Clinical, neuroimaging, and behavioural risk factors for neurocognitive impairment in Chinese patients with haemophilia: A multicentre study.

BACKGROUND: Few studies have evaluated the impact of subclinical microstructural changes and psychosocial factors on cognitive function in patients with haemophilia. OBJECTIVES: To determine the prevalence and characteristics of cognitive impairment in patients with haemophilia, and identify associated risk factors. METHODS: We recruited haemophilia A or B patients who were aged ≥10 years old from three public hospitals in Hong Kong. A neurocognitive battery was administered to evaluate their attention, memory, processing speed and cognitive flexibility performances. They also underwent magnetic resonance imaging to identify cerebral microbleeds. Validated self-reported questionnaires were administered to assess their mental health status and adherence to prophylactic treatment. General linear modelling was used to investigate the association of neurocognitive outcomes with risks factors, adjusting for age and education attainment. RESULTS: Forty-two patients were recruited (median age 32.0 years; 78.6% haemophilia A; 80.9% moderate-to-severe disease). Six patients (14.3%) had developed cerebral microbleeds. A subgroup of patients demonstrated impairments in cognitive flexibility (30.9%) and motor processing speed (26.2%). Hemarthrosis in the previous year was associated with worse attention (Estimate = 7.62, 95% CI: 1.92-15.33; p = .049) and cognitive flexibility (Estimate = 8.64, 95% CI: 2.52-13.29; p = .043). Depressive (Estimate = 0.22, 95% CI: 0.10-0.55; p = .023) and anxiety (Estimate = 0.26, 95% CI: 0.19-0.41; p = .0069) symptoms were associated with inattentiveness. Among patients receiving prophylactic treatment (71.4%), medication adherence was positively correlated with cognitive flexibility (p = .037). CONCLUSION: A substantial proportion of patients with haemophilia demonstrated cognitive impairment, particularly higher-order thinking skills. Screening for cognitive deficits should be incorporated into routine care. Future studies should evaluate the association of neurocognitive outcomes with occupational/vocational outcomes.

Clinical outcomes of patients with acute lymphoblastic leukemia receiving the hyper-CVAD regimen and assessment of the risk of hepatitis flares due to hepatitis B virus reactivation after chemotherapy.

INTRODUCTION: Hyper-CVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin and dexamethasone) has become a popular regimen for adults with acute lymphoblastic leukemia (ALL). We assessed the efficacy and tolerability of hyper-CVAD in the treatment of adult ALL. MATERIAL AND METHODS: We retrospectively reviewed ALL patients aged 18 or above receiving the hyper-CVAD regimen. We assessed complete remission rate and overall survival, as well as hepatitis B carrier rate and hepatitis flare due to hepatitis B virus (HBV) reactivation. RESULTS: Fifty-two patients were treated with the hyper-CVAD regimen. The median age at diagnosis was 42 years; 27% of patients were Philadelphia (Ph) chromosome positive. The complete remission (CR) rate was 90.4% after the first cycle of chemotherapy. The induction mortality rate was 1.9%. Three patients required two cycles of hyper-CVAD to achieve CR. The median overall survival was 39.6 months and the 5-year overall survival was 50%. Age over 30 years and white blood cell count of more than 30 × 109/l were found to be prognostic for poor overall survival in multivariate analysis. The hepatitis B carrier rate was 17% in our cohort, and the rate of hepatitis flare due to HBV reactivation was 11% in patients with current infection. CONCLUSIONS: Hyper-CVAD is feasible and tolerable with a good CR rate in the treatment of adult ALL patients. It is an option for the treatment of ALL. Antiviral prophylaxis should be considered in ALL patients with HBV infection to reduce the risk of HBV reactivation.

Diagnostic and treatment hurdles in plasma cell myeloma with t(11;14) translocation: A case report.

t(11;14) translocation is one of the most common chromosomal abnormalities in plasma cell myeloma. The present case report presented a case of plasma cell myeloma with t(11;14) translocation, in which the plasma cells were small lymphoplasmacytoids in morphology with positive cluster of differentiation-20 and Cyclin D1 expression. These results led to initial diagnostic difficulties. The patient was refractory to bortezomib-based therapy, and responsive to vincristine, doxorubicin and dexamethasone. However, the prognostic value of t(11;14) in plasma cell myeloma remains to be determined. With recent advances in treatment options, physicians should be aware of the clinical and pathological characteristics of this translocation in plasma cell myeloma.

Post-transplant lymphoproliferative disorder presenting with skin ulceration in a renal transplant recipient who achieved sustained remission with rituximab therapy: A case report.

Post-transplant lymphoproliferative disorder (PTLD) is associated with a variety of clinical presentations, but rarely involves the skin. We herein report a case of PTLD presenting with skin ulceration in a renal transplant recipient. A biopsy of the ulcer confirmed the diagnosis of diffuse large B-cell lymphoma. The patient was initially treated with immunosuppression reduction, but the skin ulcer persisted. He was then treated with two courses of chemotherapy, but his condition was complicated with cryptococcal infection. Antifungal agents were administered to control the fungal infection. The patient later developed lymphoma recurrence and was successfully treated with single-agent rituximab. The patient remains well 6 years after treatment, with no evidence of disease relapse. Therefore, PTLD may manifest as skin lesions and physicians must be aware of this rare presentation.

