Apolipoprotein C-II Deposition Amyloidosis: A Potential Misdiagnosis as Light Chain Amyloidosis.

Hereditary amyloidoses are rare and pose a diagnostic challenge. We report a case of hereditary amyloidosis associated with apolipoprotein C-II deposition in a 61-year-old female presenting with renal failure and nephrotic syndrome misdiagnosed as light chain amyloidosis. Renal biopsy was consistent with amyloidosis on microscopy; however, immunofluorescence was inconclusive for the type of amyloid protein. Monoclonal gammopathy evaluation revealed kappa light chain. Bone marrow biopsy revealed minimal involvement with amyloidosis with kappa monotypic plasma cells on flow cytometry. She was started on chemotherapy for light chain amyloidosis. She was referred to the Mayo clinic where laser microdissection and liquid chromatography mass spectrometry detected high levels of apolipoprotein C-II, making a definitive diagnosis. Apolipoprotein C-II is a component of very low-density lipoprotein and aggregates in lipid-free conditions to form amyloid fibrils. The identification of apolipoprotein C-II as the cause of amyloidosis cannot be solely made with routine microscopy or immunofluorescence. Further evaluation of biopsy specimens with laser microdissection and mass spectrometry and DNA sequencing of exons should be done routinely in patients with amyloidoses for definitive diagnosis. Our case highlights the importance of determining the subtype of amyloidosis that is critical for avoiding unnecessary therapy such as chemotherapy

Type 2 mixed monoclonal IgM and polyclonal IgG cryoglobulinemia can be associated with concurrent renal and intestinal thrombosis: Case report

Three variants of cryoglobulinemic glomerulopathy are well known, however, the concurrent acute enteritis secondary to a mixed polyclonal cryoglobulin associated thrombotic vasculitis is rarely reported in the literature. We report a case of a 72-year-old man with medical history of hypertension, leukocytoclastic vasculitis, positive serum cryoglobulin, hepatitis C, and membranoproliferative pattern of glomerulonephritis diagnosed 4 years ago. This patient recently presented with abdominal pain for one week. Labs showed worsening renal function, monoclonal IgM-kappa, and positive cryoglobulin in his serum, but negative hepatitis C test. The second renal biopsy showed a membranoproliferative pattern of glomerulopathy with many hyaline thrombi in the glomerular capillary loops. A mixed monoclonal IgM and polyclonal IgG cryoglobulinemic glomerulonephritis (type 2) was diagnosed. His concurrent jejunal biopsy revealed infiltration of neutrophils into glands and submucosal thrombus consistent with ischemic acute jejunitis. The submucosal thrombus of the jejunal biopsy was morphologically similar to the hyaline-thrombi found in glomerular capillary loops. Therefore, we concluded that cryoglobulin associated hyaline thrombi were the most likely etiology for both of his renal disease and acute ischemic jejunitis in this patient. This patient’s symptoms are due to simultaneous renal and intestinal thrombosis occurring in cryoglobulinemia. This concurrent thrombosis has not been well described in the literature

0261: Inflammatory response phenomena mediate by cytokines in the myocardial remodeling post myocardial infarct

IntroductionMyocardial infarction (MI) is a major cause of morbidity and mortality and is a main etiology of heart failure. The process of MI is modulated by the inflammatory response. Many studies have shown a significant inflammatory response phenomenon in pathogenesis of MI.ObjectivesThe aim of this study is to discuss the role of pro-inflammatory (IL8, TNFα) and anti-inflammatory (IL10) cytokines that are among others decried involved in the process of remodeling post-myocardial infarction.MethodsProspective, monocentric case-control study that included 82 patients diagnosed with a first episode of myocardial infarction and low LVEF and 30 healthy subjects.The quantification of IL10 was based on the technique of sandwich ELISA. The CLIA technique was used for quantification of IL8 and TNFα.All patients were evaluated by an echocardiography after revascularisation and one month after to study cardiac remodeling.ResultsThe study shows that patients after MI had high levels of IL8 (1.54±0.6 vs 0.56±0.12, p<0.001) and TNFα (1.21±0.24 vs 0.77±0.14 p<0.001) contrasting with low levels of IL10(0.05±1.23 vs 0.12±0.10, p=0.06). Significant positive correlation between IL8 and TNFα with CRP was found (r=0.543, p=0.002 and r=0.458, p=0.034 respectively). The low level of IL10 was positively correlated with the ejection fraction of the left ventricle (LVEF), r=0.679, p=0.002 and negatively correlated with the diastolic diameter of left ventricle (LV) (r=-0.345, p=0.029) and the systolic volume of the LV (r=–0.377, p=0.022).One month after MI, a significant decrease of the level of TNFα (to 0.96) was observed the in the group of patients with a LVEF amelioration (34.7±5.6% vs 37.8±6%, p=0.017)and LVESV reduction (from76.9±15.8mL to 72.5±13.6mL, p=0.0017).ConclusionAs a result, an increase in TNFα and IL8 associated with decreased IL10 after MI with low LVEF. A correlation was found between TNFα level decreased one month after MI and the LV remodeling

