Adult allogeneic haematopoietic stem cell transplantation: a single centre experience in Malaysia.

We analysed the outcome of 104 patients from a single institution who underwent allogeneic haematopoietic stem cell transplantation (AHSCT) from their HLA-identical siblings between 1993 and 2006. Sixty-nine percent of patients had peripheral blood stem cell (PBSC) as the stem cell source and the remaining had bone marrow (BM). The majority of patients are Chinese (60%) followed by Malays (24%) and Indians (14%). The median time to reach white cell counts of >1 x 10(9)/L and platelet counts of >30 x 10(9)/L was 13 and 15 days, respectively in patients who had PBSC transplantation compared with 16 and 25 days in patients who had BM transplantation, (p < 0.0001 and p < 0.001). Acute graft-versus-host disease (aGVHD) of grade II to IV was observed in 34% of patients and chronic graft-versus-host disease (cGVHD) in 38% of patients. Although not statistically significant, there was a higher incidence of overall aGVHD in Indian patients (73%) compared to Chinese and Malays (57% and 56% respectively). There was no significant difference in the incidence of aGVHD and cGVHD with the source of stem cells. Overall survival (OS) and disease free survival (DFS) was 50% and 60% at five years respectively. Multivariate analysis showed that patients transplanted in standard risk and those who had limited cGVHD had a significant better OS, (p = 0.05 and p = 0.05). Patients who had cGVHD and transplanted in standard risk had a better DFS, (p = 0.002 and p < 0.001). In summary, AHSCT in standard risk patients is associated with a better outcome than those transplanted in high risk and although not statistically significant, there is a higher incidence of aGVHD in Indian patients.

The use of mycophenolate mofetil in treating patients with non responding aplastic anemia.

Aplastic anemia is a relatively uncommon disease and conventional management options include immunosuppressive drugs and/or haematopoeitic stem cell transplantation. It is now known that the pathogenesis of aplastic anemia is immune mediated. Mycophenolate mofetil is a common immunosuppressive drug now used mainly in prophylaxis of graft rejection in organ transplant and also for prevention/treatment for graft versus host disease in haemtopoeitic stem cell transplantation. It is thought that mycophenolate mofetil may be useful in this group of patients. In this short report, mycophenolate mofetil was tried in 6 patients who had severe aplastic anemia with variable doses for a minimum duration of 9 months. The result has however not been encouraging.

Incidence of upper limb venous thrombosis associated with peripherally inserted central catheters (PICC).

The objective of this study was to prospectively determine the incidence of venous thrombosis (VT) in the upper limbs in patients with peripherally inserted central catheters (PICC). We prospectively investigated the incidence of VT in the upper limbs of 26 patients who had PICC inserted. The inclusion criteria were all patients who had a PICC inserted, whilst the exclusion criterion was the inability to perform a venogram (allergies, previous contrast medium reaction and inability of gaining venous access). Both valved and non-valved catheters were evaluated. Prior to removal of the PICC, an upper limb venogram was performed. The number of segments involved with VT were determined. The duration of central venous catheterization was classified as; less than 6 days, between 6 days and 14 days and more than 14 days. VT was confirmed in 38.5% (10/26) of the patients. The majority 85.7% (12/14) were complete occlusive thrombi and the majority of VT only involved one segment. There was no statistical correlation between the site of insertion of the PICC and the location of VT. Neither was there any observed correlation between the occurrence of VT with the patient's history of hypertension, hypercholesterolaemia, coronary artery disease, diabetes mellitus, cardiac insufficiency, smoking or cancer. There was also no statistical correlation with the size of the catheter. In conclusion, PICCs are associated with a significant risk of upper extremity deep vein thrombosis (UEVT).

Familial hemophagocytic lymphohistiocytosis in two brothers.

Familial hemophagocytic lymphohistiocytosis is a disorder which presents with fever, pancytopenia, liver dysfunction and also an increase in non-malignant histiocytes with prominent hemophagocytosis in various organs. It is usually difficult to distinguish from other hemophagocytic syndrome in the absence of family history. It rarely manifests in adults. Chemotherapy is usually indicated. Here, we report the occurrence of this disorder in two brothers in their twenties.

A case of T-cell acute lymphoblastic leukemia after treatment of acute promyelocytic leukemia.

We diagnosed T-cell acute lymphoblastic leukemia (T-ALL) with multiple cytogenetic abnormalities in a 17-year-old girl a year after she had received a diagnosis of acute promyelocytic leukemia (APML). After the diagnosis of APML in June 2001, the patient was treated with idarubicin and all-trans-retinoic acid. In September 1999, her younger sister also received a diagnosis of APML and to date has remained well. T-ALL after remission of APML is very rare, and only 1 such case has been reported. Possible causes include therapy-related reasons, genetic susceptibility to leukemia, and environmental exposure.

Scopulariopsis brevicaulis infection in a patient with acute myeloid leukemia.

Scopulariopsis brevicaulis is a soil fungus normally associated with onychomycosis. It causes subcutaneous infection in immunocompromised patients and is rarely isolated from blood. A case of systemic Scopulariopsis brevicaulis infection was reported in a patient with acute myeloid leukemia. The patient developed persistent fever that did not respond to wide spectrum antibiotics and amphotericin B. Scopulariopsis brevicaulis was the only pathogen isolated from blood cultures. The fever subsided with itraconazole and there was no recurrence of fungal infection with prolonged maintenance of oral itraconazole.