We report a case of hemolysis during a hemodialysis (HD) session in a 71-year-old man. His end-stage kidney disease is secondary to light-chain amyloidosis with renal involvement. Despite immunosuppressive treatment, his renal function continued to decline, and dialysis had to be initiated. Peritoneal dialysis (PD) was started but that had to be converted to HD because of pleural effusion due to PD fluid leakage. On the event day, the patient presented a respiratory distress 2 h after the initiation of HD. He developed a sudden onset of dyspnea with hypoxemia, associated with abdominal pain, nausea, and vomiting. He also presented chest pain with arterial hypertension. The pre-pump arterial and post-pump pressures were, respectively, 40 and 100 mm Hg, with no machine alarm. The blood color in the circuit changed and became darker, so HD was stopped immediately without blood restitution, and then a blood workup was obtained, and the patient was treated with oxygen therapy, IV methylprednisolone 40 mg, and IV furosemide 100 mg. Tubing checkup performed after the incident showed a kinked arterial tube which led to the suspicion of acute hemolysis. Blood transfusion was therefore urgently ordered, and the patient was immediately transferred to the intensive care unit (ICU). Artificial ventilation was required for 4 days, with initial massive blood transfusion. A 24-h treatment with extracorporeal cytokine adsorber CytoSorb<sup>®</sup> was also performed, followed by the regular HD sessions thrice weekly. Evolution was favorable, and the patient was discharged from the ICU 18 days later.
ObjectiveAntiphospholipid antibody (aPL) nephropathy (aPL-N) can be challenging to recognize due to lack of established classification or diagnostic criteria. As part of efforts to develop new antiphospholipid syndrome (APS) classification criteria (CC), the APS CC Renal Pathology subcommittee aimed to better characterize the entity of aPL-N.MethodsWe used a four-pronged approach including: 1) administering Delphi surveys to worldwide APS physicians to generate aPL-N terminology; 2) conducting literature reviews to demonstrate the association of nephropathy with aPL and identify published aPL-N histopathological terminology and descriptions; 3) evaluating aPL-N terminology used in renal biopsy reports from an international patient registry; and 4) evaluating proposed kidney pathologic features for aPL-N by assessment of international Renal Pathology Society (RPS) members.ResultsAfter completing our meta-analysis demonstrating an association of nephropathy with aPL, we used Delphi surveys, literature review, and international renal biopsy reports to develop a preliminary definition of aPL-N. These include specific terms associated with acute (i.e., thrombotic microangiopathy in glomeruli or arterioles/arteries) and chronic (i.e., organized arterial or arteriolar microthrombi with or without recanalization, organized glomerular thrombi, fibrous and fibrocellular [arterial or arteriolar] occlusions, focal cortical atrophy with or without thyroidization, and fibrous intimal hyperplasia) lesions. Most RPS survey respondents agreed with this terminology and the importance of knowing aPL results for histopathological diagnosis.ConclusionOur results support the inclusion of "aPL nephropathy" in the new APS CC, and provide the most accepted terminology for both acute and chronic pathologic lesions of aPL-N.
Background: Arteriovenous fistula delay or absence of maturation is a common problem in hemodialysis. The prevalence of antiphospholipid antibody is higher among hemodialysis patients compared to the general population and is inconsistently associated with arteriovenous fistula thrombosis or stenosis. However, the effect of antiphospholipid antibodies on delayed or absence of maturation of the arteriovenous fistula has never been studied. Methods: We retrospectively identified 103 hemodialyzed patients with arteriovenous fistula whether used or not. We collected the clinical and biological data potentially involved in arteriovenous fistula maturation and investigated the association between antiphospholipid antibody positivity and arteriovenous fistula maturation failure according to KDOQI guidelines. Results: In our cohort, the prevalence of arteriovenous fistula maturation failure was of 45.8%. The prevalence of antiphospholipid syndrome was of 7.8 %, whereas only 10.7% of patients fulfilled only antiphospholipid laboratory criteria. The persistent positivity of antiphospholipid antibody was a risk factor for arteriovenous fistula maturation failure. In multivariate analysis, this association was independent of stenosis. Conclusions: To our knowledge, we report for the first time, a statistically significant association between arteriovenous fistula maturation failure, antiphospholipid antibody persistent positivity and antiphospholipid syndrome. This association was independent of arteriovenous stenosis. Our data suggests a potential non-stenotic and/or non-thrombotic mechanism of antiphospholipid antibody related arteriovenous fistula maturation failure in hemodialysis patients.
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