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Geir E Tjønnfjord

Publications and source records attributed to Geir E Tjønnfjord.

16 recordsLinked to original sources
PubMed2006

Molecular cytogenetic analysis of leukemic mantle cell lymphoma with a cryptic t(11;14).

We report a case of leukemic mantle cell lymphoma of the blastoid variant with complex chromosomal rearrangements leading to the recombination of BCL1 from 11q13 and IGH from 14q32. G-banding analysis showed, in addition to multiple other chromosome abnormalities, a del(11)(q13) and addition of unknown material to chromosome arms 13p and 21p. The two latter rearrangements were revealed, by use of multicolor in situ hybridization (M-FISH) analysis, to be a der(13)(qter-->p11::14q32::11q13-->11q22::14q32-->14q11::11q22-->11qter) and a similar der(21)(qter-->p11::14q32::11q13-->11q23::14q32-->14q11::11q22-->11qter). FISH with locus-specific probes for BCL1 and IGH showed a fusion signal on both derivative chromosomes.

Chromosome Banding↗
PubMed2006

Surgery in patients with hemophilia and inhibitors: a review of the Norwegian experience with FEIBA.

Surgical interventions in patients with hemophilia and inhibitors have often been postponed as long as possible due to difficulties in maintaining intra- and postoperative hemostatic control. Nonactivated and activated prothrombin complex concentrates have been successful in controlling acute bleeding in patients with inhibitors and have been useful in the surgical setting. At the Rikshospitalet-Radiumhospitalet University Hospital in Oslo, Norway, 17 minor and seven major surgical procedures were performed in nine patients with congenital hemophilia A and two patients with acquired hemophilia. Patients are generally treated according to the following dosing regimen, with changes made on a case-by-case basis: a preoperative loading dose of 100 U/kg of Factor Eight Inhibitor Bypassing Activity, Anti-Inhibitor Coagulant Complex, Vapor Heated (FEIBA; Baxter AG, Vienna, Austria), followed by 200 U/kg per day for 3 days. The dose is then tapered to 150 U/kg per day and subsequently to 100 U/kg per day. Hemostatic control was attained in all cases and only 1 major adverse event was observed. A 69-year-old patient experienced a non-ST-elevation myocardial infarction 3 days after undergoing a sigmoidectomy. He continued on FEIBA therapy and was stabilized with nitrates, opioid analgesia, and diuretics without clinical signs of heart failure.

Adult↗
PubMed2006

A common origin of the 4143insA ADAMTS13 mutation.

Severely deficient activity of the von Willebrand Factor (VWF) cleaving metalloprotease, ADAMTS13, is associated with thrombotic thrombocytopenic purpura (TTP). The mutation spectrum ofADAMTS13 is rather heterogeneous, and numerous mutations spread across the gene have been described in association with congenital TTP. The 4143insA mutation is unusual with respect to its geographic concentration. Following the initial report from Germany in which the 4143insA mutation was detected in four apparently unrelated families, we have now identified this mutation in a further eleven patients from Norway, Sweden, Poland, Germany, the Czech Republic and Australia. Confirmation that the Australian patient is of German ancestry, together with the Northern and Central European origin of most of the other patients, suggests that the 4143insA mutation has a common genetic background. We established ADAMTS13 haplotypes by analyzing 17 polymorphic intragenic markers. The haplotypes linked to 4143insA were identical in all informative families. Three novel candidate mutations, C347S, P671L and R1060W, as well as the known mutation R507Q, were also identified during the course of the study. We conclude that 4143insA has a common genetic background and is frequent among patients with hereditary ADAMTS13 deficiency in Northern and Central European countries.

ADAM Proteins↗
PubMed2006

Primary chronic cold agglutinin disease: a population based clinical study of 86 patients.

BACKGROUND AND OBJECTIVES: Our knowledge of primary chronic cold agglutinin disease (CAD) is incomplete. The aim of this study was to collect comprehensive and precise data the on epidemiology, clinical and pathological features, course, and therapy of CAD. DESIGN AND METHODS: We performed a population-based retrospective follow-up study of as many as possible of all CAD patients in Norway. Eighty-six patients were studied. RESULTS: The prevalence of primary CAD was 16 cases per million inhabitants. The incidence rate was 1 per million per year. The median age at onset was 67 years (range, 30-92) and the male to female ratio was 0.55. The median survival was 12.5 years from onset. Autoimmune diseases other than CAD were reported in 8% of patients, cold-induced circulatory symptoms in 91%, and exacerbation of hemolytic anemia during febrile illness in 74%. At least 51% had received red blood cell transfusions. The mean initial hemoglobin level was 9.2 g/dL (range, 4.5-15.6) and the median monoclonal immunoglobulin level 4.0 g/L (range, 0.0-47.3). Most laboratory findings did not change significantly during a median follow-up of 5 years. Monoclonal IgM was detected in 90%; IgG and IgA in 3.5% each; with kappa light chains in 94%. An abnormal kappa/lambda ratio in bone marrow was found in 90%, lymphoma in 76%, and lymphoplasmacytic lymphoma in 50%. Transformation to aggressive lymphoma occurred in 3.5% during 10 years. Rituximab therapy was the only treatment showing acceptable response rates (60%). INTERPRETATION AND CONCLUSIONS: Primary CAD represents a spectrum of clonal lymphoproliferative bone marrow disorders, in most cases with morphological signs of lymphoma. Despite a favorable prognosis for survival, the disease is not indolent in terms of clinical manifestations.

