losartan-potassium and Hemosiderosis

Parenteral ascorbic acid has been frequently used to overcome problems of vitamin C deficiency in haemodialysis patients. The benefits of vitamin C supplementation in clinical studies have been controversial and did not consider toxicological aspects. The review summarizes recent findings of the effects of parenteral ascorbic acid and discusses toxicological effects.. Vitamin C deficiency in haemodialysis patients, which has been frequently described, cannot be improved with oral supplementation due to limited absorption of high dosages. To avoid consequences of vitamin C deficiency, parenteral vitamin C solutions should be administered because this intervention is the only way to guarantee a sufficient supply to the cells. A beneficial consequence of parenteral vitamin C on the recombinant human erythropoietin resistance is an additional therapeutic effect, which contributes to the prevention of iron deficiency anaemia in haemodialysis patients. Thus, large amount of supplemental vitamin C are required for extended periods of time (up to 500 mg 3 times a week). To avoid hyperoxaluria, plasma oxalate levels should be monitored on a regular basis, for example, once a week.. Parenteral administration of ascorbic acid may be an approach that can overcome problems of vitamin C deficiency in haemodialysis patients - in particular problems of iron overload, erythropoetin resistance, and chronic inflammation.

Topics: Ascorbic Acid; Ascorbic Acid Deficiency; Drug Resistance; Erythropoietin; Hemosiderosis; Humans; Inflammation; Infusions, Parenteral; Renal Dialysis

Intravenous (i.v.) iron and recombinant human erythropoietin (EPO), like all other medications, are associated with the risk of adverse events. Historically, the primary concern with iron therapy has been the possibility of iron overload, which exposes the individual to the effects associated with nontransferrin-bound iron. Experience with EPO use has demonstrated an association with hypertension and with the upregulation of a number of markers of inflammation. The impact of these potential adverse effects merits careful analysis, given that both i.v. iron and EPO are designed for long-term use in a patient population at high risk for infection and cardiovascular disease. However, the incidence of iron overload and the risks associated with nontransferrin-bound iron have dramatically been reduced since the introduction of EPO therapy, and no data exist that demonstrate a definitive association between i.v. iron and an increased risk of morbidity related to infection or cardiovascular disease. On the other hand, EPO use is associated with hypertension, endothelial dysfunction, and prothrombotic and inflammatory states in hemodialysis patients. Risks associated with hypertension can be minimized by using the lowest effective EPO dose, which may be achieved through the regular use of i.v. iron. Judicious use of both i.v. iron and EPO may optimize cardiovascular outcomes.

Topics: Anemia, Iron-Deficiency; Blood Pressure; Cardiovascular Diseases; Comorbidity; Dose-Response Relationship, Drug; Erythropoiesis; Erythropoietin; Ferritins; Hemoglobins; Hemosiderosis; Humans; Hypertension; Inflammation; Injections, Intravenous; Iron; Patient Care Planning; Recombinant Proteins; Renal Dialysis; Risk Assessment

Six chronic hemodialysis patients suffering from iron overload (serum ferritin ranged between 2053-15704 mu g/L) were treated with human recombinant erythropoietin (EPO) alone for eighteen months. An initial rapid decrease of serum ferritin was observed in all six patients when the loading dose of EPO was given. Three patients (Cases 4, 5, 6) who suffered from chronic blood loss maintained a continuous decline of serum ferritin in the subsequent stage of maintenance dose EPO therapy, however, the decrease of serum ferritin became sluggish under a maintenance dose in the other three patients (Cases 1, 2 and 3). The serum ferritin eventually decreased to a satisfactory level (975-1761 mu g/L) in Cases 4, 5 and 6, whereas it remained high (4232-8196 mu g/L) in Cases 1, 2, and 3 who had higher basal levels of serum ferritin (5641-15704 mu g/L) and a plateau of response under a maintenance dose of EPO. These three patients were then treated with EPO and phlebotomy. Their serum ferritin decreased quickly from 6752 +/- 1264 mu g/L to 2454 +/- 482 mu g/L after six months of phlebotomy therapy; it was a dramatic improvement in contrast to the stagnant response under a maintenance dose of EPO alone. Our experience indicates that EPO therapy alone has its limitations in treating severe iron overload. Although there is an initial rapid decrease of serum ferritin during the period of the loading dose, the response might become stagnant during the period of maintenance dose. Phlebotomy effectively eliminated the excessive iron stores in these refractory cases. Therefore, we suggest that phlebotomy be considered in severe iron overload if a stagnant response is observed under a maintenance dose EPO therapy.

