Sjögren-like syndrome after bone marrow transplantation.

OBJECTIVE: To study the incidence of dry eye in Sjögren-like syndrome, graft-versus-host disease (GVHD) in hematological patients undergoing bone marrow transplantation (BMT). MATERIAL AND METHOD: Prospective, cross-sectional study in twenty-six patients that were planned for BMT (group I). Twenty-nine patients undergoing BMT before study were classified as group II no GVHD (9), and group III with GVHD (20). Thirty-two normal subjects were controls. All subjects were examined by slit lamp biomicroscopy and had their tear samples analyzed about tear osmolarity. They were also evaluated for aqueous tear production by phenol red thread test, Schirmer test without anesthesia, tear film stability by tear break-up time (TBUT), and rose bengal staining 2 weeks before BMT (for group I) as well as 6 weeks, 3 months, and 6 months after BMT. The patients with GVHD were followed up 1 month later. Main outcome measures were amount of tear production, tear film stability, and dry eye symptoms. RESULTS: Average aqueous tear production in group III was less than control and group II (p < 0.001). Mean TBUT in group III was faster than control (p < 0.001) and group I before BMT (p = 0.001). Mean score of rose bengal staining in group III was more than control and group I before BMT (p < 0.001). Keratoconjunctivitis sicca and red eye developed in 27.5%, and 20% of group III, with incidence of dry eye by Schirmer test without anesthesia (67.5%). This compares with group II having incidence of dry eye of 16.7%. However, 42.3% of group I before BMT had dry eye compared with 9.4% in the controls (p < 0.001). CONCLUSION: Trend of dry eye in patients with BMT and GVHD were higher than no-GVHD group. Doctors should be aware of ocular symptoms and signs of dry eye in patients with BMT and follow-up for proper management.

Intravascular lymphomatosis: a study of 20 cases in Thailand and a review of the literature.

BACKGROUND: According to the World Health Organization classification (2001), intravascular large B-cell lymphoma (IVLBCL) is characterized by the presence of lymphoma cells only in the lumina of small vessels. It has not been proven whether IVLBCL is a specific clinicopathologic entity. Intravascular large B-cell lymphoma and other intravascular lymphomatoses (IVLs), including IVL with B-cell phenotype and extravascular growth (B-IVL) and IVL with T-cell phenotype (T-IVL), were compared in a series of cases diagnosed at a single institution and in cases reported in the literature. PATIENTS AND METHODS: Twenty cases of IVL diagnosed among 1826 consecutive cases of non-Hodgkin's lymphoma (NHL,1.1%) at Siriraj Hospital included 3 cases of IVLBCL, 14 cases of B-IVL, and 3 cases of T-IVL. In the literature, 102 cases of IVLBCL, 88 cases of B-IVL, and 18 cases of T-IVL were described in sufficient detail to be analyzed. RESULTS: All 3 groups were quite similar in clinical manifestations and outcome. Contrary to the previous review of 79 cases of IVL in 1989, blood, marrow, and nodal involvement could be detected in approximately 30% of cases. Patients who received chemotherapy had better survival than patients without treatment (statistically significant in IVLBCL and B-IVL; P < 0.05). Cases with skin involvement had better survival than cases without skin involvement (statistically significantly in T-IVL; P < 0.05). CONCLUSION: These results indicate that IVLBCL is not different from B-IVL or T-IVL in a biologic sense, and IVL seems to be better terminology than IVLBCL because it includes the T-cell phenotype that constitutes approximately 9% of cases. Early diagnosis is very important because chemotherapy significantly prolongs survival.

Efficacy of immunotherapy using antigens of Pythium insidiosum in the treatment of vascular pythiosis in humans.

