Heparin-induced Thrombocytopenia with Anaphylactoid Reaction during Hemodialysis.

We herein report a 64-year-old man with heparin-induced thrombocytopenia accompanied by anaphylactoid reaction during hemodialysis. The patient was admitted to our hospital with acute myocardial infarction and developed acute kidney injury after percutaneous coronary intervention. When maintenance hemodialysis with heparin was initiated, the patient developed an anaphylactoid reaction with dyspnea, hypotension, nausea, and vomiting. Laboratory tests revealed thrombocytopenia. Immunoglobulin G antibodies to heparin-platelet factor 4 complexes were positive, and a functional assay showed heparin-independent platelet activation. These results provide a definitive diagnosis of heparin-induced thrombocytopenia. The onset timing supported a diagnosis of 'rapid-onset' heparin-induced thrombocytopenia.

Renal intravascular large B-cell lymphoma predominantly localized in peritubular capillaries.

We report on a 70-year-old woman with intravascular large B-cell lymphoma, in whom the renal lesion was localized in the peritubular capillaries. The patient complained of fatigue, general malaise, and unsteadiness when walking. Laboratory tests showed anemia, increased C-reactive protein, and mild renal failure, with a serum creatinine level of 1.31 mg/dL and no remarkable proteinuria or hematuria. Renal biopsy showed intravascular large B-cell lymphoma. The large atypical cells were mainly accumulated within the peritubular capillaries and no large atypical cells were found in the glomeruli. Skin and bone marrow biopsies confirmed intravascular large B-cell lymphoma. Brain magnetic resonance imaging showed multiple small infarctions in the cerebral white matter. The patient was treated with dexamethasone, methotrexate, and cytarabine followed by CHOP (combined cyclophosphamide, doxorubicin, vincristine, and prednisone) and R-CHOP (CHOP with the recombinant anti-CD20 antibody rituximab), and her renal function improved soon after the start of chemotherapy.

Hemophagocytic syndrome with acute kidney injury accompanied by erythrophagocytic macrophages in the tubular lumen.

Hemophagocytic syndrome (HPS) is a life-threatening syndrome involving excessive immune activation. It is often accompanied by renal involvement known as acute kidney injury (AKI), which is a poor prognostic factor of HPS. Thus, early diagnosis and treatment are very important. However, it is rarely identified in renal biopsy specimens, and its major manifestation is acute tubular necrosis. We report a rare case of erythrophagocytic macrophage presence in the tubular lumen of a patient with HPS-associated AKI. A kidney biopsy showed acute tubular necrosis, interstitial massive macrophage infiltration, and phagocytic macrophage casts without glomerular change. Some arteriolar vascular smooth muscle cells showed vacuolization because they were positive for α-smooth muscle actin. The patient's renal function improved after methylprednisolone pulse therapy followed by oral prednisolone after a month. Our case presents a new pathologic pattern of HPS. Careful urinalysis could suggest renal involvement with HPS. Having knowledge of this pathologic pattern of HPS is important to recognize the disease and to treat it appropriately.

A case of primary (AL) amyloidosis with predominantly vascular amyloid deposition in the kidney.

We report a 70-year-old man with primary (AL) amyloidosis with predominantly vascular deposition of amyloid diagnosed by renal biopsy, who was successfully treated using two chemotherapy regimens. There was rapid elevation of the serum creatinine level without remarkable proteinuria or hematuria. Renal histological examination showed some thickened arterial walls with amyloid fibril accumulation, and only a small amount of amyloid deposition in the glomeruli. Immunohistochemical examination was positive for anti-kappa staining. Serum immunoelectrophoresis and immunofixation testing did not show monoclonal proteins, and urine immunoelectrophoresis did not show Bence-Jones proteins. Serum free light chain (FLC) analysis showed that the serum FLC level and FLC kappa/lambda ratio were abnormally high for his renal function. He received two courses of VAD (vincristine, doxorubicin, and dexamethasone), followed by BD (bortezomib and dexamethasone), resulting in a hematologic partial response. Renal amyloidosis with vascular-limited amyloid deposition has few urinary findings. Early diagnosis of this condition is challenging, because kidney biopsies are not usually performed in patients without significant urinary findings. We suggest several currently available methods of achieving earlier detection of this condition.

Clearance of oxidized erythrocytes by macrophages: involvement of caspases in the generation of clearance signal at band 3 glycoprotein.

