Bortezomib, in combination with dexamethasone (VD) or with the addition of cyclophosphamide (VCD), is highly effective in patients with amyloid light-chain (AL) amyloidosis. Currently, VCD is considered as a primary regimen for patients with AL, but it is not clear whether the addition of cyclophosphamide to VD further and significantly improves efficacy, given the substantial activity of bortezomib itself. We retrospectively compared the outcomes of 101 patients with AL amyloidosis who received VD (n=59) or VCD (n=42) in two consecutive periods. Early mortality after adjustment for Mayo stage was similar. On intent to treat, a hematologic response rate was 68% for patients treated with VD and 78% for VCD (P=0.26), while complete response+very good partial response (CR+VGPR) rate was 47.5% and 35%, respectively. Higher doses of dexamethasone or twice-weekly bortezomib were not associated with significantly higher CR+VGPR rates. Organ responses occurred in similar rates between the two groups. Median survival was similar (33 vs 36 months, P=0.45) even after adjustment for Mayo stage and dose and schedule of bortezomib and dexamethasone. In conclusion, bortezomib even with low doses of dexamethasone is effective for the treatment of AL amyloidosis; higher doses of dexamethasone and addition of cyclophosphamide do not seem to have a profound effect on efficacy and survival.
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http://dx.doi.org/10.1038/bcj.2017.47 | DOI Listing |
Curr Treatm Opt Rheumatol
December 2024
Division of Rheumatology, Inflammation, and Immunity, Brigham and Women's Hospital, Boston, MA USA.
Purpose Of Review: To summarize the current treatment landscape of rheumatoid arthritis-associated interstitial lung disease (RA-ILD) in the context of the recent 2023 American College of Rheumatology/American College of Chest Physicians guideline for ILD treatment in systemic autoimmune rheumatic diseases.
Recent Findings: The guideline conditionally recommends mycophenolate, azathioprine, and rituximab for first-line RA-ILD therapy, with cyclophosphamide and short-term glucocorticoids as additional options. For RA-ILD progression after first line, mycophenolate, rituximab, nintedanib, tocilizumab, cyclophosphamide, and pirfenidone are conditionally recommended, while long-term glucocorticoids are conditionally recommended against.
Heliyon
July 2024
Department of Vascular Surgery, the Affiliated Hospital of Southwest Medical University, Luzhou, 646000, Sichuan, China.
Introduction: Compared to aortic dissection and isolated visceral artery dissection, multiple peripheral arterial dissections have not been formally reported to date. Currently, there is no well-established treatment for this condition, and large-scale studies with extensive sample data are lacking.
Case Presentation: A 56-year-old male, was provisionally diagnosed with " idiopathic multiple peripheral arterial dissections.
Blood Res
January 2025
Division of Hematology and Oncology, Department of Internal Medicine, Chungnam National University Hospital, 282 Munwha-Ro, Jung-Gu, Daejeon, 35015, South Korea.
Background: Post-transplantation cyclophosphamide (PTCy) and anti-thymocyte globulin (ATG) are common prophylactic strategies for graft-versus-host disease (GVHD) after haploidentical hematopoietic stem cell transplantation (haplo-HSCT). Interleukin (IL)-6 is a surrogate marker for cytokine release syndrome (CRS) and acute GVHD.
Method: The clinical outcomes and complications of haplo-HSCT with PTCy plus ATG versus PTCy monotherapy were compared according to serum IL-6 levels at Chungnam National University Hospital (Daejeon, South Korea) from January 2019 to February 2023.
Cureus
December 2024
Hematology and Medical Oncology, East Carolina University (ECU) Health Medical Center/Brody School of Medicine, Greenville, USA.
Primary cutaneous B-cell lymphoma (PCBCL) has three subtypes, among those, the leg type variant is the rarest with the highest rates of relapse and recurrence making it an intriguing focus for researchers. Nevertheless, prior to framing a diagnosis solely based on the lesion's location, it is prudent to reconsider whether it is genuinely a primary cutaneous B-cell lymphoma (PCBCL) or if it aligns more closely with the more prevalent lymphoma variants such as diffuse large B-cell lymphoma (DLBCL) with cutaneous involvement. We are reporting a case of an 85-year-old African American lady, who presented with unilateral left leg DLBCL with cutaneous involvement.
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