Effects of addition of rituximab to chemotherapy on central nervous system events in patients with diffuse large B-cell lymphoma.

The aim of this study was to evaluate whether the addition of rituximab to chemotherapy reduces central nervous system (CNS) events and to identify the risk factors associated with CNS involvement. Patients who were diagnosed with diffuse large B-cell lymphoma (DLBCL) between January, 1995 and December, 2012, without prior CNS disease, were recruited in this study. The patients received chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisolone (CHOP) or CHOP with rituximab (R-CHOP), with curative intent. The incidence rate of subsequent CNS events was compared between the two groups. A total of 110 patients were recruited, 45 (41%) of whom received CHOP and 65 (59%) R-CHOP. A total of 12 patients (10.9%) subsequently exhibited CNS involvement. The median time from the initial DLBCL diagnosis to CNS disease was 6.7 months (range, 1.3-23.8 months). The CNS disease rate was 15.5% (7/45) in the CHOP group vs. 7.6% (5/65) in the R-CHOP group. The projected 3-year CNS disease rate was 18% in the CHOP group vs. 9% in the R-CHOP group (P=0.15). The survival of patients with CNS disease was poor, with a median survival of 5.8 months. On multivariate analysis using the Cox proportional model, stage IV disease remained an independent predictor of CNS disease (hazard ratio = 7.75, 95% confidence interval: 1.67-35.92, P=0.009). In conclusion, the addition of rituximab to chemotherapy did not appear to reduce the risk of CNS events in our study. Other effective prophylactic measures are required to reduce the incidence of CNS events. High-dose intravenous methotrexate crosses the blood-brain barrier and may be used as CNS prophylaxis in high-risk patients.

Rare case of intussusception in an adult with acute myeloid leukemia.

Intussusception is rarely reported in adult patients with acute leukemia. We report a case of intussusception in a 29-year-old woman with acute myeloid leukemia (AML). She developed right lower quadrant pain, fever, and vomiting on day 16 of induction chemotherapy. Physical examination showed tenderness and guarding at the right lower quadrant of the abdomen. Abdominal computed tomography (CT) showed distension of the cecum and ascending colon, which were filled with loops of small bowel, and herniation of the ileocecal valve into the cecum. We proceeded to laparotomy and revealed ileocecal intussusception with the ileocecal valve as the leading point. The terminal ileum was thickened and invaginated into the cecum, which showed gangrenous changes. Right hemicolectomy was performed and microscopic examination of the colonic tissue showed infiltration of leukemic cells. The patient recovered after the operation and was subsequently able to continue treatment for AML. This case demonstrates that the diagnosis of intussusception is difficult because the presenting symptoms can be non-specific, but abdominal CT can be informative for preoperative diagnosis. Resection of the involved bowel is recommended when malignancy is suspected or confirmed. Intussusception should be considered in any leukemia patients presenting with acute abdomen. A high index of clinical suspicion is important for early diagnosis.

Clinical features and treatment outcomes of Hodgkin's lymphoma in Hong Kong Chinese.

INTRODUCTION: Little information is available on the outcomes of Hodgkin's lymphoma in Chinese patients. We analyzed the clinical and histopathological characteristics, treatment types, clinical course and treatment outcomes of Hong Kong Chinese patients. MATERIAL AND METHODS: Patients with Hodgkin's lymphoma diagnosed from January 1991 to December 2010 were recruited. A retrospective analysis of these patients was performed. RESULTS: Sixty-seven Chinese patients (38 males and 29 females) were identified and the median age was 36 (range 16-80). Nodular sclerosis was the most common histology (54%), followed by mixed cellularity (36%). Twenty-four patients had early favorable, 20 patients had early unfavorable and 23 patients had advanced-stage diseases. The most common presentation was palpable lymph node or mass (85%) followed by fever, weight loss, night sweating and mediastinal mass. Ninety percent of patients received chemotherapy and 40% received radiotherapy as consolidation. Seven patients with stage I lymphoma received radiotherapy alone. ABVD (doxorubicin, bleomycin, vinblastine and dacarbazine) was the most commonly used chemotherapeutic regimen. Following treatment, 87% of patients achieved complete remission. Six patients relapsed after first remission and 3 achieved second remission after re-induction therapy. The 5-year overall survival of the entire cohort was 89% and the freedom from treatment failure (FFTF) at 5 years was 82%. The 5-year overall survival rate for early favorable, early unfavorable and advanced stages was 95.7%, 95.0% and 74.7%, respectively. CONCLUSIONS: Despite the relatively low incidence of Hodgkin's lymphoma in Hong Kong Chinese, the treatment outcomes are comparable to Caucasian patients.