Perirenal fluid collections and monoclonal gammopathy

Viglietti, Denis; Sverzut, Jean-Michel; Péraldi, Marie-Noëlle

doi:10.1093/ndt/gfr433

Cited by 20 publications

(29 citation statements)

References 8 publications

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“…To date, 11 patients have been identified. [1][2][3][4][5][6] During the course of preparation of this manuscript, an additional patient was reported, 5 thus totaling five new patients since the initial six cases described in 2011. 1 It is certain that more patients with undiagnosed TEMPI syndrome exist and would benefit from a specific diagnosis, as symptomatic improvement may be achieved with a bortezomib-based regimen.…”

Section: Discussionmentioning

confidence: 99%

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Bone marrow findings of the newly described TEMPI syndrome: when erythrocytosis and plasma cell dyscrasia coexist

et al. 2015

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TEMPI syndrome (telangiectasias, elevated erythropoietin level and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting) is a recently described syndrome that, owing to erythrocytosis, may be confused with polycythemia vera. It is best classified as a type of plasma cell dyscrasia with paraneoplastic manifestations, similar to POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin abnormalities). To date, 11 patients have been identified. This is the first morphologic review of TEMPI syndrome bone marrow samples, in order to define pathologic features that may aid in the recognition of the syndrome and to identify post-therapy changes. Seven bone marrow aspirates and biopsies from three patients, including two post-treatment marrows, were examined. Patients were 36, 49, and 49 years old at time of diagnosis. In all cases, erythropoietin levels were extremely elevated at 45000 IU/l, the paraprotein was IgG kappa, JAK2 V617F was negative and vascular endothelial growth factor levels were normal. In one case, the increase in clonal plasma cells reached levels of smoldering myeloma (18%), but remaining marrows showed few monoclonal plasma cells (o5%). All pre-treatment biopsies showed erythroid hyperplasia, with mild nonspecific megakaryocytic, and erythroid cytologic atypia in one marrow. Prominent plasma cell vacuolization and reactive-appearing lymphoid aggregates were noted in one case. Findings of myeloproliferative neoplasms, including megakaryocyte clusters and fibrosis, were not identified. In conclusion, TEMPI syndrome should be considered when erythrocytosis and plasma cell dyscrasia coexist. The bone marrow findings, although nonspecific, differ significantly from polycythemia vera. Peculiar clinical and laboratorial findings of TEMPI, including elevated erythropoietin and normal vascular endothelial growth factor level, allow the diagnosis and distinction from POEMS syndrome. Significant decrease in erythropoietin level following treatment suggests a role of erythropoietin in monitoring therapeutic response.

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Section: Discussionmentioning

confidence: 99%

“…To our knowledge, 11 patients have been identified. [1][2][3][4][5][6] The mechanisms of disease and epidemiologic findings, including prevalence, are unknown.…”

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confidence: 99%

Bone marrow findings of the newly described TEMPI syndrome: when erythrocytosis and plasma cell dyscrasia coexist

et al. 2015

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“…[2][3][4][5] Our patient presented erythropoietin levels within normal range, measured from serum and perinephric collection aspirate. Ischemia from renal compression was thought to be the mechanism underlying polycythemia in our case.…”

Section: Discussionmentioning

confidence: 70%

“…1 It is sometimes associated with hypertension and rarely with polycythemia; there are only 4 reported cases. [2][3][4][5] We present a case of renal lymphangiectasia associated with both hypertension and polycythemia.…”

Section: Introductionmentioning

confidence: 99%

Renal lymphangiectasia presenting with hypertension and polycythemia

Blanc

Schmutz²,

Belzile³

et al. 2014

CUAJ

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We report a very rare case of renal lymphangiectasia, an accumulation of lymph in the renal lymphatic system secondary to obstruction. Our patient presented to his family doctor for an annual check-up which resulted with high blood pressure and polycythemia. An ultrasound was performed and revealed a large right perirenal collection. A year later, a controlled abdominal computed tomography scan showed a progression and compression of perirenal collection on the right kidney, as well as apparition of a left perirenal collection. Percutaneous drainage of both collections was unsuccessful. Laparoscopy-guided marsupialisation was performed and successful. Blood pressure lowered to normal range without medication. The patient's hemoglobin level decreased within normal range after 3 additional phlebotomies postoperatively.

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“…Literatürde sadece 5 hastada renal lenfanjiektaziye polisiteminin eşlik ettiği gösterilmiştir. [1][2][3][4][5] …”

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