Adult↗
PubMed2005

[Surgery in patients with bleeding disorders--expensive treatment for a small group of patients].

BACKGROUND: During surgical procedures, patients with bleeding disorders have a major risk of bleeding complications. To reduce the risk of bleeding it is necessary to provide pre-, peri- and postoperative antihaemorrhagic therapy. In less severe bleeding disorders, pharmacologic treatment may be sufficient, but in patients with severe bleeding disorders there is always a need for clotting factor concentrates. MATERIALS AND METHODS: This study includes all patients with bleeding disorders admitted to Rikshospitalet University Hospital between 1997 and 2003 for surgical procedures during which therapy with clotting factor concentrates was mandatory. RESULTS AND INTERPRETATION: Over the study period, 135 patients underwent a total of 255 different surgical procedures. In 47% of the patients there was a causal relationship between the need for surgery and the bleeding disorder. Our results show that patients with severe bleeding disorders, including patients with inhibitors, can be treated safely provided that the patients receive adequate treatment with clotting factor concentrates. However, substitution therapy with clotting factor concentrates is very expensive. Cost related to substitution therapy is the main determinant of how many patients with severe bleeding disorders can undergo elective surgery in our hospital each year.

Blood Coagulation Disorders↗
PubMed2005

Continuous infusion of recombinant factor VIIa for surgery in patients with deficiency of factor VII.

The administration of recombinant activated factor VII (rFVIIa) by continuous infusion has provided a safe and convenient alternative to bolus injections in haemophiliacs with inhibitors, but it has only been reported in a single case with congenital factor VII (FVII) deficiency. The results of 12 consecutive surgical procedures in 7 patients with congenital FVII deficiency are reported here. rFVIIa was always given in continuous infusion,aiming at plasma FVII activity of 0.5 IU/mL. Treatment was given for 2 to 7 days with a mean total dose of 7.8 mg rFVIIa. Blood loss was as expected from the different types of procedures and the only thromboembolic complication was a superficial thrombophlebitis at the infusion site. This mode of substitution was therefore safe, effective and well tolerated.

Adolescent↗
PubMed2004

[Treatment of T-cell prolymphocytic leukemia with monoclonal anti- CD52 antibody (alemtuzumab].

INTRODUCTION: T-cell prolymphocytic leukaemia (T-PLL) is a rare post-thymic T-cell malignancy with an aggressive clinical course. It has generally been resistant to alkylating chemotherapy, but some effect has been observed with the purine analog 2-deoxycoformicin with documented partial or complete response rates in up to 45% of patients. Treatment with monoclonal antibodies against CD 52 has been shown to be highly effective in T-PLL with response rates of up to 76%. This may allow for further consolidating treatment with high-dose chemotherapy with autologous stem cell support or allogeneic stem cell transplantation. MATERIALS AND METHODS: Five patients treated at Rikshospitalet University Hospital are evaluated and the literature on T-PLL is reviewed. RESULTS AND INTERPRETATION: Four of our patients were treated with alemtuzumab. Three showed complete or partial response. One patient underwent allogeneic stem cell transplantation with an HLA-identical sibling, but died on day 21 as a result of transplantation complications. The treatment is generally well tolerated; the principal management problem is immunosuppression, as shown in one patient who developed a varicella-zoster meningoencephalomyelitis as a consequence of not receiving antiviral prophylaxis. The main infusion-related adverse effects are fever and chills.

Adult↗
PubMed2003

Rituximab for primary chronic cold agglutinin disease: a prospective study of 37 courses of therapy in 27 patients.

Conventional therapies for primary chronic cold agglutinin disease (CAD) are ineffective, but remissions after treatment with the anti-CD20 antibody rituximab have been described in a small, prospective trial and in some case reports. In this study we report on 37 courses of rituximab administered prospectively to 27 patients. Fourteen of 27 patients responded to their first course of rituximab, and 6 of 10 responded to re-treatment. In both groups combined, responses were achieved after 20 of 37 courses, giving an overall response rate of 54%. We observed 1 complete and 19 partial responses. Two nonresponders and 3 patients who experienced relapse received second-line therapy with interferon-alpha combined with a new course of rituximab, and 1 nonresponder and 2 patients who experienced relapse achieved partial responses. Responders achieved a median increase in hemoglobin levels of 40 g/L (4 g/dL). Median time to response was 1.5 months, and median observed response duration was 11 months. We conclude that rituximab is an effective and well-tolerated therapy for CAD. Histologic and flow cytometric findings suggest that some of the effect may be mediated by mechanisms other than the elimination of clonal lymphocytes. We were unable to predict responses from the hematologic, immunologic, or histologic parameters before therapy.

Anemia, Hemolytic, Autoimmune↗
PubMed2003

CD7 expression by CD34+ cells in CML patients, of prognostic significance?

