Tricuspid valve repair with artificial chordae in a 72-year-old woman.

Expanded polytetrafluoroethylene sutures have been used for the replacement of chordae tendineae since 1985, especially for mitral valve prolapse. There are only a few reports of artificial chordae tendineae to treat tricuspid valve regurgitation. We report on a 72-year-old woman in NYHA class III preoperatively, who underwent successful tricuspid valve repair after preoperative echocardiography revealed tricuspid valve regurgitation grade IV, caused by prolapse of the anterior leaflet (A1-A2) and annular dilatation. Tricuspid valve repair was performed using artificial chords consisting of two polytetrafluoroethylene sutures and a ring annuloplasty. Postoperative echocardiography revealed mild tricuspid valve regurgitation of less than 1°, even after three years. Gore-Tex® sutures as used in mitral valve repair are an excellent option to replace chordae tendineae in tricuspid valve prolapse. This approach represents a safe and effective technique for tricuspid valve repair.

Allogeneic SCT for relapsed composite and transformed lymphoma using related and unrelated donors: long-term results.

Outcome is poor with conventional therapy for relapsed transformed non-Hodgkin's lymphoma (NHL). Autologous SCT has been successfully employed; however the impact of allogeneic SCT has not been well defined. We therefore studied 40 consecutive patients who received allogeneic SCT for relapsed composite and transformed NHL (25 transformed, 8 composite (same site) and 7 discordant (different sites)) with related (n=25) and unrelated donors (n=15) to evaluate long-term outcome. Conditioning was myeloablative in the majority (39 of 40). Of 40 patients, 11 survive with median follow-up of 25 months. Death occurred in similar proportions due to relapsed NHL (n=14) or treatment-related complications (transplant-related mortality, TRM; n=15). The cumulative incidence of TRM was 36% at 3 years and disease relapse was 42% at 5 years. Probability of 2- and 5-year event-free survival is 36 and 23% with overall survival 39 and 23%. Performance of SCT within 1 year of NHL diagnosis predicted improved outcome. Relapse and TRM remain significant problems in this setting, indicating the need for strategies whereby patients at high risk of transformation should be selected for early SCT, ideally before their actual transformation.

Management of mesenteric vascular occlusion.

INTRODUCTION: The aim of this study was to evaluate our recent clinical management of mesenteric vascular occlusion (MVO) at Al-Ain Hospital, United Arab Emirates. METHODS: A retrospective study was performed including all patients who were diagnosed to have MVO from December 2001 to May 2005. The records were studied with regard to clinical features, risk factors, diagnosis, treatment, and outcome. RESULTS: Of the 14 patients studied, seven patients experienced mesenteric venous thrombosis (MVT), five patients mesenteric arterial occlusion (MAO), and two patients were found to have both MVT and MAO. The main risk factor for MAO was ischaemic heart disease with atrial fibrillation in four patients (80 percent). No predisposing factors were identified in three patients with MVT (primary MVT 43 percent). Contrast-enhanced computed tomography was performed in all patients and was diagnostic in 12 (86 percent) patients. Seven patients (50 percent) underwent surgery. One patient died on the ninth postoperative day (overall mortality rate 7 percent). Seven patients (50 percent) were successfully managed conservatively, five of them had only MVT, one had combined MVT and MAO, and one had only MAO. CONCLUSION: Early diagnosis and prompt initiation of anticoagulation therapy, with operative intervention when indicated, are essential for a favourable outcome.

A clinicopathological retrospective study of 131 patients with primary bone lymphoma: a population-based study of successively treated cohorts from the British Columbia Cancer Agency.