Topics: Adult; Combined Modality Therapy; Erythropoietin; Female; Hemosiderosis; Humans; Male; Middle Aged; Phlebotomy; Prospective Studies; Recombinant Proteins; Uremia

Haemodialysis patients with iron overload sometimes develop resistance to erythropoietin therapy due to 'functional iron deficiency'. It is known that this resistance may be overcome by iron supplementation; however, the latter could worsen haemosiderosis. Therefore, we treated four iron-overloaded haemodialysis patients who had developed relative resistance to erythropoietin (among whom three had features of 'functional iron deficiency') with ascorbic acid (500 mg intravenously after haemodialysis, 1-3 times a week). The erythropoietin doses were voluntarily kept unchanged during the study. After a latency of 2-4 weeks, haematocrit and haemoglobin had increased respectively from 26.5 +/- 0.7 to 32.7 +/- 0.4 vol% and from 8.8 +/- 0.3 to 10.8 +/- 0.2 g/dl (means +/- SEM, P < 0.001). While serum ferritin remained unchanged, transferrin saturation increased from 27 +/- 7 to 54 +/- 12% (P < 0.05), suggesting that ascorbic acid supplementation had allowed mobilization of iron from tissue burdens. In one patient, haematocrit declined after withdrawal of vitamin C and increased again after rechallenge. Also, ascorbate supplementation was continued after the study in two patients and allowed the erythropoietin doses to be decreased, 8 and 11 weeks, respectively, after the start of the trial. When a control group of seven patients with normal iron status and without resistance to erythropoietin were challenged in the same manner with ascorbate, no elevation of haematocrit or transferrin saturation was noted. We conclude that ascorbate supplementation may circumvent resistance to erythropoietin that sometimes occurs in iron-overloaded patients, in particular, in the setting of 'functional iron deficiency'.

Topics: Adult; Ascorbic Acid; Drug Resistance; Erythropoietin; Hematocrit; Hemoglobins; Hemosiderosis; Humans; Iron Deficiencies; Middle Aged; Renal Dialysis

Erythropoietic response to exogenously administered recombinant human erythropoietin (rHuEpo) was examined in 11 maintenance haemodialysis patients with iron overload (IO). All had required numerous blood transfusions earlier (> 12 units/year). Diagnosis of IO was established by high serum ferritin (SF) levels (> 1,100 micrograms/l), high hepatic CT density (> 70 Hounsfield units; HU) and excessive iron stores in bone marrow aspirate (grade 6). None of the patients had osteitis fibrosa cystica, aluminium intoxication, haemoglobinopathy or haemochromatosis alleles (HLA A3, B7 and B14). All patients responded to rHuEpo treatment (target haemoglobin level of 9-10 g/dl). None of the patients required iron supplementation or developed 'functional anaemia'. During 30 +/- 3 months of therapy, the initial maintenance dose of rHuEpo (103 +/- 12 units/kg/week) and median SF levels (2,250 micrograms/l) fell (50 +/- 8 units/kg/week and 1,060 micrograms/l, respectively) (p = 0.0003 and 0.0007). The initial and final rHuEpo doses correlated well with the respective SF levels (r = 0.89, p < 0.001). The maintenance dose of rHuEpo required for patients with IO at the start of the treatment period was significantly higher than that (50 +/- 5 units/kg/week) required by a control group of patients with adequate iron stores (SF = 100-600 micrograms/l) who were matched for age, sex and frequency of previous blood transfusions (p = 0.002). The findings suggested that excessive IO caused relative resistance to erythropoiesis on exogenous administration of rHuEpo and that iron supplementation was not warranted during rHuEpo therapy in those patients.

