Pancytopenia due to systemic lupus erythematosus (SLE) is rarely reported, and among those reported, it is mostly due to immunologically mediated cell destruction. Pancytopenia due to bone marrow fibrosis secondary to SLE is an extremely rare entity. Myelofibrosis secondary to SLE per se is reported only in 21 cases in the literature. Ours probably is the 22nd case report on SLE with myelofibrosis. Primary presentation of SLE with bleeding manifestation is also a rare phenomenon. Partial to complete regression of myelofibrosis is noted following treatment in secondary myelofibrosis caused by SLE. We report a case of a woman in her late 40s who presented to us with bleeding manifestations of petechial rash and menorrhagia, which on further evaluation showed pancytopenia due to myelofibrosis secondary to SLE. Our case underlines multiple features like primary bleeding manifestation and regression of myelofibrosis following treatment which is rarely reported in association with SLE.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10441047 | PMC |
http://dx.doi.org/10.1136/bcr-2023-255229 | DOI Listing |
Blood Cancer J
December 2024
Department of Hematology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, PR China.
To compare the efficacy and safety of gecacitinib (also known as jaktinib) with hydroxyurea (HU) in treating myelofibrosis (MF) patients. In this multicenter, randomized phase 3 trial (ZGJAK016), intermediate- or high-risk primarily JAK inhibitor naïve MF patients were assigned in a 2:1 ratio to receive either gecacitinib (100 mg twice a day, BID) or HU (500 mg BID). The primary endpoint was the proportion of patients with ≥35% reduction in spleen volume (SVR35) from baseline at week 24.
View Article and Find Full Text PDFCancers (Basel)
December 2024
Atrium Health Wake Forest Baptist Comprehensive Cancer Center, Wake Forest University School of Medicine, Winston-Salem, NC 27157, USA.
Anemia is a common and progressive clinical manifestation of myelofibrosis that may occur as part of the disease pathogenesis as well as due to the myelosuppressive effects of some treatments, with a substantial impact on quality of life, prognosis, and healthcare resource utilization. Despite these burdens, anemia management has traditionally been a secondary priority to spleen and symptom control, due in part to the limitations of available therapeutic approaches. With the initial regulatory approvals of momelotinib, a Janus kinase 1 (JAK1), JAK2, and activin A receptor type 1 inhibitor that provides anemia-related benefits in addition to addressing splenomegaly and symptoms, re-evaluation of anemia as an early and prominent treatment consideration is warranted.
View Article and Find Full Text PDFPak J Med Sci
December 2024
Xian-hua Yuan Vice President, First Affiliated Hospital of Xingtai Medical College, Xingtai 054000, Hebei, China.
ETV6-ASCL6 fusion gene plays an important role in hematopoiesis and hematological malignancies, but it is rare in hematological diseases. This paper describes a case of etv6-ascl6 fusion gene positive patient who was successively diagnosed as myelofibrosis, eosinophilic leukemia, basophilic leukemia and secondary acute myeloid leukemia. The clinical manifestations and disease evolution have its unique characteristics.
View Article and Find Full Text PDFJ Clin Med
November 2024
Internal Medicine Unit, University Hospital "Duilio Casula", AOU Cagliari, 09123 Cagliari, Italy.
Thrombotic adverse events and disease progression are crucial in Polycythemia Vera (PV), as it stands as the leading cause of mortality. The pulse wave velocity (PWV) is a valuable indicator of arterial aging and often plays a significant independent role in contributing to cardiovascular adverse events (CV-AEs). The aim of this study was to examine the relationship between PWV and critical vascular function parameters, with the goal of identifying new predictive factors of vascular damage and exploring a potential connection with disease progression.
View Article and Find Full Text PDFFront Med (Lausanne)
November 2024
Department of Hematology, Catholic Hematology Hospital, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!