Human pythiosis is an emerging disease in the tropical, subtropical and temperate regions of the world. It is caused by the straminipilan, fungus-like, aquatic organism Pythium insidiosum. Pythiosis occurs in localized as well as systemic or vascular forms. Most patients with arterial pythiosis usually have underlying hematologic disorders such as thalassemia and aplastic anemia/paroxysmal nocturnal hemoglobinuria (PNH) syndrome. Vascular pythiosis is characterized by ascending blood vessel infections and thrombosis of the major arteries especially those of the lower extremities. When the infection reaches a main artery, the patient usually dies within weeks. Since this pathogen is resistant to most antifungal drugs, immunotherapy was recently used to cure humans and animals with the disease. A modified P. insidiosum-antigen (PIA) formulation had already saved a young boy with life-threatening arterial pythiosis. Here, we report the therapeutic benefits of the PIA in eight patients with vascular pythiosis. Six of them had thalassemia and the other two had PNH. All of the patients had arterial occlusion of the lower limbs. P. insidiosum was isolated and identified by culture and by histopathology. All patients had evidence of active infection when immunotherapy began. After two injections of 100-200 microl of PIA (2.0mg/ml), at a 14-day interval, four patients (50%) had dramatic and complete remission. Two patients showed partial responses to PIA while the other two did not. Clinical responses correlated with the immunological reactions at the site of injection, clearance of the arteries and cytokine production. The latter included the shifting in serum levels of IL4 and IL5 to IL2 suggesting a switching from a T helper 2 (Th2) to a T helper 1 (Th1) subset. Our findings provide further evidence that immunotherapy using PIA is a safe and effective method to treat pythiosis in humans.

Collection and processing of umbilical cord blood for cryopreservation.

Umbilical cord blood (UCB) is being increasingly used as an alternative source of hematopoietic stem cells for allogeneic bone marrow transplantation. UCB transplantation has been successfully used to treat a variety of genetic, hematological, and oncological disorders in children and adults. The objectives of this study was to establish a closed-system technique for UCB collection and buffy coat separation by Optipress I device. Thirty-four UCB were collected by triple-bag system from pregnant mothers whose fetuses were not affected by thalassemic diseases after prenatal diagnosis. The mean volumn of UCB collection were 120 +/- 5 ml (range 65-180 ml). Total WBC, CD34+ cells, the progenitor cell erythroid burst-forming unit (BFU-E) and granulocyte-macrophage colony-forming unit (CFU-GM) in the UCB units were (9.36 +/- 0.84) x 10(8), (3.61 +/- 0.52) x 10(6), (9.12 +/- 1.60) x 10(5), and (5.32 +/- 1.23) x 10(5), respectively. Good correlation between the nucleated cell and net cord blood volume could be demonstrated (p < 0.0001). The correlation between CD34+ cells and the following parameters: nucleated cell, BFU-E or CFU-GM were also demonstrated (p = 0.001, 0.0105 or 0.0001, respectively). Buffy coat was subsequently separated from 18 UCB units by Optipress I device. 70 +/- 3 ml of buffy coat were collected and cryoprocessing was done by automatic controlled-rate freezer. Good recovery of total WBC, CD34+ cells, progenitor cells BFU-E and CFU-GM after buffy coat separation were observed 89 per cent, 95 per cent, 109 per cent, and 102 per cent respectively. There was no aerobic bacterial or fungal contamination in the separated blood products. By using this technique, the UCB units were easily collected, rapidly separated within one hour, and high recovery of the hematopoietic progenitor cells could be obtained.

Reversal of marrow fibrosis in agnogenic myeloid metaplasia by allogeneic peripheral blood stem cell transplantation.

Agnogenic myeloid metaplasia (AMM) is a clonal hematopoietic stem cell disorder characterized by bone marrow fibrosis, extramedullary hemopoiesis, splenomegaly and a leukoerythroblastic blood picture. Current standard therapies using hydroxyurea, interferon, androgens or corticosteroids have not shown to prolong survival of patients with AMM. In this study, we performed a curative approach using an HLA-matched sibling as a donor for allogeneic peripheral blood stem cell transplantation (PBSCT) for a 45-year-old woman with AMM. Busulfan and cyclophosphamide were given as a conditioning regimen from day -7 to day -2 with cyclosporinA and methotrexate as post-transplant immunosuppressive therapy. Donor PBSCs were mobilized by G-CSF at 16 microg/kg/day for five days and transplantation was performed on March 2-3, 2000. The patient rapidly engrafted within 2 weeks after PBSC infusion without evidence of graft versus host disease. Her blood counts and bone marrow 2 years after transplantation were normal with full donor pattern by molecular analysis. In conclusion, marrow fibrosis can be reverted to normal by allogeneic PBSCT. Allogeneic PBSCT should thus be offered to AMM patients if an HLA-matched sibling is available. This report represents the first SCT for AMM in Thailand.