Child ; Humans ; Plasma Exchange ; Thrombotic Microangiopathies ; therapy

China ; Consensus ; Humans ; Purpura, Thrombotic Thrombocytopenic ; Thrombotic Microangiopathies/therapy*

Mitomycin (MMC) is a naturally ocurring alkylating agent, introduced for clinical use as early as 1958. This drug is useful in the therapy of gastrointestinal carcinomas when used in combination with 5-fluorouracil. Nephrotoxicity among toxicities from MMC is unusual with cumulative doses less than 30 mg/m2. In large studies in which the incidence of MMC nephrotoxicity were assessed, 3-15% of patients developed total dose related renal dysfunction. Three patients in our clinical practice have developed thrombotic microangiopathy clearly related to MMC. We report the clinical and pathologic features of our cases. In view of the probable dose-related and delayed toxicity of MMC, it seems necessary to monitor regularly after initiation of chemotherapy. Early detection of the renal impairment and withdrawal of MMC might halt further progression of renal failure.
Drug Therapy ; Fluorouracil ; Humans ; Incidence ; Mitomycin* ; Renal Insufficiency ; Thrombotic Microangiopathies*

No abstract available.
Catheters* ; Humans ; Oxygen Inhalation Therapy ; Oxygen* ; Thrombotic Microangiopathies*

No abstract available.
Catheters* ; Humans ; Oxygen Inhalation Therapy ; Oxygen* ; Thrombotic Microangiopathies*

Humans ; Lupus Erythematosus, Systemic/therapy* ; Thrombotic Microangiopathies/complications*

Thrombotic microangiopathy (TMA) is a group of highly heterogeneous, acute and severe clinicopathological syndromes, characterized by microangiopathic hemolytic anemia (MAHA), thrombocytopenia and ischemic injury of end organs. TMA has the characteristics of dangerous condition, multiple organ involvement and high mortality. Patients with severe TMA need to be admitted to intensive care unit (ICU) for organ function support therapy. Early and rapid evaluation, differential diagnosis, and timely and effective treatment are the key to improve the prognosis of TMA patients. Here, we review the pathophysiological changes, diagnosis differential diagnosis, and treatment of the severe TMA in adult.
Adult ; Humans ; Thrombotic Microangiopathies/therapy* ; Purpura, Thrombotic Thrombocytopenic/therapy* ; Anemia, Hemolytic/therapy* ; Treatment Outcome ; Diagnosis, Differential

Transplantation-associated thrombotic microangiopathy (TA-TMA) is one of the serious complications mostly occurring within 100 days after hematopoietic stem cell transplantation (HSCT). Risk factors of TA-TMA include genetic predispositions, GVHD, and infections. The pathophysiological mechanisms of TA-TMA start with endothelial injury caused by complement activation, which leads to microvascular thrombosis, and microvascular hemolysis, ultimately resulting in multi-organ dysfunction. In recent years, the development of complement inhibitors has markedly improved the prognosis of TA-TMA patients. This review will give an update on risk factors, clinical manifestations, diagnosis, and treatment of TA-TMA, so as to provide references for clinical practice.
Humans ; Thrombotic Microangiopathies/therapy* ; Prognosis ; Thrombosis/etiology* ; Risk Factors ; Hematopoietic Stem Cell Transplantation/adverse effects*

Pericardial effusion (PcE) is one of the uncommon complications after hematopoietic stem cell transplantation (HSCT). Although many causes are related with PcE after HSCT, PcE after HSCT is usually late-onset and can be presented as a sign of acute or chronic graft-versus-host disease in allogeneic transplantation. Previous reports of PcE after autologous HSCT are very uncommon. Transplantation-associated thrombotic microangiopathy (TA-TMA) is a kind of renal microvascular complications after HSCT, which is similar to thrombotic thrombocytopenic purpura. The authors report a case of early-onset PcE, which maybe resulted from TA-TMA, after high-dose chemotherapy and autologous peripheral blood HSCT in a 4-year-old child with neuroblastoma.
Child ; Drug Therapy ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells ; Humans ; Neuroblastoma ; Pericardial Effusion ; Preschool Child ; Purpura, Thrombotic Thrombocytopenic ; Thrombotic Microangiopathies ; Transplantation, Homologous

OBJECTIVES: To investigate the clinical characteristics, pathological features, treatment regimen, and prognosis of children with lupus nephritis (LN) and thrombotic microangiopathy (TMA), as well as the treatment outcome of these children and the clinical and pathological differences between LN children with TMA and those without TMA. METHODS: A retrospective analysis was conducted on 12 children with LN and TMA (TMA group) who were admitted to the Department of Nephrology, Children's Hospital of Nanjing Medical University, from December 2010 to December 2021. Twenty-four LN children without TMA who underwent renal biopsy during the same period were included as the non-TMA group. The two groups were compared in terms of clinical manifestations, laboratory examination results, and pathological results. RESULTS: Among the 12 children with TMA, 8 (67%) had hypertension and 3 (25%) progressed to stage 5 chronic kidney disease. Compared with the non-TMA group, the TMA group had more severe tubulointerstitial damage, a higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score at onset, and higher cholesterol levels (P<0.05). There were no significant differences between the two groups in the percentage of crescent bodies and the levels of hemoglobin and platelets (P>0.05). CONCLUSIONS There is a higher proportion of individuals with hypertension among the children with LN and TMA, as well as more severe tubulointerstitial damage. These children have a higher SLEDAI score and a higher cholesterol level.
Child ; Humans ; Lupus Nephritis/complications* ; Kidney/pathology* ; Retrospective Studies ; Thrombotic Microangiopathies/therapy* ; Prognosis ; Hypertension/complications* ; Cholesterol ; Lupus Erythematosus, Systemic