BACKGROUND: Primary bone lymphoma (PBL) is a distinct clinicopathological entity. Although PBL has been reviewed in several small studies, few reflect recent improvements in primary treatment. METHODS: We used the British Columbia Cancer Agency Lymphoid Cancer Database to identify all patients with PBL (1983-2005). All were staged in a uniform manner and treated with era-specific protocols. RESULTS: We identified 131 patients with a median age of 63 years (18-87). One third had disease in long bones and another one third had disease in the spine, of which half presented with spinal cord compression. Patients with diffuse large-cell lymphoma (DLCL) (n=103, 79%) had 5- and 10-year overall survivals (OS) of 62% and 41%, respectively. Multivariate analysis identified three prognostic groups: age<60 with International Prognostic Index (IPI) 1-3 (n=43), age>or=60 with IPI 0-3 (n=23) and age>or=60 with IPI 4-5 (n=33), with markedly different 5-year OS of 90%, 61% and 25%, respectively (P<0.0001). Neither primary site nor pathological fracture at presentation had an impact on OS. The 3-year progression-free survival in patients who received rituximab plus combination chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisone (CHOPR) chemotherapy was 88% compared with 52% in those who received CHOP-like chemotherapy without rituximab (P=0.005). The 10-year OS for those with advanced-stage disease who received irradiation plus chemotherapy was 25% versus 56% for those who received chemotherapy alone (P=0.025). Patients received irradiation if spinal cord compression was present or residual disease at the end of chemotherapy was thought to require it. CONCLUSIONS: PBL is usually of DLCL type and has an improved outcome with CHOPR. Younger patients with good IPI score have a favorable prognosis.

Novel and Mediterranean beta thalassemia mutations in the indigenous Northern Ireland population.

beta thalassemia is one of the most common genetic diseases worldwide resulting from aberrant beta-globin chain production. It is highly prevalent in regions with endemic malaria, but it is also present at low frequency in the indigenous populations of non-tropical areas such as Britain. Screening beta thalassemia trait individuals from Northern Ireland has detected 2 Mediterranean mutations, 39 (C --> T) and IVS-I-110 (G --> A); the previously reported IVS-II-850 (G --> A) mutation originally described in individuals of Scottish/English ancestry; and 2 novel mutations, initiation codon A --> C and 109 delG. Haplotype analysis indicates that the Mediterranean mutations are present on previously described haplotypes, suggesting that they have arisen due to migration. It remains to be established whether the novel mutations have arisen de novo in Northern Ireland.

Idiopathic pure red cell aplasia: first report on CD8 positive lymphocytosis in bone marrow biopsy sections.

There is no information in the literature regarding the lymphocyte content or type in bone marrow biopsies from patients with "idiopathic" pure red cell aplasia (PRCA). This report describes the bone marrow biopsy sections of a patient with PRCA. A diffuse CD3 positive (CD8 positive, granzyme B negative) lymphocytosis of approximately 1500/mm3 was revealed by immunohistochemical staining. The extent of the T cell increase was not evident from morphological examination of the bone marrow aspirate or biopsy, from flow cytometric analysis of the aspirate, or from the peripheral blood lymphocyte count. Therefore, immunohistochemical analysis should be performed routinely in this rare disease and the data acquired may help to inform the choice of treatment.

Efficacy and response to intravenous anti-D immunoglobulin in chronic idiopathic thrombocytopenic purpura.

This review explored the effectiveness of anti-D in the management of chronic idiopathic thrombocytopenic purpura (ITP). Of 16 patients, 14 non-splenectomized and two splenectomized, with chronic ITP received anti-D immunoglobulin at a dose of 50-75 mcg/kg. A total number of 100 doses anti-D were given. Fourteen patients had previous treatment with steroids, which was discontinued either because of unresponsiveness or unacceptably high maintenance doses. Two patients had no previous treatments with any modality. Anti-D was given as a short i.v. infusion whenever platelet count dropped below 30 x 10(9)/l or patient was haemorrhagic or preoperatively. Response was defined as an absolute platelet count >30 x 10(9)/l or an increment by > or =20 x 10(9)/l. Response was obtained in 14 patients with a response rate of 87%. Fifteen patients were not on any other form of treatment at the time of anti-D therapy and one patient had a concurrent steroid therapy. The improvement in platelet count lasted for more than 8 weeks post-57% of anti-D infusions. We report two patients with previous splenectomy for ITP who responded to anti-D therapy. The side-effects profile was very mild with no patients required red cell transfusion.