| 1. |
|
|
| 2. |
- Geyer, Holly L., et al.
(författare)
-
Associations between gender, disease features and symptom burden in patients with myeloproliferative neoplasms : an analysis by the MPN QOL International Working Group
- 2017
-
Ingår i: Haematologica. - : Ferrata Storti Foundation (Haematologica). - 0390-6078 .- 1592-8721. ; 102:1, s. 85-93
-
Tidskriftsartikel (refereegranskat)abstract
- The myeloproliferative neoplasms, including polycythemia vera, essential thrombocythemia and myelofibrosis, are distinguished by their debilitating symptom profiles, life-threatening complications and profound impact on quality of life. The role gender plays in the symptomatology of myeloproliferative neoplasms remains under-investigated. In this study we evaluated how gender relates to patients' characteristics, disease complications and overall symptom expression. A total of 2,006 patients (polycythemia vera=711, essential thrombocythemia=830, myelofibrosis=460, unknown=5) were prospectively evaluated, with patients completing the Myeloproliferative Neoplasm-Symptom Assessment Form and Brief Fatigue Inventory Patient Reported Outcome tools. Information on the individual patients' characteristics, disease complications and laboratory data was collected. Consistent with known literature, most female patients were more likely to have essential thrombocythemia (48.6% versus 33.0%; P<0.001) and most male patients were more likely to have polycythemia vera (41.8% versus 30.3%; P<0.001). The rate of thrombocytopenia was higher among males than females (13.9% versus 8.2%; P<0.001) and males also had greater red-blood cell transfusion requirements (7.3% versus 4.9%; P=0.02) with shorter mean disease duration (6.4 versus 7.2 years, P=0.03). Despite there being no statistical differences in risk scores, receipt of most therapies or prior complications (hemorrhage, thrombosis), females had more severe and more frequent symptoms for most individual symptoms, along with overall total symptom score (22.8 versus 20.3; P<0.001). Females had particularly high scores for abdominal-related symptoms (abdominal pain/discomfort) and microvascular symptoms (headache, fatigue, insomnia, concentration difficulties, dizziness; all P<0.01). Despite complaining of more severe symptom burden, females had similar quality of life scores to those of males. The results of this study suggest that gender contributes to the heterogeneity of myeloproliferative neoplasms by influencing phenotypic profiles and symptom expression.
|
|
| 3. |
- Scotch, Allison H, et al.
(författare)
-
Symptom burden profile in myelofibrosis patients with thrombocytopenia : Lessons and unmet needs
- 2017
-
Ingår i: Leukemia Research. - : Elsevier BV. - 0145-2126 .- 1873-5835. ; 63, s. 34-40
-
Tidskriftsartikel (refereegranskat)abstract
- Myelofibrosis is a myeloproliferative neoplasm associated with progressive cytopenias and high symptom burden. MF patients with thrombocytopenia have poor prognosis but the presence of thrombocytopenia frequently precludes the use of JAK2 inhibitors. In this study, we assessed quality of life and symptom burden in 418 MF patients with (n = 89) and without (n = 329) thrombocytopenia using prospective data from the MPN-QOL study group database, including the Myeloproliferative Neoplasm Symptom Assessment Form (MPN-SAF) and Total Symptom Score (MPN10). Thrombocytopenia, defined as platelet count < 100 x10(9)/L (moderate 51-100 x 10(9)/L; severe <= 50 x10(9)/L), was associated with anemia (76% vs. 45%, p < 0.001), leukopenia (29% vs. 11%, p < 0.001), and need for red blood cell transfusion (35% vs. 19%, p = 0.002). Thrombocytopenic patients had more fatigue, early satiety, inactivity, dizziness, sad mood, cough, night sweats, itching, fever, and weight loss; total symptom scores were also higher (33 vs. 24, p < 0.001). Patients with severe thrombocytopenia were more likely to have anemia (86% vs. 67%, p = 0.04), leukopenia (40% vs. 20%, p = 0.04), and transfusion requirements (51% vs. 20%, p = 0.002) but few differences in symptoms when compared to patients with moderate thrombocytopenia. These results suggest that MF patients with thrombocytopenia experience greater symptomatic burden than MF patients without thrombocytopenia and may benefit from additional therapies.
|
|
| 4. |
- Geyer, Holly, et al.