Topics: Adult; Erythropoietin; Female; Ferritins; Hemoglobins; Hemosiderosis; Humans; Iron; Kidney Failure, Chronic; Male; Middle Aged; Recombinant Proteins; Renal Dialysis

1. A randomized, partial-crossover study was conducted in uraemic patients with dialysis-associated anaemia and transfusional iron overload to evaluate the effects of desferrioxamine chelation therapy and of recombinant human erythropoietin treatment on hepatic iron storage determined by computed tomography, as well as by serum ferritin concentration and transferrin saturation. 2. Twenty-one haemodialysis patients with moderate iron overload, confirmed by values of serum ferritin concentration, transferrin saturation and hepatic computed tomography density exceeding 1000 micrograms/l, 45% and 68 Hounsfield units respectively, were randomly allocated to three groups and were followed for 12 months. 3. During the first 6 months group 1 (n = 7) received desferrioxamine chelation therapy (30 mg/kg intravenously three times a week) and group 2 (n = 7) underwent recombinant human erythropoietin treatment (36 units/kg intravenously three times a week). Thereafter, in the second 6 months of observation patients in group 1 were switched to receive recombinant human erythropoietin. Because of a poor response in the desferrioxamine-treated group in the initial 6 months, patients in group 2 continued on the maintenance dose of recombinant human erythropoietin (18 units/kg three times a week) until the end of the trial. Patients in group 3 (n = 7) were maintained on placebo throughout the study. 4. In comparison with placebo, recombinant human erythropoietin treatment, but not desferrioxamine chelation therapy, reduced serum ferritin concentration, transferrin saturation and hepatic computed tomography density, and was associated with a rise in haemoglobin and packed cell volume. Hepatic computed tomography density, serum ferritin concentration and transferrin saturation decreased in 13 out of 14 patients (93%) during treatment with recombinant human erythropoietin.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Adult; Aged; Chelation Therapy; Deferoxamine; Erythropoietin; Female; Ferritins; Hemosiderosis; Humans; Iron; Liver; Male; Middle Aged; Recombinant Proteins; Renal Dialysis; Tomography, X-Ray Computed; Transferrin; Uremia

Renal hypoplasia due to a congenitally reduced number of nephrons progresses to chronic kidney disease and may cause renal anemia, given that the kidneys are a major source of erythropoietin in adults. Hypoplastic kidney (HPK) rats have only about 20% of the normal number of nephrons and develop CKD. This study assessed the renal function and hematologic changes in HPK rats from 70 to 210 d of age. HPK rats demonstrated deterioration of renal excretory function, slightly macrocytic erythropenia at all days examined, age-related increases in splenic hemosiderosis accompanied by a tendency toward increased hemolysis, normal plasma erythropoietin levels associated with increased hepatic and decreased renal erythropoietin production, and maintenance of the response for erythropoietin production to hypoxic conditions, with increased interstitial fibrosis at 140 d of age. These results indicate that increases in splenic hemosiderosis and the membrane fragility of RBC might be associated with erythropenia and that hepatic production of erythropoietin might contribute to maintaining the blood Hgb concentration in HPK rats.

