Successful allogeneic peripheral blood stem cell transplantation in Wiskott-Aldrich Syndrome Patients: first report in Thailand.

Wiskott-Aldrich syndrome (WAS), an X-linked recessive disorder, is characterized by primary progressive T cell immunodeficiency, impaired antipolysaccharide antibody production, eczema, and thrombocytopenia. Stem cell transplantation is the only curative therapy. To evaluate the use of allogeneic peripheral stem cell transplantation (PBSCT) in this group of patients, we performed allogeneic PBSCT in two WAS patients (3 and 12 years old). The conditioning regimen consisted of busulfan 4 mg/kg/day for 4 days, and cyclophosphamide 50 mg/kg/day for 4 days. Graft-versus-host disease prophylaxis was consistent with cyclosporin A and methotrexate. Peripheral blood stem cells were collected from their brother donors (6 and 16 years old) by continuous flow leukapheresis after mobilization with granulocyte-colony-stimulating factor at a dose of 7.5 microg/kg/day for 5 days. Both recipients achieved neutrophils engraftment on days 11 and 12. The first patient achieved platelets engraftment on day 30. The second patient did not have platelet count below 20.0 x 10(9)/l during PBSCT procedure. Both did not develop acute or chronic graft-versus-host disease. At present, they are healthy after PBSCT. The follow up time after transplantation is 1,170 days and 269 days, respectively. Allogeneic PBSCT is economically feasible for WAS. The cost of PBSCT in Thailand is 20 to 30% less than bone marrow and cord blood stem cell transplantation. The cost of the transplant procedure for each patient in Thailand is US $ 12,000. This study is the first report of a successful stem cell transplantation in WAS patients in Thailand.

Prevalence of factor V Leiden in Thai blood donors.

Factor V Leiden was recently found to be the most common cause of familial venous thrombosis in the European population. We have studied the prevalence of factor V Leiden by DNA analysis among 500 Thai blood donors (male 285, female 215). Their ages ranged from 18 to 60 years with a mean of 33 years and 2 months. All of them were healthy voluntary blood-donors who met the standard criteria of the American Association of Blood Banks. No history of thrombosis was found. The results revealed that factor V Leiden was not present among 1,000 chromosomes from Thai blood donors. This suggests that factor V Leiden is not the common genetic predisposing factor of venous thrombosis in the Thai population as compared to the European population.

Iron stores in autologous blood donors.

Depletion of body iron stores is a major factor limiting regular blood donation in volunteer donors. Autologous blood donors are requested to donate more frequently. To determine iron stores in autologous donors, 9 men and 10 women studied gave a total of 24 donations before their elective surgery (range 1-2 donations). All donors were tested for serum ferritin (SF) and hemoglobin (Hb) level. Iron supplements were taken by 88.89 per cent of men and 90 per cent of women. Mean SF before donations was 147.75 ng/mL in men and 53.19 ng/mL in women. After donations, mean SF decreased to 124.26 ng/mL in men and 38.81 ng/mL in women. None of them had depleted iron stores (SF < or = 15 ng/mL). In conclusion, iron supplementation was beneficial in maintaining body iron stores in autologous blood donors.

The organization of Kidney Transplantation Services at Ramathibodi Hospital: fourteen years experience on waiting list, kidney donors and kidney transplantation.

The Kidney Transplantation Program at Ramathibodi Hospital was established in 1985. By the end of 1998, there were 1,614 patients on the cumulative waiting list. The first kidney transplantation (KT) was started in 1986 by using kidney from living-related donor (LD) while cadaveric KT (CD-KT) was started in 1987. A total of 528 KT were done, 278 cases (52.7%) were CD-KT and 250 cases (47.3%) were LD-KT. Six patients had two kidney transplants. 278 kidneys were donated from 189 cadaveric donors. Fifty cadaveric donors (26.4%) were from Ramathibodi Hospital while the rest were from other hospitals and the Organ Donation Center, Thai Red Cross Society. For LD, 233 out of 250 (93.2%) were from living-related, more than 50 per cent of these donors were from siblings. 17 spousal donors have been accepted for KT at Ramathibodi Hospital since 1997. Concerning the recipient pools, 522 patients (32.3%) were transplanted, 123 patients (7.6%) died without KT, 111 patients (6.9%) underwent KT at other hospitals, and 78 patients (4.8%) changed to waiting lists at other hospitals. The rest were lost to follow-up. At present, only 265 patients are still actively waiting (send serum every month). The number of KT and living donors has gradually increased, whereas, the number of cadaveric donors has decreased. However, cooperation with the "Organ Donation Center" has improved the number of cadaveric donation in the last two years. Sufficient organ donations and an active working team will provide a good kidney transplant service for the patients.

Iron stores in Thai blood donors.

A high number of blood donations may cause iron depletion. In order to evaluate iron stores in volunteer Thai blood donors, 82 male and 72 female donors were studied. All were tested for serum ferritin (SF), hemoglobin (Hb) level and asked for detailed histories of donations and iron supplementation. Mean SF in first-time donors was 161.12 ng/mL in men (n = 16) and 53.92 ng/mL in women (n = 23). Mean SF in multiple-time donors was 52.72 ng/mL in men (n = 66) and 25.72 ng/mL in women (n = 49). Depleted iron stores (SF < or = 15 ng/mL) were found in 8.7 per cent of first-time female donors, 21.21 per cent and 32.65 per cent of multiple-time male and female donors, respectively. The mean numbers of total donation were 51.42 +/- 30.8 in men and 8.22 +/- 6.97 in women. The estimation of depleted iron stores from Hb level could be determined in 57.14 per cent of male and 88.89 per cent of female donors. In conclusion, iron supplementation will benefit female donors and multiple-time male donors. The frequency of donations per year was more predictive of decreased iron stores than the number of total donations.

