Erythrocytapheresis with recombinant human erythropoietin in hereditary hemochromatosis therapy: a new alternative.

BACKGROUND AND OBJECTIVES: The purposes of this study were to evaluate the tolerance, efficacy and safety of isovolemic erythrocytapheresis (EA) in nonanemic patients with hereditary hemochromatosis (HH), and to assess the usefulness of recombinant human erythropoietin (rHuEPO) associated with EA to reduce treatment duration. MATERIALS AND METHODS: In 10 asymptomatic patients with serum ferritin >400 microg/l, transferrin saturation >50%, and GPT elevation, EA with rHuEPO and folic acid was performed. RESULTS: Red cell indices, serum ferritin, and other iron metabolism parameters (serum iron, transferrin, and transferrin saturation); GPT and other laboratory data were considerably improved. CONCLUSION: This method offers better results in less time than traditional phlebotomy. EA with rHuEPO is an effective therapeutic alternative for patients with HH.

[Allogeneic hematopoietic transplantation with stem cells extracted from peripheral blood]. / Trasplante hematopoyético alogénico con células progenitoras extraidas de la sangre periférica.

Fifty three patients (pts) received an allogeneic hematopoietic transplant using peripheral blood progenitor cells (PBPC). Diagnosis were acute myeloid leukemia (AML) in 16 pts, acute lymphoblastic leukemia (ALL) in 15, chronic myeloid leukemia (CML) in first chronic phase in 12, aplastic anemia in 4, myelodysplasia in 3 and Hodgkin's disease, major thalasemia and Hunter's syndrome in one each. Mean age was 20 years-old (2-55), 28 males and 25 females. Conditioning regimens were total body irradiation with 1200 cGy and cyclophosphamide 120 mg/kg in 38 pts, busulfan 16 mg/kg and cyclophosphamide 120 mg/kg in 10 pts, total lymphoid irradiation and cyclophosphamide in 3, 2 pts received other chemotherapy based conditionings. PBPC were infused unmanipulated through a central catheter. Graft versus host disease (GVHD) prophylaxis was cyclosporin and short course methotrexate. Donors were 6/6 HLA compatible siblings in 52 cases and 5/6 match in one case. PBPC mobilization was done with G-CSF at a dose of 10 micrograms/kg/day subcutaneously for four days, pheresis started on day 5. Bone marrow harvest was also done in the first thirty cases. Mean cellularities for CD34, CD3, CD4, CD8, CD56, CD19 (cel x 10(6)/kg) were 4.12; 4.59; 2.57; 1.9; 0.55 and 0.68, respectively. Mean recovery of neutrophils > 500/microL was obtained on day +11 and platelets > 20,000/microL on day +13. Patients were hospitalized for a mean period of 26 days (range 18-39) and days with parenteral antibiotics were 12.2 (5-45). Two pts had venoocclusive disease of the liver. Transplant related mortality was 15%. Acute graft versus host disease (GVHD) was observed in 43.4% of pts, only 5 pts had acute GVHD III or IV. Mean time for aGVHD diagnosis was +23 (8-76). Forty three pts were evaluable for chronic GVHD with a mean follow-up of 18 months (4-39). Chronic GVHD was observed in 26.4% by day +240, only 2 pts developed severe cGVHD. The present experience demonstrates an acceptable incidence for cGVHD; however, taking into account recent reports showing an increase of this complication, it seems reasonable not to perform this procedure for non-malignant diseases in which graft versus malignancy effect is not to be expected.

Trasplante hematopoyético alogénico con células progenitoras extraidas de la sangre periférica. / [Allogeneic hematopoietic transplantation with stem cells extracted from peripheral blood]

