Thrombotic thrombocytopenic purpura due to anti-ADAMTS13 antibodies in multiple myeloma.

CONTEXT: Thrombotic thrombocytopenic purpura (TTP) is a particularly serious form of thrombotic microangiopathy (TMA) due to the risk of multiple organ dysfunction. Several etiological factors such as infection, auto-immune disease, certain medications and cancers have been associated with TTP. CLINICAL CASES: A 74-year-old hypertensive woman with a history of thromboembolic disease was hospitalized for acute kidney injury (AKI) associated with pneumonia. Initial investigations were suggestive of Pneumocystis jirovecii infection and myeloma cast nephropathy. Several days later, the patient presented features of TTP. Von Willebrand factor-cleaving protease activity was less than 5% with a high level of IgG antibody directed against ADAMTS13. Treatment consisted of monthly 4-day cycles of dexamethasone and melphalan in combination with plasmapheresis and resulted in a favorable outcome. Three years after ceasing treatment, the patient presented no signs of hemolysis, but required chronic hemodialysis. CONCLUSION: The association of TMA, especially TTP, and multiple myeloma is exceptional. The authors report such a case that induced irreversible renal damage, but with stable clinical and laboratory parameters with a follow-up of 4 years.

Assessing urine human papillomavirus polymerase chain reaction testing as a tool for screening anal HPV infection in HIV-positive MSM.

Multiple types of human papillomavirus (HPV) are responsible for most cervical cancers but also cause anal cancers-especially in HIV-positive patients. Furthermore, men who have sex with men (MSM) are twice as likely to develop anal cancers as non-MSM. A simple screening test for HPV infection would be useful in these patients. The aim of our study was to evaluate the detection of HPV by real-time polymerase chain reaction (PCR) in urine as a marker of anal infection in MSM. The study included 52 HIV-positive MSM treated at Amiens University Hospital (Amiens, France). After obtaining informed consent, we performed an anal swab and gathered 10 mL of first-void urine. Samples were extracted and amplified in a real-time PCR. Genotypes were determined with a PapilloCheck(®) system (Greiner Bio-One, Frickenhausen, Germany). The anal test was the gold standard for calculating the characteristics of the urine test. The sensitivity of the urine test for diagnosing anal HPV infection was 15%, the specificity was 66%, the positive predictive value was 87.5%, and negative predictive value was 4.5%. The prevalence of anal HPV infection in the study population was 94%. Genotype 42 was the most common. The anal HPV viral load was significantly lower in men in a stable relationship than in single men. However, there was no statistically significant relationship between anal viral load and anal intraepithelial lesions. We conclude that urine-based HPV is a poor predictor of anal HPV infection in HIV-positive MSM.

[TREAT or not to treat: anemia in type 2 diabetes and chronic kidney disease at stages 3 and 4]. / TREAT or not to treat: anémie du patient diabétique de type 2 aux stades 3 et 4 de la maladie rénale chronique.

Anemia is associated with an increased risk of cardiovascular and renal events among patients with type 2 diabetes and chronic kidney disease (CKD). Although erythropoiesis-stimulating agents (ESA) can effectively increase hemoglobin (Hb) levels, their effect on clinical outcomes has not been demonstrated in CKD patients. The TREAT study is the first randomized, double-blind, placebo-controlled trial with the aim to evaluate the effect of a Hb level of 13 g/dL on the risk of death, cardiovascular events and progression to end-stage renal disease (ESRD) in type 2 diabetes with stage 3 to 4 CKD. Four thousand and thirty-eight patients were included. Death, cardiovascular events and progression to ESRD were not different between the two groups. Stroke occurred in 101 patients assigned to DA and 53 patients assigned to placebo (p<0.001), and red-cell transfusions were administered in 14.8% patients in the DA group and in 24.5% patients assigned to placebo (p<0.001). There was a modest improvement in patient-reported fatigue in the DA group. Studies performed in CKD patients who were not undergoing dialysis failed to show a benefit of the use of ESA to target a Hb level of 13 g/dl or more, on the risk of death, cardiovascular morbidity and progression to ESRD. Post hoc analysis of randomized studies suggest that the increase cardiovascular risk induced by targeting a high Hb level is more related to the resistance state of patients who failed to increase their Hb level under high ESA doses and by the rate of change in Hb concentration over time. After the release of the TREAT study, the recommendations of a Hb level of 10 to 12 g/dl in CKD patients seems adequate. This target needs to be tailored for each patient taking into account the comorbidity, age and physical activity.

Localized amyloidosis of the genitourinary tract: report of 5 new cases and review of the literature.

Primary localized amyloidosis of the genitourinary tract is a rare entity characterized by small pseudotumors localized in the renal pelvis, ureters, or bladder. Amyloid fibrils are derived from immunoglobulin light chains, but no systemic plasma cell proliferation is detected. The clinical and radiologic features mimic urinary tract cancer, and local treatment is indicated. The prognosis is excellent in most cases, although disease recurrence is possible. We report 5 new cases of localized amyloidosis of the urinary tract, with lambda (4/5), or kappa (1/5) chain amyloid protein, involving the bladder (5/5), and the ureter and renal pelvis (1/5), with multiple, bilateral lesions in 1 case. The presenting complaint was painless hematuria in 4 cases. All cases were of primary (AL)-type amyloidosis. All patients underwent extensive investigation, and none presented any signs of generalized amyloidosis. A favorable outcome was observed in every case. We performed a comprehensive review of the literature, and summarize the data.