Patients with multiple myeloma (MM) are susceptible to developing thrombotic microangiopathies (TMAs), an etiologically diverse group of syndromes which include atypical hemolytic uremic syndrome (aHUS) and thrombotic thrombocytopenic purpura (TTP). The TMAs are characterized by thrombocytopenia and microangiopathic hemolytic anemia (MAHA), and are associated with a high mortality risk and irreversible end-organ damage when treatment is delayed. In MM patients, TMAs may be triggered by specific chemotherapies, bone marrow transplantation (BMT), and progression of underlying disease. Because many characteristics of TMAs overlap with sequelae of MM and its treatments, diagnosis requires a high index of suspicion. Furthermore, our understanding of optimal treatments for these entities is rapidly evolving and clinical practice guidelines do not yet exist. Historically, consideration of a diagnosis of TMA has prompted initiation of therapeutic plasma exchange. In this review, we present an overview of the MM-related TMAs, an approach to workup and diagnosis, and argue for initial empiric MM-related TMA treatment with eculizumab rather than plasma exchange.
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http://dx.doi.org/10.1016/j.leukres.2019.106195 | DOI Listing |
J Pediatr Hematol Oncol
January 2025
Departments of Pediatric Hematology.
Congenital thrombotic thrombocytopenic purpura (cTTP), which is associated with mutations in the gene for a disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13 (ADAMTS13), is a chronic and lifelong disease. The clinical course is variable. Regularly using ADAMTS13-containing products such as fresh frozen plasma (FFP) for long-term prophylaxis is the most important treatment to prevent thrombotic microangiopathy (TMA) episodes.
View Article and Find Full Text PDFThis article presents a clinical case of ocular thrombotic microangiopathy of mixed origin (antiphospholipid syndrome, malignant arterial hypertension, multigenic thrombophilia). Multimodal imaging of the fundus provides a detailed assessment of its structures. Pathological changes in the choroid, the "retinal pigment epithelium - Bruch's membrane" complex, and the neurosensory retina, identified using fundus photography, short-wavelength autofluorescence, optical coherence tomography (OCT), and OCT angiography, are described as nonspecific in nature.
View Article and Find Full Text PDFToxins (Basel)
December 2024
Department of Anesthesiology and Intensive Care Medicine, Jena University Hospital, Am Klinikum 1, 07747 Jena, Germany.
Hemolytic-uremic syndrome (HUS) is a systemic complication of an infection with Shiga toxin (Stx)-producing enterohemorrhagic , primarily leading to acute kidney injury (AKI) and microangiopathic hemolytic anemia. Although free heme has been found to aggravate renal damage in hemolytic diseases, the relevance of the heme-degrading enzyme heme oxygenase-1 (HO-1, encoded by ) in HUS has not yet been investigated. We hypothesized that HO-1 also important in acute phase responses in damage and inflammation, contributes to renal pathogenesis in HUS.
View Article and Find Full Text PDFCureus
November 2024
Department of Medicine, Mercyhealth Graduate Medical Education (GME) Consortium, Rockford, USA.
Thrombotic microangiopathies (TMA) are a group of conditions that present with varying degrees of microthrombi, thrombocytopenia, microangiopathic hemolytic anemia, renal dysfunction, and neurological impairment. Etiologies can be primary, such as thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), and atypical hemolytic uremic syndrome (aHUS), or secondary, such as due to systemic infections, malignancies, immune-mediated conditions, and hypertensive emergencies. In hypertensive emergencies, this presentation can occur from mechanical stress placed on red blood cells as they pass through narrowed arteries due to edema and microangiopathic changes within the vessels themselves.
View Article and Find Full Text PDFProbl Radiac Med Radiobiol
December 2024
State Institution «National Research Center for Radiation Medicine, Hematology and Oncology of the National Academy of Medical Sciences of Ukraine», 53 Yuriia Illienka Str., Kyiv, 04050, Ukraine.
We present а rare clinical case of successful surgical treatment - combined left-sided hemicolectomy and cecal resection for colon cancer of two locations in a patient with idiopathic thrombocytopenic purpura, which, in our opinion, is due to radiation exposure is presented. It is shown that the biological effects of long-term ionizing radiation on critical organs can have a diverse nature both in terms of the time of occurrence and in their structure, which requires an individual approach, the involvement of related specialists and the necessary medical preoperative preparation for precise planning of surgical intervention taking into account all possible risks.
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