Topics: Age Factors; Anemia; Animals; Biomarkers; Carrier Proteins; Disease Models, Animal; Disease Progression; Erythrocytes; Erythropoietin; Fibrosis; Genetic Predisposition to Disease; Hemolysis; Hemosiderosis; Iron; Kidney; Kidney Diseases; Male; Mutation; Osmotic Fragility; Phenotype; Rats; Rats, Inbred Strains; Renal Insufficiency, Chronic; Spleen

Current regular blood transfusion programs and chelation treatment have considerably improved survival of patients with thalassemia, which resulted in a larger proportion of adult patients. However, disease- and treatment-related complications in these patients progress over time, causing severe morbidity and shortened life expectancy. Stem cell transplantation still remains the only cure currently available for patients with thalassemia. This study updates transplant outcomes in 107 adult patients with median age of 22 years (range, 17-35 years) who received bone marrow transplantation (BMT) from human leukocyte antigen (HLA)-identical related donors between 1988 and 1996 (group A) and describes the results of BMT in 15 adult patients with median age of 21 years (range, 17-31 years) who were treated with a new treatment protocol (Protocol 26) between 1997 and 2003 (group B). The probability of survival, event-free survival, nonrejection mortality, and rejection for group A patients were 66%, 62%, 37%, and 4%, respectively, with a median follow-up of 12 years (range, 8.3-16.2 years). Group B patients treated with the new protocol had some improvement in thalassemia-free survival (67%) and lower transplant-related mortality (27%) than that of previous protocols. However, transplant-related mortality in these high-risk patients remains elevated. Current myeloablative BMT in adult patients is characterized by higher transplant-related toxicity due to an advanced phase of disease. Although this new approach to transplant adult patients with a reduced-dose intensity-conditioning regimen has improved thalassemia-free survival, transplant-related mortality in these high-risk patients remains elevated.

Topics: Adolescent; Adult; Azathioprine; Bone Marrow Transplantation; Busulfan; Chelation Therapy; Clinical Protocols; Combined Modality Therapy; Comorbidity; Deferoxamine; Disease-Free Survival; Erythrocyte Transfusion; Erythropoietin; Female; Follow-Up Studies; Graft vs Host Disease; Granulocyte Colony-Stimulating Factor; Hematopoietic Cell Growth Factors; Hemosiderosis; Humans; Hydroxyurea; Immunosuppressive Agents; Iron Chelating Agents; Life Tables; Liver Cirrhosis; Male; Phlebotomy; Postoperative Complications; Survival Analysis; Thalassemia; Transfusion Reaction; Transplantation Conditioning; Transplantation, Homologous; Treatment Outcome; Vidarabine

Liver dysfunction is a frequent complication that arises in the period following kidney transplantations, often resulting in death. We reported a case proving hemosiderosis as a cause of prolonged liver dysfunction after cadaveric kidney transplantation.. A 47-year-old man, who had been undergoing hemodialysis, was referred to our hospital on 2 November 1999. On the same day, cadaveric kidney transplantation was performed, and serum creatinine level reached a normal level within 2 weeks after surgery. However, serum transaminase gradually increased in the postoperative period. Serum ALT rose up to 116 IU/L on day 20 after the operation and 215 IU/L on day 30. Microscopic examination by needle biopsy revealed hemosiderosis of the liver. Recombinant human erythropoietin was administered and phlebotomy was performed. Liver function improved as a result.. Early histological diagnosis can be a useful marker in predicting the course of chronic liver disease.

Topics: Cadaver; Chronic Disease; Erythropoietin; Hemosiderosis; Humans; Kidney Transplantation; Liver Diseases; Male; Middle Aged; Recombinant Proteins

Juvenile hemochromatosis is a rare genetic disorder that causes iron overload. Clinical complications, which include liver cirrhosis, heart failure, hypogonadotropic hypogonadism and diabetes, appear earlier and are more severe than in HFE-related hemochromatosis. This disorder, therefore, requires an aggressive therapeutic approach to achieve iron depletion. We report here the case of a young Italian female with juvenile hemochromatosis who was unable to tolerate frequent phlebotomy because of coexistent ss-thalassemia trait. The patient was successfully iron-depleted by combining phlebotomy with recombinant human erythropoietin.