Rilpivirine in HIV-1-positive women initiating pregnancy: to switch or not to switch?

International audienceBackgroundSafety data about rilpivirine use during pregnancy remain scarce, and rilpivirine plasma concentrations are reduced during second/third trimesters, with a potential risk of viral breakthroughs. Thus, French guidelines recommend switching to rilpivirine-free combinations (RFCs) during pregnancy.ObjectivesTo describe the characteristics of women initiating pregnancy while on rilpivirine and to compare the outcomes for virologically suppressed subjects continuing rilpivirine until delivery versus switching to an RFC.MethodsIn the ANRS-EPF French Perinatal cohort, we included women on rilpivirine at conception in 2010–18. Pregnancy outcomes were compared between patients continuing versus interrupting rilpivirine. In women with documented viral suppression (<50 copies/mL) before 14 weeks of gestation (WG) while on rilpivirine, we compared the probability of viral rebound (≥50 copies/mL) during pregnancy between subjects continuing rilpivirine versus those switching to RFC.ResultsAmong 247 women included, 88.7% had viral suppression at the beginning of pregnancy. Overall, 184 women (74.5%) switched to an RFC (mostly PI/ritonavir-based regimens) at a median gestational age of 8.0 WG. Plasma HIV-1 RNA nearest delivery was <50 copies/mL in 95.6% of women. Among 69 women with documented viral suppression before 14 WG, the risk of viral rebound was higher when switching to RFCs than when continuing rilpivirine (20.0% versus 0.0%, P = 0.046). Delivery outcomes were similar between groups (overall birth defects, 3.8/100 live births; pregnancy losses, 2.0%; preterm deliveries, 10.6%). No HIV transmission occurred.ConclusionsIn virologically suppressed women initiating pregnancy, continuing rilpivirine was associated with better virological outcome than changing regimen. We did not observe a higher risk of adverse pregnancy outcomes

Rilpivirine in HIV-1-positive women initiating pregnancy: to switch or not to switch?

AbstractBackgroundSafety data about rilpivirine use during pregnancy remain scarce, and rilpivirine plasma concentrations are reduced during second/third trimesters, with a potential risk of viral breakthroughs. Thus, French guidelines recommend switching to rilpivirine-free combinations (RFCs) during pregnancy.ObjectivesTo describe the characteristics of women initiating pregnancy while on rilpivirine and to compare the outcomes for virologically suppressed subjects continuing rilpivirine until delivery versus switching to an RFC.MethodsIn the ANRS-EPF French Perinatal cohort, we included women on rilpivirine at conception in 2010–18. Pregnancy outcomes were compared between patients continuing versus interrupting rilpivirine. In women with documented viral suppression (&amp;lt;50 copies/mL) before 14 weeks of gestation (WG) while on rilpivirine, we compared the probability of viral rebound (≥50 copies/mL) during pregnancy between subjects continuing rilpivirine versus those switching to RFC.ResultsAmong 247 women included, 88.7% had viral suppression at the beginning of pregnancy. Overall, 184 women (74.5%) switched to an RFC (mostly PI/ritonavir-based regimens) at a median gestational age of 8.0 WG. Plasma HIV-1 RNA nearest delivery was &amp;lt;50 copies/mL in 95.6% of women. Among 69 women with documented viral suppression before 14 WG, the risk of viral rebound was higher when switching to RFCs than when continuing rilpivirine (20.0% versus 0.0%, P = 0.046). Delivery outcomes were similar between groups (overall birth defects, 3.8/100 live births; pregnancy losses, 2.0%; preterm deliveries, 10.6%). No HIV transmission occurred.ConclusionsIn virologically suppressed women initiating pregnancy, continuing rilpivirine was associated with better virological outcome than changing regimen. We did not observe a higher risk of adverse pregnancy outcomes.</jats:sec