Fifty three patients (pts) received an allogeneic hematopoietic transplant using peripheral blood progenitor cells (PBPC). Diagnosis were acute myeloid leukemia (AML) in 16 pts, acute lymphoblastic leukemia (ALL) in 15, chronic myeloid leukemia (CML) in first chronic phase in 12, aplastic anemia in 4, myelodysplasia in 3 and Hodgkins disease, major thalasemia and Hunters syndrome in one each. Mean age was 20 years-old (2-55), 28 males and 25 females. Conditioning regimens were total body irradiation with 1200 cGy and cyclophosphamide 120 mg/kg in 38 pts, busulfan 16 mg/kg and cyclophosphamide 120 mg/kg in 10 pts, total lymphoid irradiation and cyclophosphamide in 3, 2 pts received other chemotherapy based conditionings. PBPC were infused unmanipulated through a central catheter. Graft versus host disease (GVHD) prophylaxis was cyclosporin and short course methotrexate. Donors were 6/6 HLA compatible siblings in 52 cases and 5/6 match in one case. PBPC mobilization was done with G-CSF at a dose of 10 micrograms/kg/day subcutaneously for four days, pheresis started on day 5. Bone marrow harvest was also done in the first thirty cases. Mean cellularities for CD34, CD3, CD4, CD8, CD56, CD19 (cel x 10(6)/kg) were 4.12; 4.59; 2.57; 1.9; 0.55 and 0.68, respectively. Mean recovery of neutrophils > 500/microL was obtained on day +11 and platelets > 20,000/microL on day +13. Patients were hospitalized for a mean period of 26 days (range 18-39) and days with parenteral antibiotics were 12.2 (5-45). Two pts had venoocclusive disease of the liver. Transplant related mortality was 15

. Acute graft versus host disease (GVHD) was observed in 43.4

of pts, only 5 pts had acute GVHD III or IV. Mean time for aGVHD diagnosis was +23 (8-76). Forty three pts were evaluable for chronic GVHD with a mean follow-up of 18 months (4-39). Chronic GVHD was observed in 26.4

by day +240, only 2 pts developed severe cGVHD. The present experience demonstrates an acceptable incidence for cGVHD; however, taking into account recent reports showing an increase of this complication, it seems reasonable not to perform this procedure for non-malignant diseases in which graft versus malignancy effect is not to be expected.

High-dose intravenous immunoglobulin in non-ABO transfusion incompatibility.

The administration of intravenous immunoglobulin (IVIG) in immune and autoimmune diseases led us to use this agent to ameliorate or prevent the consequences of non-ABO incompatible transfusions in patients who need this form of therapy. IVIG (400 mg/kg/day) was infused within 24 h of transfusion in 5 patients with: (1) intestinal angiodysplasia, gastrointestinal bleeding, and anti-Kpb; (2) paroxysmal nocturnal hemoglobinuria, anti-c, anti E, anti Fyb, anti-K and autoantibodies; (3) lymphoma and autoimmune hemolytic anemia (AIHA); (4) systemic lupus erythematosus (SLE), AIHA, and anti-D, and (5) SLE and AHIA. A sustained increase in hematocrit was noted and no transfusion reaction developed in any of the cases. A single dose of pretransfusion IVIG may therefore be a useful therapeutic alternative in patients for whom no compatible blood is available. Patients with severe anemia, allo- and autoantibodies, either showing hemolysis in their pathophysiology or not, cause a serious problem in any transfusion center, especially when dealing with emergencies. In order to reduce the risks of incompatible transfusions, different modalities have previously been attempted, all with poor results. In 1989 we reported the successful use of pretransfusional high-dose intravenous immunoglobulin (IVIG) in a patient with gastrointestinal bleeding and anti-Kpb. The transfusion of incompatible red blood cells improved the anemia and allowed the exploratory laparotomy to take place. A protocol was then developed based on this case administering pretransfusion IVIG in high doses for patients for whom no compatible blood (non-ABO) is available.

Recombinant Interferon Alpha-2b in Patients with Progressive and/or Recurrent Hodgkin's Disease.

Six patients were included in a trial involving the SC administration of rIFN{alfa)-2b (Schering Plough) at a dose of 5 {multi} 10(6) IU daily, 5 days a week and continued while a clinical response was observed. All patients had previously received between 3 to 5 salvage chemotherapy programs without a disease free interval prior to the administration of rIFN{ALFA}-2b. Two patients had extranodal disease (lung, bone) and all 6 patients had "B" symptoms. Five of the patients showed stable disease, for a period of 3 to 12 months. One patient with recurrent HD obtained partial remission which lasted 26 months, at which time he developed autoimmune hemolytic anemia. Toxicities observed were mild and consisted of a flulike syndrome, supraventricular tachycardia and mild increase in transaminases. The quality of life improved while on rIFN{ALFA}-2b therapy, contrasting with the toxicity of the aggressive chemotherapy regimens previously administered which required frequent hospitalizations. This preliminary experience suggests that the role of rIFN{ALFA}-2b in the treatment of HD requires more study.