Topics: Adrenal Cortex Hormones; Adrenal Insufficiency; Adult; Arrhythmias, Cardiac; beta-Thalassemia; Chelation Therapy; Chromosomes, Human, Pair 1; Deferoxamine; Erythropoietin; Estrogen Replacement Therapy; Female; Hemochromatosis; Hemosiderosis; Hormone Replacement Therapy; Humans; Hypogonadism; Liver Cirrhosis; Phlebotomy; Progesterone; Recombinant Proteins

A 46-year-old man was admitted to our clinic because of acute heart failure. Six years before admission he was pointed out cardiomegary and hematuria. One year later, he was diagnosed as having jugular foramen syndrome. On admission, he had a fever and dyspnea. Pansystolic blowing murmur was audible at the apex. The chest ratio on his chest X-ray was 52.5%. An electrocardiogram showed left ventricular hypertrophy. An echocardiogram showed marked dilatation and severe dysfunction of left ventricle. Radionuclide scanning with technetium 99 m pyrophosphate identified inflammatory change in the apex. Myocardial biopsy showed fibrotic degeneration and IgG deposits in myocardium. Blood examination showed anemia, lymphopenia. positive anti-nuclear antibody (1000 times, shaggy pattern), positive anti ds-DNA antibody and hypocomplementemia. Furthermore, proteinuria was pointed out. Renal biopsy showed focal segmental glomerulonephritis with active necrotizing lesion (type III nephritis). Lupus myocarditis and nephritis was diagnosed. After prednisolone (80 mg/day) was administered. left ventricular function and hypocomplementemia improved. The ACE inhibitor was also used for proteinuria. In spite of a little amount of blood transfusion, he showed hepatic hemosiderosis. We suspect that the cause of hemosiderosis was related chronic inflammation of active lupus. It was treated with Erythropoietin.

Topics: Angiotensin-Converting Enzyme Inhibitors; Anti-Inflammatory Agents; Cranial Nerve Diseases; Erythropoietin; Hemosiderosis; Humans; Lupus Erythematosus, Systemic; Lupus Nephritis; Male; Middle Aged; Myocarditis; Polyneuropathies; Prednisolone; Syndrome

Long-term follow-up data are presented on changes in peripheral blood counts and Hb requirements of 11 patients with myelodysplastic syndromes (MDS) during iron chelation treatment with desferrioxamine for up to 60 months. The erythroid marrow activity was indirectly evaluated by repeated determinations of the serum transferrin receptor concentration. The efficacy of iron chelation was evaluated by repeated quantitative determination of the liver iron concentration by magnetic resonance imaging. Reduction in the Hb requirement ( > or = 50%) was seen in 7/11 (64%) patients. Five patients (46%) became blood transfusion independent. Platelet counts increased in 7/11 (64%) patients and the neutrophil counts in 7/9 (78%) evaluable patients. All patients in whom iron chelation was highly effective showed improvement of erythropoietic output accompanied by an increase in the serum transferrin receptor concentration. It is concluded that reduction in cytopenia in MDS patients may be accomplished by treatment with desferrioxamine, if the iron chelation is efficient and the patients are treated for a sufficiently long period of time. Exactly how treatment with desferrioxamine works remains a challenge for further investigation.

Topics: Adolescent; Aged; Bone Marrow Diseases; Chromosome Aberrations; Deferoxamine; Erythropoietin; Female; Follow-Up Studies; Hematopoiesis; Hemoglobins; Hemosiderosis; Humans; Iron; Karyotyping; Leukocyte Count; Male; Middle Aged; Myelodysplastic Syndromes; Platelet Count; Receptors, Transferrin; Transfusion Reaction; Treatment Outcome

Patients with hemosiderosis who also suffer from coexistent anemia may be unable to tolerate frequent phlebotomies needed for depletion of body iron stores. Chelation therapy, an alternative approach, may be unsuitable for some patients due to allergic reactions, poor response or intolerance of long-duration subcutaneous administration. The use of recombinant human erythropoietin in such patients could increase the hematocrit and improve exercise tolerance allowing for more frequent phlebotomies. We report the successful use of this combined approach in two such patients.