(författare)
-
Symptomatic Profiles of Patients With Polycythemia Vera : Implications of Inadequately Controlled Disease
- 2016
-
Ingår i: Journal of Clinical Oncology. - 0732-183X .- 1527-7755. ; 34:2, s. 151-
-
Tidskriftsartikel (refereegranskat)abstract
- PURPOSE: Polycythemia vera (PV) is a myeloproliferative neoplasm (MPN) associated with disabling symptoms and a heightened risk of life-threatening complications. Recent studies have demonstrated the effectiveness of JAK inhibitor therapy in patients with PV patients who have a history of prior hydroxyurea (HU) use (including resistance or intolerance), phlebotomy requirements, and palpable splenomegaly. We aimed to determine how these features contribute alone and in aggregate to the PV symptom burden.PATIENTS AND METHODS: Through prospective evaluation of 1,334 patients with PV who had characterized symptom burden, we assessed patient demographics, laboratory data, and the presence of splenomegaly by disease feature (ie, known HU use, known phlebotomy requirements, splenomegaly).RESULTS: The presence of each feature in itself is associated with a moderately high symptom burden (MPN symptom assessment form [SAF] total symptom score [TSS] range, 27.7 to 29.2) that persists independent of PV risk category. In addition, symptoms incrementally increase in severity with the addition of other features. Patients with PV who had all three features (PV-HUPS) faced the highest total score (MPN-SAF TSS, 32.5) but had similar individual symptom scores to patients with known HU use (PV-HU), known phlebotomy (PV-P), and splenomegaly (PV-S).CONCLUSION: The results of this study suggest that patients with PV who have any one of the features in question (known HU use, known phlebotomy, or splenomegaly) have significant PV-associated symptoms. Furthermore, it demonstrates that many PV symptoms remain severe independent of the number of features present.
|
|
| 5. |
- Burn, Garth L., et al.
(författare)
-
Superresolution imaging of the cytoplasmic phosphatase PTPN22 links integrin-mediated T cell adhesion with autoimmunity
- 2016
-
Ingår i: Science Signaling. - : American Association for the Advancement of Science (AAAS). - 1945-0877 .- 1937-9145. ; 9:448
-
Tidskriftsartikel (refereegranskat)abstract
- Integrins are heterodimeric transmembrane proteins that play a fundamental role in the migration of leukocytes to sites of infection or injury. We found that protein tyrosine phosphatase nonreceptor type 22 (PTPN22) inhibits signaling by the integrin lymphocyte function-associated antigen-1 (LFA-1) in effector T cells. PTPN22 colocalized with its substrates at the leading edge of cells migrating on surfaces coated with the LFA-1 ligand intercellular adhesion molecule-1 (ICAM-1). Knockout or knockdown of PTPN22 or expression of the autoimmune disease-associated PTPN22-R620W variant resulted in the enhanced phosphorylation of signaling molecules downstream of integrins. Superresolution imaging revealed that PTPN22-R620 (wild-type PTPN22) was present as large clusters in unstimulated T cells and that these disaggregated upon stimulation of LFA-1, enabling increased association of PTPN22 with its binding partners at the leading edge. The failure of PTPN22-R620W molecules to be retained at the leading edge led to increased LFA-1 clustering and integrin-mediated cell adhesion. Our data define a previously uncharacterized mechanism for fine-tuning integrin signaling in T cells, as well as a paradigm of auto-immunity in humans in which disease susceptibility is underpinned by inherited phosphatase mutations that perturb integrin function. 2016
|
|
| 6. |
|
|
| 7. |
- Prentice, H. A., et al.