The purpose of the study was to identify a unique immunophenotype of normal or Philadelphia chromosome positive (Ph+) CD34+ cells that might be used to purify normal CD34+ cells from chronic myelogenous leukemia (CML) patients. An immunophenotypical study of CD34+ bone marrow cells of 20 patients with CML at diagnosis and during hydroxyurea treatment, and 39 controls were performed. All patients were Ph+, two patients had variant translocations and three patients displayed cytogenetic signs of clonal evolution. The immature progenitor cell compartment (CD34+ HLA-DR- and CD34+ CD38- cells) was comparable. The CD34+ AC133+ progenitor cell compartment was decreased in CML patients. We found no difference for any of the adhesion molecules examined except for CD62L, where the percentage of CD34+ CD62L+ cells was decreased in CML patients. The number of myeloid progenitors (CD34+ CD33+) was increased at the expense of B-lymphoid progenitors (CD34+ CD10+ and CD34+ CD19+) in CML patients indicating that B-lymphopoiesis is inhibited in CML. The megakaryocytic (CD34+ CD61+) and erythroid (CD34+ CD71+) progenitors were increased in CML patients. The number of CD34+ CD7+ cells was also significantly increased (mean 25.3% vs. 4.9%). However, the level of CD7 expression was quite heterogeneous, and the patients could be separated into two populations according to CD7 expression (more or less than 20% CD7+ CD34+ cells). The Sokal and Hasford risk scores did not differ between CD34+ CD7- CML and CD34+ CD7+ CML, but all patients with signs of disease progression clustered in the CD34+ CD7+ population indicating that the level of CD7 expression on CD34+ cells may be of prognostic importance in CML.

Adult↗
PubMed2002

[Hairy cell leukemia treated with cladribine].

BACKGROUND: Hairy cell leukaemia is a chronic B-cell disorder that follows an indolent course. Cladribine has in the last decade emerged as the drug of choice for treating hairy cell leukaemia. MATERIAL: We report on the long-term follow-up of 26 patients treated from January 1992 to June 1993 with cladribine administered subcutaneously. RESULTS: 25 patients were evaluable for response. 21 patients (84%) achieved complete remission, three patients (12%) achieved partial remission, and one patient had no response. At a median follow-up of 6.8 years, 24 patients (92%) were still alive. One patient died from infections four months after treatment, while the other patient died from a malignant melanoma 4.4 years after treatment. Relapse assessed by flow cytometry was diagnosed in 95% of the patients. 38% of those in complete and 67% of those in partial remission were treated by a second course of cladribine during the follow-up. Retreatment led to normalisation of peripheral blood count in all patients. INTERPRETATION: Cladribine is not a curative treatment in hairy cell leukaemia, but it induces long lasting remission.

Adult↗
PubMed2002

[Intensive chemotherapy in Burkitt's lymphoma and aggressive non-Hodgkin's lymphoma].

BACKGROUND: Clinical studies over the last 20 years using more intensive cytostatic regimens show improved results in children and adolescents with aggressive non-Hodgkin's lymphoma and in adult patients specifically with Burkitt's lymphoma. MATERIAL AND METHODS: We present a retrospective analysis of the use of the Berlin-Frankfurt-Munster (BFM) regimen for patients older than 15 years from three Norwegian university hospitals during the 1992-99 period. RESULTS: Survival data for 24 patients 15-69 years old with Burkitt's lymphoma/B-cell acute lymphoblastic leukaemia (B-ALL) show an estimated overall five year survival of 70% (75% for Burkitt's lymphoma only). Eight of ten adolescent patients 15-20 years old with other aggressive lymphomas were alive and disease free at last follow-up. All nine patients given the regimen after failure of prior therapy died of lymphoma within six years. INTERPRETATION: The BFM regimen yields impressive results as the primary treatment of adolescent and adult patients with Burkitt's lymphomas/B-ALL.

Adolescent↗
PubMed2002

[Paroxysmal nocturnal hemoglobinuria--a rare disease with many faces].

BACKGROUND: Paroxysmal nocturnal haemoglobinuria (PNH) is a rare acquired clonal disorder of haematopoietic stem cells causing intravascular haemolysis, haemoglobinuria and occasionally severe venous thrombotic complications. PNH is associated with severe aplastic anaemia. MATERIAL AND METHODS: A total of nine patients referred to and followed at Rikshospitalet University Hospital over a period of more than 20 years were evaluated and compared with other patient materials. RESULTS: All patients are alive with a median observation time of 46 months (7-258 months). One patient has only limited clinical signs of PNH six years after diagnosis, one patient underwent allogenic bone marrow transplantation with an HLA-identical sibling and is healthy and free of disease nine and a half years posttransplant. Three patients have experienced severe thrombotic episodes, two with development of Budd-Chiari's syndrome despite anticoagulant treatment. Five patients including these two patients are being treated with anticoagulant therapy. Two patients probably having PNH have gone through pregnancies with normal delivery of healthy children. INTERPRETATION: These nine cases illustrate the many different aspects of this rare disease, including a relatively favorable prognosis with respect to survival.

Adolescent↗