed, and selected clinical data were recorded. The latter included the test performed; the blood collection container used; the infant's location (ie, neonatal intensive care unit [NICU] and intermediate intensive care unit); the infant's weight at sampling; and the phlebotomist's level of experience, work shift, and clinical role. Data were analyzed by univariate and multivariate procedures. Phlebotomists included laboratory technicians stationed in the neonatal satellite laboratory, phlebotomists assigned to the hospital's central laboratory, and neonatal staff nurses. Phlebotomists were considered experienced if they had worked in the nursery setting for >1 year. Blood was sampled from a venous or arterial catheter or by capillary stick from a finger or heel. Blood collection containers were classified as tubes with marked fill-lines imprinted on the outside wall, tubes without fill-lines, and syringes. Infants were classified by weight into 3 groups: <1 kg, 1 to 2 kg, and >2 kg. The volume of blood removed was calculated by subtracting the weight of the empty collection container from that of the container filled with blood and dividing by the specific gravity of blood, ie, 1.050 g/mL. The volume of blood withdrawn for individual laboratory tests was expressed as a percentage of the volume requested by the hospital laboratory. RESULTS: The mean (+/- standard error of the mean) volume of blood drawn for the 578 tests drawn exceeded that requested by the hospital laboratory by 19.0% +/- 1.8% per test. The clinical factors identified as being significantly associated with greater phlebotomy overdraw in the multiple regression model included: 1) collection in blood containers without fill-lines; 2) lighter weight infants; and 3) critically ill infants being cared for in the NICU. Because the overall R(2) of the multiple regression for these 3 clinical factors was only.24, the random factor of individual phlebotomist was added to the model. This model showed that there was a significant variation in blood overdraw among individual phlebotomists, and as a result, the overall R(2) increased to.52. An additional subset analysis involving 2 of the 3 groups of blood drawers (ie, hospital and neonatal laboratory phlebotomists) examining the effect of work shift, demonstrated that there was significantly greater overdraw for blood samples obtained during the evening shift, compared with the day shift when drawn using unmarked tubes for the group of heavier infants cared for in the NICU. CONCLUSION: Significant volumes of blood loss are attributable to overdraw for laboratory testing. This occurrence likely exacerbates the anemia of prematurity and may increase the need for transfusions in some infants. Attempts should be made to correct the factors involved. Common sense suggests that blood samples drawn in tubes with fill-lines marked on the outside would more closely approximate the volumes requested than those without. Conversely, the use of unmarked tubes could lead to phlebotomy overdraw because phlebotomists may overcompensate to avoid having to redraw the sample because of an insufficient volume for analysis. We were surprised to observe that the lightest and most critically ill infants experienced the greatest blood overdraw. (ABSTRACT TRUNCATED)

Anemia↗

[Side effects of phlebotomy: pathophysiology, diagnosis, treatment and prophylaxis].

Although phlebotomy is on the whole a safe procedure, the frequency of side effects being low and their severity weak, there have still been rare incidents of serious accidents such as nerve injury, vasovagal reaction and infections. Medical staff performing phlebotomy must be aware of the pathophysiology of these side effects, be careful in avoiding them, and be trained to properly treat them. Nerve injury is the complication of which phlebotomists should take the greatest caution. It sometimes causes permanent motor and/or sensory nerve dysfunction of arms and hands. Appropriate selection of vein and careful procedure of venipuncture are required. Vasovagal reaction is a relatively common complication causing hypotension, palor and occasional syncope. For emergency cases, a bed, oxygen tanks, and a utility cart with drug supplies should be provided in the phlebotomy room. Infections, especially those by blood-borne pathogen, are rare but serious complications. When performing evacuated tube venipuncture procedure, blood collecting tubes sterilized on the inside and single-use holders must be used to avoid infections through backflow of blood. Phlebotomists must follow the procedure presented in the guidelines to avoid backflow. Hematoma, allergy, hyperventilation, air embolism, anemia and thrombosis are other side effects occasionally caused by phlebotomy. Finally, medical staff should recognize that sincere communication with patients receiving phlebotomy is essential and efforts to inform them of the possibility of these side effects are becoming increasingly necessary in the current medical environment.

