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 case of primary myelofibrosis complicated with pericardial effusion and proteinuria is described. A 66-year-old female was admitted to our hospital because of abdominal fullness and shortness of breath. On admission, hepatosplenomegaly and pericardial effusion were observed. Blood examination revealed leukoerythroblastic anemia and thrombocytosis with tear drop cells and giant platelets. Bone marrow aspiration was dry tap and its biopsy showed remarkable myelofibrosis. Urinalysis indicated severe proteinuria. Although neutrophilic alkaline phosphatase score was low, no signs of acute blastic crisis of chronic myelogenous leukemia was found. The diagnosis of an atypical type of primary myelofibrosis was obtained. Administration of MCNU was started in August 1987. Hepatosplenomegaly, pericardial effusion and proteinuria were gradually improved after the administration. The etiology of the pericardial effusion and proteinuria were not obvious, however, these facts suggest that these abnormal findings might be related to PMF itself and MCNU was effective to PNF.
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PMID:[The use of MCNU to a patient of primary myelofibrosis complicated with pericardial effusion and proteinuria]. 276 70

Three cases of amyloidosis occurring in the later course of idiopathic myelofibrosis were studied at autopsy. In these cases, the amyloid deposition was seen only in the renal glomerulus, which had caused proteinuria. Although their exact nature could not be determined by immunohistochemistry, the amyloid deposits in the three cases were permanganate resistant and exhibited the same organ distribution, indicating a similarity in their character. These cases suggest that amyloidosis might be a complication of idiopathic myelofibrosis, implying that idiopathic myelofibrosis could be a disorder underlying amyloidosis.
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PMID:Amyloidosis complicating idiopathic myelofibrosis. 357 8

Functional potential of the kidneys, principal causes of nephrolithiasis and chronic renal failure were investigated in 30 idiopathic myelofibrosis (IMF) patients by combination of different modalities, sonography, in particular. Sonography proved highly informative in diagnosis of nephroliths the formation of which is attributed to derangement of uric acid metabolism, of renal elimination, and anatomical features of the urinary system. A correlation analysis between various factors showed that in IMF hyperuricemia, hyperfiltration and proteinuria damage cortical and medullary layers of the kidneys provoking their functional defects and development of chronic renal failure.
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PMID:[Kidney involvement in idiopathic myelofibrosis]. 798 23

Secondary amyloidosis without a known cause, diagnosed antemortem in a patient with idiopathic myelofibrosis, is reported here. This is the first such case, to our knowledge. Amyloid deposits were seen in the bone marrow, renal glomeruli and jejunum. Reasons for investigating for amyloidosis were hypogammaglobulinemia, proteinuria and recurrent diarrhea.
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PMID:Secondary amyloidosis in the course of idiopathic myelofibrosis. 939 8

The myeloproliferative disorders (MPDs) are clonal disorders of the hematopoietic stem cell and classified as polycythemia vera (PV), essential thrombocythemia (ET), or agnogenic myeloid metaplasia (AMM), depending on the main hematopoietic lineage involved. Primary renal parenchymal lesions are not commonly reported in these cases. We conducted a retrospective analysis of 138 consecutive patients with MPD to determine the frequency of renal parenchymal complications. Five patients (3.6%) (two PV, two ET, one AMM) were found to have focal segmental glomerulosclerosis (FSGS) and diffuse mesangial sclerosis, presenting as proteinuria in all the cases and progressing to chronic renal failure in two cases. A possible common risk factor was a high platelet count, because abnormal platelet activation in MPD has been shown to contribute to the development of glomerulosclerosis. The pathophysiologic basis of our observations and the implications in management of MPD patients remain to be studied.
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PMID:Focal segmental glomerulosclerosis and mesangial sclerosis associated with myeloproliferative disorders. 1056 Nov 46

An 84-year-old man presented with ankle edema, significant proteinuria, and mild impairment of renal function soon after treatment was started for idiopathic myelofibrosis. Renal amyloidosis was found on biopsy. The amyloid deposit was resistant to potassium permanganate treatment and showed no immunoreactivity to immunoglobulin light chains, beta-amyloid protein, or amyloid A component. A review of the literature showed that the occurrence of amyloidosis in idiopathic myelofibrosis is very rare, and the chemical nature of the amyloid involved remains unclear.
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PMID:Amyloidosis complicating idiopathic myelofibrosis. 1058 33

