Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 1-year-old boy with hemophagocytic lymphohistiocytosis exhibited proteinuria 1 month after unrelated cord blood cell transplantation, which persisted without hematuria. Laboratory study showed an increase of factor VIII-related antigen and total plasminogen activator inhibitor, suggesting endothelial injury. Histological examination of autopsy materials showed increased mesangial matrices and double-contoured basement membranes, and ultrastructurally, swelling of the endothelial cells and widening of the subendothelial space with mesangial interposition. Thrombosis was not observed at any of the sites. This case may be vasculopathy distinct from thrombotic microangiopathy (TMA) or a variant form of TMA following blood stem cell transplantation (BSCT). This vasculopathy should be considered in the differential diagnosis of proteinuria in the early stages after BSCT.
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PMID:Glomerular vasculopathy after unrelated cord blood transplantation. 1270 Sep 71

Hemophagocytic lymphohistiocytosis (HLH) is a heterogeneous disorder characterized by excessive activation and proliferation of nonmalignant histiocytes, which are commonly found in bone marrow, lymph nodes, spleen and liver in affected patients. Here, we report the presence of glomerular macrophages, including one showing erythrophagocytosis, on renal biopsy in a 25-year-old patient with clinical presentation and laboratory changes consistent with HLH. The clinical course was marked by persistent fever for 2 months, pleural and pericardial effusion, splenomegaly, lymphadenopathy, pancytopenia, cardiac arrhythmias, multiple organ dysfunction, and proteinuria, with demise after a 2-month hospitalization. Positive assay for Epstein-Barr virus (EBV), marked hyperferritinemia, hypofibrinogenemia, hypertriglyceridemia, elevated anti-nuclear antibody, proteinuria, and decreased circulating NK cells by flow cytometry were compatible with the diagnosis of HLH. We suggest that the glomerular hemophagocytic macrophages, which have not heretofore been described in the kidney of a patient with HLH, may have contributed to renal dysfunction manifest as proteinuria.
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PMID:Glomerular hemophagocytic macrophages in a patient with proteinuria and clinical and laboratory features of hemophagocytic lymphohistiocytosis (HLH). 2203 68

Hemophagocytic lymphohistiocytosis (HLH) is still an important elusive and misdiagnosed condition despite of improved knowledge. Nephrotic syndrome associated with HLH is not a common feature and has been rarely reported in hemophagocytic syndrome. We report a 27-year-old man with HLH who progressed to multi-organ failure as well as nephrotic-range proteinuria, generalized edema, and hypoalbuminemia.
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PMID:Hemophagocytic [corrected] lymphohistiocytosis associated with nephrotic syndrome and multi-organ failure. 2314 87

To investigate the clinical features of adult patients with hemophagocytic lymphohistiocytosis (HLH) and to explore possible risk factors for death, we retrospectively reviewed the medical records of 103 adult HLH patients hospitalized from 1997 to 2012. We analyzed the underlying diseases, clinical characteristics, laboratory findings, outcomes, and prognostic factors. The most common cause of HLH was hematologic malignancies (n = 49), followed by infectious diseases (n = 24) and autoimmune disorders (n = 14); 24 cases were of unknown etiology. Eight patients had a combination of underlying diseases. HLH was clinically characterized by high fever (96.1%), splenomegaly (79.6%), hepatomegaly (65.0%), lymphadenopathy (53.4%), proteinuria (31.1%), skin rash (25.2%), gastrointestinal hemorrhage (14.6%), disseminated intravascular coagulation (13.6%), increased creatinine (7.8%), and central nervous system involvement (12.6%) including altered mental status (9.7%) and cranial hemorrhage (2.9%). Laboratory abnormalities included cytopenia (99.0%), serum ferritin >500 ug/L (98.4%), liver dysfunction (98.1%), hypertriglyceridemia (88.5%), hemophagocytosis in bone marrow smear (87.4%), and hypofibrinogenemia (60.9%).In addition to the treatment they received for the underlying causes, patients received therapy for HLH consisting of corticosteroids, immunosuppressive drugs, and intravenous immunoglobulin. Twenty-six patients (25.2%) recovered after treatment, and 19 of them achieved long-term remission during follow-up. Seventy-seven patients (74.8%) died because of tumor, sepsis, multiple organ failure, or HLH-related organ hemorrhage and coagulopathy. The deceased patients were more likely to be older at disease onset, male, and to present with splenomegaly and thrombocytopenia, compared to the survivors. Treatment for the underlying diseases combined with corticosteroids, immunosuppressive agents, and immunoglobulin therapy may improve the prognosis of HLH. More attention should be paid to high-risk patients to prevent the development of serious complications associated with HLH.
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PMID:Hemophagocytic lymphohistiocytosis: clinical analysis of 103 adult patients. 2464 66

