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Recent developments in fetal therapy
Published in Hung N. Winn, Frank A. Chervenak, Roberto Romero, Clinical Maternal-Fetal Medicine Online, 2021
Although the overall benefit of bladder shunting seems to translate into increased survival (OR 2.5; 95% CI, 1.0–5.9; p < 0.03) especially in the most severe cases (OR 8; 95% CI, 1.2–52.9; p < 0.03) (41), this hardly translates into improved renal function. Several potential explanations have recently been identified: Shunting is performed under local anesthesia to the mother as well as fetal analgesia and curarization. Shunts most commonly used are 2.5mm in diameter and placed through a 3.5mm trocar introduced percutaneously into the uterus and into the fetal bladder. It is not an easy procedure to perform and one-fourth to one-third of shunts get displaced.Shunting should optimally be performed when renal function is still normal. The best single parameter seems to be beta-2-microglobulin in fetal blood since fetal urine analysis and ultrasound assessment of the kidneys have a poor predictive value.The diagnosis of PUV is the only one amenable to significant improvement in survival and renal function. However, the diagnosis is rightly made in only around 60% of the cases in experienced hands.Overall, it is likely that shunting is too often being placed to late in the course of the natural history of the disease.
The Non-Hodgkin’s Lymphomas and Plasma Cell Dyscrasias
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Lynne V. Abruzzo, L. Jeffrey Medeiros
Plasma cell myeloma is a progressive disease with a poor prognosis. The median survival with conventional therapy is approximately 3 years. The length of survival is closely related to the stage of the disease at diagnosis. Patients are monitored with periodic determinations of serum and urine M-protein levels, and serum beta-2 microglobulin levels. Elevated levels of serum beta-2 microglobulin are associated with progressive disease and a worse prognosis. Complete remissions are unusual, and patients die of the disease. Infection is the most common cause of death. Renal failure is a contributing factor in many cases.
Case 30
Published in Atul B. Mehta, Keith Gomez, Clinical Haematology, 2017
Bone marrow aspirate and possibly aspirate of the cervical lesion will confirm the diagnosis. A skeletal survey is more sensitive than a bone scan at detecting myeloma deposits and magnetic resonance imaging (MRI) is more sensitive than x-ray. Both the beta-2 microglobulin level and the plasma C-reactive protein give useful prognostic information in myeloma.
Influence of diabetes mellitus on the biochemical parameters and outcomes of multiple myeloma
Published in Hematology, 2023
Zheng-Yue Dou, Bing Xia, Chao-Yu Wang, Yan-Jie Xu, Yi-Zhuo Zhang
The International Staging System (ISS) is an important system for predicting the prognosis of MM. Since 2005, plasma beta-2 microglobulin combined with plasma albumin concentration have been recognized as simple and powerful prognostic factors for MM patients, but the reason for the combination is still not clear. In 2007, Fonseca and San Miguel stressed that cells should also be included in evaluating the prognosis of MM patients [41]. Most ISS data were derived from data for MM patients from 1981 to 2002. All patients were treated with traditional therapy. To determine whether new drugs and strategies have an impact on ISS staging, Lu et al. found that the predictive value of ISS staging for Chinese patients with MM using the new treatment methods is still applicable [42]. It is suggested that ISS staging has little predictive effect on new therapies, while the combination of hemoglobin and plasmacytoma staging plays a predictive role in the era of new therapies [43].
Avapritinib for Systemic Mastocytosis
Published in Expert Review of Hematology, 2021
Prithviraj Bose, Srdan Verstovsek
A key concept is that the KIT D816V mutation is not restricted to the neoplastic mast cells, but is also found in other cell types in the bone marrow, e.g. eosinophils, monocytes, thus making it a better indicator of overall disease burden in patients with SM-AHN than traditional measures of mast cell burden, such as serum tryptase levels and the percentage of mast cells in the bone marrow [20–22]. Conversely, several other mutations commonly encountered in myeloid malignancies have been demonstrated in both mast cells and other cell types involved in the AHN in the bone marrow of SM patients [23,24]. Some of these have been found to be prognostically adverse, viz., SRSF2, ASXL1, RUNX1 (the so-called ‘S/A/R’ mutations) [25], EZH2 [26], NRAS [27] and DNMT3A [28]. A number of prognostic models for AdvSM, incorporating just clinical [16] or a combination of clinical and genomic variables [27,29], have recently been published. A recent study by the European Competence Network on Mastocytosis (ECNM) found only age ≥60 years and serum alkaline phosphatase ≥100 U/L to be prognostic for overall survival (OS) in non-advanced SM [16]. Serum beta-2-microglobulin levels have been shown to powerfully predict progression in non-advanced SM [28].
Effect of clinical factors on trajectory of functional performance in patients undergoing hemodialysis
Published in Renal Failure, 2021
Jin-Bor Chen, Lung-Chih Li, Wen-Chin Lee, Sin- Hua Moi, Cheng-Hong Yang
We attempted to determine the association between uremic toxins and trajectory of functional performance in patients undergoing HD. We analyzed this association with the indicators of small uremic solutes and middle molecules in circulation. Our result did not exhibit a positive association between uremic toxins and trajectory of KPS scales in patients undergoing HD, except with BUN and beta-2-microglobulin. In our previous study, we found that BUN was one of the major determinants of functional performance in patients undergoing HD by the classification and regression tree approach [8]. It is well known that HD patients have high beta-2-microglobulin levels [22]. The deposit of beta-2-microglobulin is mainly in musculoskeletal system. The preferential deposition in tendons and bones can result in physical functional impairment [22–24]. Our findings elicit a hypothesized strategy to utilize large-pore hemodialyzers to remove large-size uremic toxins. The effects of improving physical functional impairment by these hemodialyzers in HD patients warrant to be investigated in the future. Our study also implied that holistic evaluation should be taken into account in making decisions for the management of impaired functional performance in patients undergoing HD. It is concerned with not only dialysis adequacy but also other potential contributors in clinical scenario.