Brachial Plexus↗

Phlebotomy for pulmonary edema in dialysis patients.

STUDY OBJECTIVE: To assess the efficacy of phlebotomy in the treatment of pulmonary edema in hemodialysis patients. PROCEDURE: Maintenance hemodialysis patients presenting to the emergency room in respiratory distress from apparent pulmonary edema were assessed with regard to clinical response, change in blood pressure, change in hematocrit, and interval until the next hemodialysis treatment, RESULTS: Twenty-one patients underwent phlebotomy and seventeen improved markedly and did not require intubation or emergent dialysis. Hemodialysis was initiated 15.6 +/- 13.6 SD hours later. Four were able to have their treatment 24 or more hours later. Thirteen of 21 (62%) were hypertensive at the time of treatment and blood pressure tended to normalize in this subset. Four of 21 (19%) developed transient hypotension without permanent sequelae. Pre-mean hematocrit = 25.0 + 6.0 and post phlebotomy = 22.6 + 4.6 SD. All patients receiving phlebotomy survived to hospital discharge. CONCLUSION: Phlebotomy can often obviate the need for intubation or emergent dialysis in ESRD patients presenting with pulmonary edema.

Blood Pressure↗

Primary hemochromatosis: anatomic and physiologic characteristics of the cardiac ventricles and their response to phlebotomy.

M-mode and 2-dimensional echocardiography and gated equilibrium blood pool imaging (rest and exercise) were used in 10 patients with primary hemochromatosis to characterize the spectrum of pathophysiologic abnormalities of the cardiac ventricles and to determine the response to chronic therapeutic phlebotomy. Dilated and restrictive cardiomyopathic patterns were identified in 1 patient each, but our data do not permit conclusions on when in the natural history a given pattern becomes overt. On entry into study, 3 patients had normal ventricles and 7 had ventricular abnormalities on echocardiography and blood pool angiography. In 2 of the latter patients, biventricular dysfunction and increased left ventricular (LV) mass normalized after phlebotomy; 1 patient achieved a normal LV response to exercise. Of the 4 patients with isolated abnormal LV ejection fraction responses to exercise, the EF normalized in 2 after phlebotomy. In 1 patient, isolated right ventricular enlargement and dysfunction (echocardiographic and radionuclide imaging) normalized after phlebotomy. Thus, primary hemochromatosis can effect LV and RV size and function; clinically occult cardiac involvement can be identified by echocardiography and equilibrium blood pool imaging; therapeutic phlebotomy can ameliorate or reverse the deleterious effects of excess cardiac iron deposition which appears to exert its harm, at least in part, by a mechanism other than irreversible connective tissue replacement.

Adult↗

Improved exercise tolerance of the polycythemic lung patient following phlebotomy.

The present study evaluated the effects of therapeutic phlebotomy on the exercise tolerance and the maximal carbon dioxide output of polycythemic patients with chronic obstructive pulmonary disease. Fifteen maximal exercise studies were performed before and after phlebotomy in patients with moderate to severe chronic obstructive pulmonary disease (mean forced expiratory volume in one second [FEV1]= 970 ml). After phlebotomy there were no significant differences in pulmonary function, blood gases, oxygen consumption, or carbon dioxide production at rest. However, after phlebotomy there was a significant increase in the exercise tolerance of the patients. The mean workload, the duration of exercise, the maximal oxygen consumption, the maximal carbon dioxide production, and the ventilation at maximal exercise all increased significantly. The improved exercise tolerance after phlebotomy appeared due to an increased cardiac output generated mainly through an increased stroke volume. We hypothesize that the increased stroke volume was due to a higher ejection fraction of the right ventricle secondary to a lower pulmonary artery pressure. This study provides further evidence that patients with chronic obstructive pulmonary disease who have polycythemia benefit by therapeutic interventions that maintain their hematocrits below 55 percent.