Transfusion requirements in patients with dengue hemorrhagic fever.

Dengue viruses are endemic in Thailand and Southeast Asian countries. A retrospective study of 175 patients with dengue virus infection admitted at the Department of Pediatrics, Ramathibodi Hospital in 1997 was carried out. Fifteen and 160 patients were clinically diagnosed with dengue fever and dengue hemorrhagic fever (DHF), respectively. DHF was commonly found in patients whose ages ranged from 10 to 14 years. The mean body weight was at the 54th percentile for age. In the management, 10.6% of patients with DHF required blood component therapy which included platelet concentrate (64.7%) in patients who exhibited active bleeding, packed red cells (47%) in patients who exhibited a rapid drop in the hematocrit and fresh frozen plasma (29.4%) in patients with circulatory failure who did not respond to intravenous fluid. The transfusion requirement was significantly correlated with the occurrence of bleeding (p < 0.008) and bleeding in the gastrointestinal tract (p < 0.0001) but not correlated with the number of platelet counts (p = 0.207). As a result, physicians in charge should be aware of the transfusion requirement and communicate this to the blood bank in advance for the preparation of appropriate blood components.

Low cost locally prepared fibrin glue for clinical applications: reported of 145 cases. Committee of Bangkok International Hemophilia Training Center.

Fibrin glue (FG) is one of the blood products known to be very useful for local hemostatic measure and as a medically valuable tool for adhesion, sealing, anastomosis, repair microvascular and nerve grafts in medical and surgical procedures. Before 1996, FG was used to a limited extent in Thailand due to the high cost. Technology for locally prepared FG was transferred to Bangkok International Hemophilia Training Center of the World Federation of Hemophilia (IHTC-WFH) in July 1996 by Prof. Uri Martinowitz and the late Prof. Henri Horoszowski. Since then FG has been widely used and proved to be very useful in Thailand. This paper reports 145 cases using low cost locally prepared FG at Ramathibodi Hospital during November 1996 to December 1997. A total of 145 cases with age range from 5 months to 73 years, which included 55 pediatrics and 90 adults, 100 males and 45 females. The amount of FG used was 1-80 ml per case. Clinical procedures included dental surgery (46), open heart surgery (35), ENT (28), orthopedic (13) including 2-3 joint correction in one session in 2 hemophiliacs, neurology (11), plastic repair (7), liver (2) and severe bleeding in dengue hemorrhagic fever (3). Forty-seven cases had hemostatic disorders. The result of local hemostatic, adhesive and sealant effect of FG was satisfactory with no complications. In open heart surgery, the amount of content in chest drain decreased and none required reopen-surgery to stop bleeding. Dental surgery was performed in 43 patients with bleeding disorders i.e. hemophilia, idiopathic thrombocytopenic purpura, leukemia, severe thrombocytopenia, patients on anticoagulant, etc. Only 3 cases (7%) required blood component compared to all of the 50 no-FG controlled cases (100%) that required blood component therapy. FG has proved to be very useful in many aspects i.e. minimizing blood product usage, decreasing medical workload, reducing medical cost and increasing patients' convenience and satisfaction in particular.

Plasmapheresis for the preparation of HIV free cryoprecipitate.

Single donor cryoprecipitate was prepared by blood cell separator for treatment of hemophilia A and von Willebrand patients to reduce a risk of transfusion associated HIV infection. A total of 7 plasmapheresis (range 1-1.5 plasma volume) was performed in 4 donors. Then fresh frozen plasma (FFP) was processed to cryoprecipitate and cryoprecipitate removed plasma (CRP). Donors were replaced with 0.9% normal saline solution (NSS) and 5% albumin for the first donation or their own CRP and 5% albumin for the second and third donation. After plasmapheresis total protein, albumin, IgG, IgA and IgM were below normal level in 71.43% (5/7), 14.3% (1/7), 28.57% (2/7), 14.3% (1/7) and 28.57% (2/7), respectively. All of these parameters returned to normal level within 3 days. Factor VIII:C was decreased after plasmapheresis in all donors and the low level of F VIII:C returned to normal within 24 hours. The donor was not exposed to any harmful effect. Donor reactions observed were mild. One donor was chilled due to unwarmed replacement solution. When this donor donated for the second time and was replaced with prewarmed replacement solution, no reaction was observed. We conclude that a preparation of single donor cryoprecipitate by plasmapheresis is safe and can reduce a risk of transfusion associated HIV infection. The donors are not at risk as the result of changes in the measured plasma protein and factor VIII:C level following plasmapheresis.

The safe blood components for a hemophilia patient: a case report.

This study comments on safe blood components prepared for a 6 year old boy with hemophilia A (F VIII:C 1.8%). He has required blood transfusion since 10 months of age and started home therapy since 3 years of age. The utilized blood components were fresh dry plasma (FDP) prepared from 8-10 selected donors who were in good health and had no risk of blood-transmitted diseases. They were parents, relatives and friends with the age ranging from 30-45 years old. The FDP would be used after the donors had at least two subsequent negative tests for anti-HIV. In addition, cryoprecipitate collected from parents by plasmapheresis was started in 1990. Twelve to fifteen bags of cryoprecipitate were collected each plasmapheresis. The blood components prepared from the selected donors is an additional management to obtain the better quality of blood. Moreover, the role of parents taking the major responsibility in the recruitment of donors will minimize the shortage of blood donors.