Human erythrocytes exposed to appropriate concentrations of H(2)O(2) for 1h became susceptible to the binding and phagocytosis by macrophages. The binding was inhibited by anti-band 3 serum and prevented by pretreatment of erythrocytes with a polylactosamine-cleaving enzyme endo-beta-galactosidase, indicating that polylactosaminyl sugar chains of band 3 are recognized by macrophages. The macrophage receptor involved was suggested to be nucleolin, a recently identified macrophage surface protein recognizing sialylpolylactosaminyl-chain clusters on early apoptotic cells, because anti-nucleolin antibody and a soluble form of recombinant nucleolin blocked the recognition. Treatment of erythrocytes with caspase inhibitors Z-VAD-fmk or Z-DQMD-fmk (caspase 3 selective) before the oxidation resulted in lowered binding of the oxidized erythrocytes to macrophages, suggesting that actions of caspases, particularly those of caspase 3, are prerequisite for the membrane changes leading to band 3 aggregation. Moreover, the cytosolic caspase 3 was found to be activated by H(2)O(2), and the extent of the activation correlated well with the susceptibility of the oxidized erythrocytes to the macrophage recognition. These results suggest that oxidative stress renders the erythrocytes susceptible to clearance by macrophages through activation of caspases leading to band 3 aggregation.

Supplementation with Alfacalcidol increases protein intake and serum albumin concentration in patients undergoing hemodialysis with hpoalbumineamia.

BACKGROUND: We have reported that vitamin D deficiency may be implicated in the pathogenesis of hypoalbuminemia observed in patients with end-stage renal disease, but the mechanism remains to be clarified. The aim of the present study was to determine whether supplementation with alfacalcidol might increase protein intake in hemodialyzed patients with hypoalbuminemia. METHODS: Twelve patients with hypoalbuminemia under 3.5 g/dl undergoing maintenance hemodialysis and not taking active forms of vitamin D were orally supplemented with 0.5 microg of alfacalcidol daily for 8 weeks. Normalized protein catabolic rate (nPCR), an index of protein intake, and serum concentrations of albumin, interleukin-6 (IL-6), IL-1beta, and soluble tumor necrosis factor-alpha receptor-II (sTNFR-II), an index of tumor necrosis factor-alpha activity, were determined before and after supplementation with alfacalcidol. RESULTS: Supplementation with alfacalcidol increased nPCR from 0.96 +/- 0.20 to 1.16 +/- 0.15 g/kg/day (p < 0.005), thereby increasing serum albumin concentration from a baseline of 3.13 +/- 0.35 to 3.32 +/- 0.29 g/dl (p < 0.05). The baseline serum concentrations of sTNFR-II and IL-6 were markedly elevated, whereas those of IL-1beta were under the detection limit. Supplementation with alfacalcidol significantly decreased serum concentration of sTNFR-II from 23.8 +/- 4.38 to 19.7 +/- 3.93 ng/ml (p < 0.001) but did not alter serum IL-6 concentration. CONCLUSION: Supplementation with alfacalcidol can increase protein intake and serum albumin concentration in hemodialyzed patients with hypoalbuminemia, probably through the suppressed tumor necrosis factor activity.

[Legionella pneumonia: report of 25 community-acquired cases].

We assessed clinical, laboratory and radiologic findings in 25 patients (21 men and 4 women; mean age 60.1 years) with Legionella pneumonia. The diagnosis was confirmed from the isolation of Legionella pneumophila in respiratory samples(nine cases), or by a fourfold increase or decrease in antibody titers in the paired serum samples (16 cases). Twelve patients were smokers, 5 patients were chronic alcoholics and 18 patients had underlying diseases. Regarding severity, the illnesses were mild (three patients), moderate (seven patients) and severe (fifteen patients). The following presenting symptoms and findings that characterize Legionella pneumonia were seen: a temperature of more than 40 degrees C (six patients), disorientation or gate disturbances(ten patients), diarrhea (eight patients), relative bradycardia(eight patients), hyponatremia(five patients), and a high creatinine phosphokinase suggesting rhabdomyolysis(five patients). Rapid progression of infiltrates on chest radiographs before appropriate antibiotic therapy was observed in seven of the twelve patients whose previous chest films were available. Only one patient, who was in endotoxin shock on admission, died. Suspected sources of infection for 13 patients were a hot spring and a public bath. We conclude that a detailed analysis of the above clinical features makes possible the diagnosis of Legionella pneumonia.