Aged↗

Influence of phlebotomy treatment on abnormal hypothalamic-pituitary function in genetic hemochromatosis.

To test the hypothesis that deficiencies in hypothalamic-pituitary function in genetic hemochromatosis result from cellular injury by iron deposits, we conducted provocative tests in 11 men with genetic hemochromatosis before and after iron depletion by serial phlebotomy and in 10 control subjects. We gave combination intravenous injections of insulin (0.15 U/kg), luteinizing hormone releasing hormone (LHRH, 100 micrograms), and thyrotropin releasing hormone (400 micrograms) and then measured plasma glucose, growth hormone, corticosteroids, follicle-stimulating hormone, luteinizing hormone, prolactin, and thyroid-stimulating hormone at 30-minute intervals for 90 minutes. Phlebotomy caused a substantial decrease in median values for serum ferritin, deferoxamine-chelatable iron, and hepatic iron concentration. Before phlebotomy, stimulation by hypoglycemia and thyrotropin releasing hormone caused significantly less secretion of growth hormone (P = 0.004) and prolactin (P = 0.03) in patients than in control subjects. No significant improvement was noted, however, in growth hormone or prolactin secretion after phlebotomy. Of the 11 patients, 7 had secondary hypogonadism, and phlebotomy did not improve the serum testosterone, follicle-stimulating hormone, luteinizing hormone, or responses to LHRH in any case. Chlorpromazine injections failed to elevate serum prolactin in all patients, and administration of levodopa caused a partial reduction in serum prolactin; thus, the hypothalamus may be an important locus of endocrine malfunction in these patients. We conclude that abnormal hypothalamic-pituitary function in genetic hemochromatosis is not substantially improved by iron-depletion therapy.

Adult↗

The effect of repeated phlebotomy on bilirubin turnover, bilirubin clearance and unconjugated hyperbilirubinaemia in the Crigler-Najjar syndrome and the jaundiced Gunn rat: application of computers to experimental design.

1. A multicompartmental model of erythrokinetics and bilirubin production has been developed to predict the consequences of chronic phlebotomy on daily bilirubin turnover. 2. Control values for four physiological variables including bilirubin turnover were determined in a 20-year-old woman with type I congenital nonhaemolytic jaundice (Crigler-Najjar syndrome). With these base-line data, the model predicted the following changes during phlebotomy: a 34% fall in bilirubin turnover; a 240% increase in the haemoglobin content of bone-marrow erythroid precursors; a 25% fall in the half-life of 51Cr-labelled erythrocytes; a characteristic alteration of the erythrocyte survival curve after labelling with [2-14C]glycine. 3. On the assumption, previously validated in normal volunteer subjects and patients with Gilbert's syndrome, that hepatic bilirubin clearance was independent of turnover and would therefore remain unchanged, a fall in plasma unconjugated bilirubin concentration during phlebotomy from 436 to 282 mumol/1 was expected. 4. Accordingly, the patient underwent phlebotomy 350 ml/week for 2 months, and 500 ml/week during an additional 3 months. Appropriate studies during phlebotomy confirmed each of the predictions in paragraph 2 above. In particular, turnover fell by 31%. Unexpectedly, plasma unconjugated bilirubin remained essentially unchanged. Analogous results were observed in phlebotomized jaundiced Gunn rats. 5. Kinetic studies in both the patient and the rats demonstrated that the failure of plasma unconjugated bilirubin to fall in parallel with bilirubin turnover resulted from a prolongation of the terminal half-life of radioactively labelled bilirubin and a fall in bilirubin clearance in every instance. 6. These studies indicate that (a) in congenital non-haemolytic jaundice, bilirubin clearance is uniquely influenced by bilirubin turnover and (b) compartmental modelling and kinetic studies are useful for predicting and interpreting the results of both physiological experiments and experimental therapeutic regimens.