Hemophagocytic syndrome (HPS), a rare and life-threatening disease, is characterized by hyperactivation of the immune system that causes hypercytokinemia and potential multiorgan failure. Acute kidney injury is the most common kidney manifestation of HPS and is generally considered a poor prognostic factor. Glomerular involvement is uncommon and usually manifests as either podocytopathy with collapsing glomerulopathy or thrombotic microangiopathy. We report a rare case of severe histiocytic glomerulopathy in a patient with HPS who presented with acute kidney injury and proteinuria. Kidney biopsy revealed massive glomerular infiltration by macrophages resembling proliferative glomerulonephritis accompanied by intraglomerular hemophagocytosis and mild features of glomerular thrombotic microangiopathy. The patient's kidney failure and proteinuria responded rapidly to high-dose pulse methylprednisolone followed by a tapering course of oral prednisone. Our case expands the renal pathologic spectrum of HPS to include histiocyte-rich glomerular infiltration and intraglomerular hemophagocytosis. Greater awareness of this entity is needed to ensure prompt recognition and appropriate therapy.
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PMID:Hemophagocytic Syndrome With Histiocytic Glomerulopathy and Intraglomerular Hemophagocytosis. 2677 67

Hemophagocytic syndrome (HPS) is a rare condition caused by dysregulated activation of the immune system leading to infiltration of bone marrow and organs by nonmalignant macrophages that phagocytose blood cells. Primary HPS is caused by inherited immune dysregulation whereas secondary HPS is triggered by neoplastic, infectious or autoimmune diseases. Clinically, the syndrome presents with continuous high-grade fever in association with multi-organ involvement. Few data are available regarding renal manifestations of HPS. We report a 60-year-old patient with NK/T cell nasopharyngeal extranodal lymphoma who presented with acute kidney injury and nephrotic range proteinuria in association with fever and pancytopenia. A kidney biopsy was consistent with collapsing glomerulopathy. A final diagnosis of HPS was made on the basis of clinical, laboratory, and bone marrow biopsy findings in accordance with established diagnostic criteria. Steroid therapy was initiated. However, the patient failed to recover his renal function and remained hemodialysis-dependent. Key diagnostic and therapeutic challenges and strategies used to overcome those challenges are discussed.
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PMID:Collapsing glomerulopathy associated with hemophagocytic syndrome in a patient with NK/T cell lymphoma. 2904 36

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and histiocytes. Often, HLH is an acquired syndrome. We report a case of a nine month-old-boy presented with hepatosplenomegaly, severe anemia, thrombocytopenia, hypertriglyceridemia and high hyperferritinemia. These clinical features of HLH prompted a wide range of infectious and auto-immune tests to be performed. After an extensive diagnostic workup, he was referred to the immune-hematologic unit for HLH suspicion with an unknown cause. Primary HLH due to familial lymphohistiocytosis (FLH) was first evoked in front of consanguinity, probable HLH in the family, early onset, and in the absence of a causative pathology like infection or cancer. However, functional tests were normal. Atypical features like the: absence of fever, hypotonia, recurrent diarrhea since diversification, hematuria, and proteinuria suggested an inborn metabolism error with gastrointestinal involvement. Specific tests were performed to reach a final diagnosis.
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PMID:A nine-month-old-boy with Atypical Hemophagocytic Lymphohistiocytosis. 2918 Nov 34

Herein, we describe the first case of renal intravascular large B cell lymphoma in Korea occurring in a 66-year-old female. She presented with mild fever and dyspnea. On physical and laboratory evaluations, hemophagocytic lymphohistiocytosis was suspected, but the bone marrow biopsy results were unremarkable. During the work-up, massive proteinuria developed, which led to a renal biopsy. The renal architecture was relatively well-preserved, but the glomeruli were hypercellular with the infiltration of atypical, large lymphoid cells with increased nucleus-cytoplasm ratio and clumped chromatin. Similar cells were also present in the peritubular capillaries. The tumor cells exhibited membranous staining for CD20 and CD79a. After the diagnosis of intravascular large B cell lymphoma, the patient received rituximab-based chemotherapy under close follow-up.
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PMID:Renal intravascular large B cell lymphoma: the first case report in Korea and a review of the literature. 3278 26