Idiopathic myelofibrosis is characterized by bone marrow fibrosis, anemia, leukoerythroblastosis, and extramedullary hematopoiesis in many organs. Renal abnormalities in idiopathic myelofibrosis have been rarely described in the literature and include extramedullary hematopoiesis in the pararenal or retroperitoneal areas resulting in obstructive uropathy and hemtopoietic cell infiltration in tubulointerstitial area and urolithiasis. These lead to azotemia or acute renal failure, which may respond well to radiotherapy and adjuvant chemotherapy. To our knowledge, there has been only one case report of nephrotic syndrome associated with glomerulonephritis in a myelofibrosis patient; however, no effective treatment was described. Herein, we report the case of a patient with idiopathic myelofibrosis who initially presented with hepatomegaly, anemia, and leukoerythroblastosis. A nephrotic syndrome developed 7 years after initial diagnosis. Renal biopsy disclosed the unique pathological finding of simultaneous mesangial proliferative glomerulonephritis, renal extramedullary hematopoiesis, and gouty nephropathy. Despite treatment with busulfan, proteinuria persisted that implied irreversible glomerular injury and a terminal prognosis. We focus on the unusual pathological finding and the association between nephrotic syndrome and idiopathic myelofibrosis.
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PMID:Idiopathic myelofibrosis associated with renal extramedullary hematopoiesis and nephrotic syndrome: case report. 1564 Dec 21

Sclerosing extramedullary hematopoietic tumor (SEMHT) is a rare lesion that typically arises in patients with chronic myeloproliferative disorders. Morphologically, it exhibits atypical megakaryocytes, granulocytic precursors, and erythroid precursors set in a background of dense collagen sclerosis. Sclerosing extramedullary hematopoietic tumor may be easily mistaken for other neoplasms, such as sarcoma, carcinoma, or Hodgkin lymphoma, particularly if pertinent clinical history is not provided. Misdiagnosis may occur because of the difficulty in recognizing the megakaryocytic lineage of the atypical cells and because of the paucity of other hematopoietic elements. We report a case of a 72-year-old man with proteinuria and renal insufficiency who underwent renal biopsy to determine the etiology of the proteinuria. The kidney biopsy demonstrated an unusual tumor in which the initial morphological impression that of sclerosing liposarcoma. However, upon learning of the patient's previous history of chronic idiopathic myelofibrosis and with the aid of immunohistochemistry, the correct diagnosis of sclerosing extramedullary hematopoietic tumor was made. Sclerosing extramedullary hematopoietic tumor should be considered in the differential diagnosis when percutaneous renal biopsy or other intra-abdominal biopsy reveals a sclerotic lesion with interspersed large atypical cells, especially in a patient with a history of chronic myeloproliferative disorder.
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PMID:Sclerosing extramedullary hematopoietic tumor: emphasis on diagnosis by renal biopsy. 1930 63

A patient with idiopathic myelofibrosis with nephrotic syndrome is reported. Seven months after the initial diagnosis of myelofibrosis, the patient was presented with dyspnea, generalized edema, heavy proteinuria, massive pleural effusion, and ascites. Renal biopsy showed focal segmental glomerulosclerosis. After starting immunosuppressive therapy consisting of cyclosporine and steroids, his renal function and proteinuria improved and transfusion requirements decreased.
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PMID:Focal segmental glomerulosclerosis associated with idiopathic myelofibrosis. 2019 91

Myeloproliferative neoplasms are clonal hematopoietic stem cell disorders that can produce an undefined glomerulopathy. To better characterize the glomerular disease associated with myeloproliferative neoplasms, we evaluated features of 11 patients with myeloproliferative neoplasm-related glomerulopathy that included 8 patients with primary myelofibrosis, and 1 each with chronic myelogenous leukemia, polycythemia vera, and essential thrombocythemia. Indications for biopsy were nephrotic-range proteinuria (nephrotic syndrome in four) and chronic renal insufficiency. The mean time from diagnosis of the neoplasms to biopsy was 7.2 years. Histologically, mesangial sclerosis and hypercellularity were seen in all 11 cases, segmental sclerosis in 8, features of chronic thrombotic microangiopathy in 9, and intracapillary hematopoietic cells in 4. On follow-up, seven patients had persistent renal dysfunction and four progressed to end-stage renal disease (ESRD). Thus, glomerulopathy appears to be a late complication of myeloproliferative neoplasms, particularly primary myelofibrosis, with guarded prognosis. Greater awareness of this entity and larger studies are needed to define possible therapies.
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PMID:Myeloproliferative neoplasms cause glomerulopathy. 2191 59


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