Adult↗

Hydroxychloroquine versus phlebotomy in the treatment of porphyria cutanea tarda.

Hydroxychloroquine and phlebotomy were compared in the treatment of porphyria cutanea tarda (PCT). Thirty patients received hydroxychloroquine (200 mg twice weekly) for 1 year and thirty-one underwent twice-monthly phlebotomies of 400 ml whole blood each, also for 1 year. Clinical signs of disease improved equally in both groups. At the end of the year, urinary porphyrin excretion had significantly improved in twenty-two out of thirty hydroxychloroquine-treated subjects, but in only eight out of the thirty-one patients who received phlebotomy. Liver histology showed significant regression of steatosis and siderosis in both groups compared with the pretrial biopsy, but the activity of liver disease, as judged by the extent of necrosis, inflammation and fibrosis, worsened in twelve hydroxychloroquine and in seven phlebotomy-treated patients. It is concluded that hydroxychloroquine is more effective than phlebotomy in decreasing porphyrin production. However, further work is needed to assess whether long-term hydroxychloroquine treatment favours the progression of the chronic liver disease associated with PCT.

Adult↗

Phlebotomy improves pulmonary gas exchange in chronic mountain polycythemia.

There is not unanimous agreement in the literature regarding the effects of bleeding on pulmonary gas exchange in polycythemic patients. Spirometry, alveolar arterial O2 and CO2 tension differences, PaO2 breathing 100% oxygen and carbon monoxide-diffusing capacity were measured before and after 1 week of chronic phlebotomy in 4 chronic mountain polycythemic patients. Studies were carried out at 3,700 m above sea level (PB = 491 mm Hg). Before phlebotomy, 2 patients showed abnormal spirometry and gas exchange. Only 1 patient had high PaCO2 and all of them showed low values of PaO2 breathing oxygen. Phlebotomy improved both spirometry and gas exchange. Improvement in arterial oxygen saturation and PaO2 could not be attributed to changes in alveolar ventilation, but rather to better distribution of VA/Qc ratios since physiological dead space decreased. Our results are similar to those reported in polycythemia vera patients. A significant correlation between the changes in PaO2 with phlebotomy and the control PaO2 have been found from 45 polycythemic patients with chronic obstructive pulmonary disease collected from the literature. It is concluded that excessive polycythemia worsened hypoxemia and that phlebotomy improved gas exchange.

Adult↗

Laboratory phlebotomy. College of American Pathologists Q-Probe study of patient satisfaction and complications in 23,783 patients.

Outpatients from 630 institutions participated in a phlebotomy module of Q-Probes, a quality assurance program of the College of American Pathologists, Northfield, Ill. This module assessed patient outcome measurements of complications, discomfort, and satisfaction with the phlebotomy procedure. Of the 29,700 ostensibly healthy individuals registered, 80.1% returned postcards containing measurements and assessments they made about the procedure and information recorded by the phlebotomist. The median time required for phlebotomy was 6 minutes, with 25% of patients requiring less than 5 minutes and 10% more than 21 minutes for completion of the procedure. The average number of phlebotomy attempts per patient was 1.03, with 95 patients (0.4%) experiencing three to 11 attempts. Ecchymoses occurred in 4048 (16.6%) attempts, with the median size of ecchymosis being 15.1 mm. On the average, an outstanding employee was identified by patients 46.6% of the time. The discomfort caused by the needle puncture was more than expected by 35.3% of patients. Although 98.6% of the patients were satisfied, 336 patients were dissatisfied with the procedure. We conclude that the technical skills of phlebotomists and patient satisfaction with phlebotomy are outstanding, but that patient discomfort from the procedure needs to be minimized.

Bloodletting↗

Transfusional iron overload in patients undergoing dialysis: treatment with erythropoietin and phlebotomy.