(författare)
-
Patient demographic and surgical characteristics in anterior cruciate ligament reconstruction: a description of registries from six countries
- 2018
-
Ingår i: British Journal of Sports Medicine. - : BMJ. - 0306-3674 .- 1473-0480. ; 52:11
-
Tidskriftsartikel (refereegranskat)abstract
- Objective Findings from individual anterior cruciate ligament reconstruction (ACLR) registry studies are impactful, but how various registries from different countries compare with different patient populations and surgical techniques has not been described. We sought to describe six ACLR registry cohorts to understand variation across countries. Methods Five European registries and one US registry participated. For each registry, all primary ACLR registered between registry establishment through 31December 2014 were identified. Descriptive statistics included frequencies, proportions, medians and IQRs. Revision incidence rates following primary ACLR were computed. Results 101 125 ACLR were included: 21820 in Denmark, 300 in Luxembourg, 17556 in Norway, 30422 in Sweden, 2972 in the UK and 28055 in the US. In all six cohorts, males (range: 56.8%-72.4%) and soccer injuries (range: 14.1%-42.3%) were most common. European countries mostly used autografts (range: 93.7%-99.7%); allograft was most common in the US (39.9%). Interference screw was the most frequent femoral fixation in Luxembourg and the US (84.8% and 42.9%), and suspensory fixation was more frequent in the other countries (range: 43.9%-75.5%). Interference was the most frequent tibial fixation type in all six cohorts (range: 64.8%-98.2%). Three-year cumulative revision probabilities ranged from 2.8% to 3.7%. Conclusions Similarities in patient demographics and injury activity were observed between all cohorts of ACLR. However, graft and fixation choices differed. Revision rates were low. This work, including >100 000 ACLR, is the most comprehensive international description of contemporary practice to date.
|
|
| 8. |
|
|
| 9. |
|
|
| 10. |
- Alentorn-Geli, Eduard, et al.
(författare)
-
Factors predictive of poorer outcomes in the surgical repair of multiligament knee injuries.
- 2019
-
Ingår i: Knee surgery, sports traumatology, arthroscopy : official journal of the ESSKA. - : Springer Science and Business Media LLC. - 1433-7347. ; 27:2, s. 445-459
-
Tidskriftsartikel (refereegranskat)abstract
- To investigate the influence of injury and treatment factors on clinical/functional outcomes in multiligament knee injuries (MLKI).Thirty-nine consecutive patients with confirmed and surgically treated MLKI who met inclusion criteria were scheduled for a follow-up visit to obtain: SF-12 and subjective feeling of normalcy between the operated and healthy knee, and IKDC, active range of motion (ROM), and stability exam (Lachman test, posterior drawer, and dial test at 30°). A chart review was used to obtain data on injury and treatment factors.The postoperative mean (SD) outcomes were: IKDC score 62.7 (25.9), flexion-extension ROM 125° (29°), and percentage of normalcy 74% (20%). The postoperative normal/nearly normal stability exam was: Lachman test 36 (95%) patients, posterior drawer at 90° 38 (97%) patients, and dial test of 39 (100%) patients. There were 24 (61.5%) and 23 (59%) patients with complications and reoperations, respectively. The presence of bicruciate injuries was associated with worse Lachman (p=0.03) and posterior drawer tests (p=0.03). Presence of injury to meniscal structures was associated with worse Lachman test (p=0.03), lower percentage of normalcy (p=0.02) and extension lag (p=0.04). Injury to cartilage structures was associated with worse IKDC scores (p=0.04). IKDC was lower in cases of posterolateral corner reconstruction (p=0.03) and use of allograft tendons for reconstruction (p=0.02); ROM was lower in allograft reconstruction (p=0.02) and need for meniscal repair (p = 0.01). Bicruciate reconstruction led to worst posterior drawer test (p=0.006).The outcomes of MLKI might be negatively influenced by bicruciate ligament, meniscal, and cartilage injuries; with regards to treatment characteristics, need for posterolateral corner or bicruciate ligament reconstruction, use of allografts, or need for meniscal repair may similarly diminish outcomes. While surgical treatment provides good overall function, ROM and stability, it rarely results in a "normal" knee and the chances of complications and reoperations are high.Cross-sectional comparative study, Level III.
|
|