Topics: Aged; Anemia, Refractory; Bloodletting; Combined Modality Therapy; Erythropoietin; Female; Hemosiderosis; Humans; Male; Middle Aged; Recombinant Proteins

Topics: Anemia; Erythropoietin; Hemosiderosis; Humans; Iron Chelating Agents; Transfusion Reaction

Two hemodialysis patients with hemosiderosis were treated with combined erythropoietin and repeated phlebotomy. Serial nuclear magnetic resonance (NMR) imaging and serum ferritin levels were used to monitor the efficacy of treatment. This treatment modality has definite advantages over chronic deferoxamine therapy. NMR image-derived parameters offer an objective, accurate, and noninvasive indication of tissue iron stores.

Topics: Adult; Aged; Bloodletting; Erythropoietin; Female; Ferritins; Hemosiderosis; Humans; Iron; Liver; Magnetic Resonance Imaging; Male; Myocardium; Renal Dialysis

A 55-year-old female patient with hemosiderosis induced by administration of excessive doses of parenteral iron was successfully treated with regular phlebotomy combined with recombinant human erythropoietin (rHuEPO). Ferrokinetic data before therapy showed 28.0 mumol/l of serum iron, 4.1 mumol/l of unsaturated iron-binding capacity, 4,060 ng/ml of serum ferritin, 148 min of plasma iron disappearance time, 45% of red cell iron utilization and 0.4 mg/kg/day of plasma iron turnover rate. She had 300-ml phlebotomies, first every other week then weekly, and subcutaneous injections of rHuEPO twice a week. Two years later, the total volume of phlebotomized blood reached 31 liters and her ferrokinetic data showed: serum iron 8.6 mumol/l, iron-binding capacity 39.6 mumol/l, serum ferritin 277 ng/ml, plasma iron disappearance time 52 min, red cell iron utilization 100% and plasma iron turnover rate 0.5 mg/kg/day. During the phlebotomy therapy, her hemoglobin levels were maintained above 12 g/dl. No adverse effect due to rHuEPO occurred. These findings provide evidence for the efficacy of rHuEPO in multiple phlebotomy therapy for hemosiderosis and may open new avenues for its clinical application.

Topics: Bloodletting; Chronic Disease; Combined Modality Therapy; Erythropoietin; Female; Hemosiderosis; Humans; Iron; Middle Aged; Recombinant Proteins

A chronic hemodialysis case, a 46-year-old woman with secondary hemosiderosis induced by parenteral iron and blood transfusion due to a refractory anemia, was effectively treated with recombinant human erythropoietin and the removal of red blood cells. The cumulative dose of the iron removed was 5,712 mg. Plasma ferritin decreased from 8,290 to 2,203 micrograms/l during 18 months. Concomitantly, liver histology performed before and after the therapy revealed a prominent regression of the deposited iron.

Topics: Anemia, Refractory; Biopsy; Blood Transfusion; Erythrocyte Count; Erythropoietin; Female; Ferritins; Follow-Up Studies; Hemosiderosis; Humans; Iron; Liver; Middle Aged; Recombinant Proteins; Renal Dialysis

Iron overload increases the risk of bacterial infection in dialysis patients, partly by impairing functions of the polymorphonuclear granulocytes (PMNs). PMN defence was studied sequentially in haemodialysis patients with transfusional haemosiderosis, treated for 6 +/- 1.5 months (n = 8) to 13 +/- 1.7 months (n = 4) with recombinant human erythropoietin (rHuEpo). Over this period, signs of iron overload (increased serum ferritin and serum iron) improved, and stainable iron disappeared in PMNs. Simultaneously, phagocytosis of Yersinia enterocolitica by PMNs improved. The decrease in serum ferritin was significantly related to the improved phagocytosis. Killing of Y. enterocolitica by PMNs also improved. It is anticipated that rHuEpo therapy in iron-overloaded dialysis patients could decrease the incidence of bacterial infection by improving PMN functions in these patients.