Five patients undergoing long-term hemodialysis with transfusional iron overload received treatment for 18 weeks with a regimen of recombinant human erythropoietin (150 U/kg) and regular phlebotomy to maintain the hematocrit value at 25% and reduce the total body iron burden. In the 149 phlebotomy sessions performed in these patients, a mean of 228 +/- 8 ml (mean +/- SEM) of whole blood was removed; it had a hematocrit value of 27.7% +/- 0.2%. The iron content of the erythrocytes removed (erythrocyte iron concentration, 787 +/- 11 micrograms/ml in 133 samples) accounted for more than 99% of the total iron removal by phlebotomy. Serum iron (serum iron concentration, 1.57 +/- 0.09 micrograms/ml in 65 samples) accounted for an insignificant fraction of the total iron removed. The iron removed at each phlebotomy session averaged 49.1 +/- 2.0 mg, similar to the amount of iron removed with deferoxamine administration in patients undergoing dialysis who had iron overload, but without the potential for adverse side effects reported with long-term deferoxamine therapy. Total iron removal during the 18 weeks of this study ranged from 732 to 2797 mg. Mean serum ferritin level decreased from 3189 +/- 1076 micrograms/L to 1676 +/- 342 micrograms/L (p less than 0.02, Wilcoxon signed rank test). When compared with a group of five patients without transfusional iron overload who received recombinant human erythropoietin and did not undergo therapeutic phlebotomy, the patients with iron overload had much greater iron losses and a larger decrease in serum ferritin levels.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Polyglobulia vera. Difficulties and complications of treatment by phlebotomy].

The authors report the results of phlebotomy for polyglobulia vera in a series of 73 patients eligible for inclusion in an international co-operative study. Previous studies usually gave actuarial survival curves but failed to mention the complications and discomfort associated with phlebotomy, although these are of importance in clinical practice. Most of the 73 patients were excluded on account of discomfort (20%), vascular thrombosis (almost 50%) or transformation into myelofibrosis within a mean period of 4 years (20%). Only 10% were treated with long-term phlebotomy. Although phlebotomies avoid the long-term risk of leukaemia attached to radiophosphorus or chemotherapy (20% on average after a mean delay of 12 years), they have practical limitations and their own, important risks. In patients over 65 and in those at high vascular risk, the best treatment is myelosuppression. However, younger subjects with polyglobulia vera but no vascular risk and/or thrombocytosis may benefit, at least temporarily, from phlebotomy.

Adult↗

Phlebotomy treatment of porphyria cutanea tarda.

The effect of phlebotomy on 44 patients (33 men and 11 women) with porphyria cutanea tarda (PCT) is described. All had typical biochemical and clinical signs of PCT. The patients were bled until slight anemia and signs of iron deficiency. In patients who were bled frequently and who were not given iron supplements in conjunction with phlebotomy, urinary uroporphyrin excretion decreased to 1 mg (or 1.2 mumol) a day or less in 1-8 months (mean 4 months) and then continued to decrease long after the treatment had been stopped and became normal in many patients. A decrease in porphyrin excretion comparable to that in patients treated with phlebotomy occurred in only two out of 12 controls. Remission occurred in patients treated with phlebotomy who had iron overload as well as in patients with quantitatively normal iron stores and in patients abstaining from alcohol as well as in patients who continued their abuse of alcohol. The disappearance of skin fragility and ulcers coincided roughly with the time when the urinary excretion of uroporphyrin decreased to about 1 mg (1.2 mumol) a day. After 43 remissions (in 43 patients) not followed by iron medication, one relapse occurred after a period of 20 months. In 5 patients in whom their iron stores were replenished a relapse occurred in all within 18 months. After longer observation periods relapses occurred also after remissions not followed by iron medication. During observation periods of 3-10 years 15 out of 41 patients relapsed biochemically. These had re-accumulated iron stores spontaneously. The results indicate that phlebotomy is consistently effective and probably exerts its effect mainly by reduction of iron stores.

Adult↗