Topics: Adult; Aged; Bacterial Infections; Blood Bactericidal Activity; Erythropoietin; Female; Hemosiderosis; Humans; Iron; Male; Middle Aged; Neutrophils; Phagocytosis; Recombinant Proteins; Renal Dialysis; Yersinia enterocolitica

Five long-term hemodialysis patients with clinical iron overload were treated with 300 U/kg of recombinant human erythropoietin (rHuEPO) intravenously (IV) after each hemodialysis. The patients were phlebotomized after each hemodialysis at any time the predialysis hematocrit was 35% or greater. Over a period of 1 year, the average phlebotomy rate varied from 0.5 to 1.1 U/wk with a mean phlebotomy rate of 45.8 +/- 5.6 U/yr (range, 27 to 57 U). The mean serum ferritin decreased from 8,412 +/- 1,599 micrograms/L (ng/mL) to 3,007 +/- 1,129 micrograms/L (ng/mL), and the mean iron removal over this period was 9.5 g. Liver iron deposition, as measured by density on computed tomographic (CT) scan, improved, while skin color lightened significantly. Patients tolerated phlebotomy with no major symptoms or complications and exhibited no change in the hemogram or serum chemistries. In patients with severe iron overload, changes in serum ferritin with erythropoietin treatment alone may not reflect true change in iron burden. Use of high-dose erythropoietin and phlebotomy is an effective and safe (at least for 1 year) method of reducing iron overload in long-term hemodialysis patients.

Topics: Adult; Bloodletting; Erythropoietin; Female; Ferritins; Hemosiderosis; Humans; Iron; Male; Middle Aged; Recombinant Proteins; Renal Dialysis; Time Factors; Transfusion Reaction

Topics: Anemia; Child; Erythropoietin; Hemosiderosis; Humans; Kidney Failure, Chronic; Recombinant Proteins

A summary of the historical developments associated with congenital anemias are discussed. The clinical status, the peripheral blood and bone marrow picture of these patients are reviewed. The effectiveness of treatment and prognosis for these patients is evaluated.

Topics: Adrenal Cortex Hormones; Adult; Anemia; Anemia, Aplastic; Blood Transfusion; Bone Marrow; Erythropoietin; Hemosiderosis; Humans; Syndrome

An 18-yr-old female with chronic active hepatitis developed a severe anemia due to a lack of red cell production. Her bone marrow showed many large proerythroblasts but an almost complete lack of more mature erythroblasts. Incubation of the marrow cells in a normal medium with erythropoietin concentrate led to increased erythropoiesis as indicated by the development of mature erythroblasts as well as a ninefold increase in hemoglobin synthesis. The patient's plasma was cytotoxic for erythroblasts. Following splenectomy, a remission of the disease occurred. This study indicates that in some cases the anemia associated with abundant marrow proerythroblasts and the absence of mature erythroblasts has the same pathogenesis as pure red cell aplasia and that splenectomy may be beneficial when there is a lack of response to immunosuppressive drugs.

Topics: Adolescent; Anemia, Aplastic; Autoantibodies; Bone Marrow Cells; Bone Marrow Examination; Cells, Cultured; Cyclophosphamide; Cytotoxicity Tests, Immunologic; Erythroblasts; Erythrocytes; Erythropoietin; Female; Hemoglobins; Hemosiderosis; Hepatitis B; Humans; Immunosuppression Therapy; Iron; Iron Radioisotopes; Karyotyping; Liver Function Tests; Prednisone; Splenectomy; Transfusion Reaction