Korean J Hematol.  2007 Mar;42(1):58-61. 10.5045/kjh.2007.42.1.58.

A Case of Polycythemia Vera Combined with Focal Segmental Glomerulosclerosis

Affiliations
  • 1Department of Internal Medicine, College of Medicine, Kangwon National University, Chuncheon, Korea. sysong@kangwon.ac.kr

Abstract

Polycythemia vera (PV) is a myeloproliferative disorder that results from clonal expansion of a transformed hematopoietic stem cell, and this is associated with a prominent overproduction of erythrocytes, and to a lesser extent, expansion of the granulocytic and megakaryocytic elements. Secondary polycythemia is occasionally associated with renal diseases such as renal tumors, cysts, hydronephorosis, renal transplantation, renal artery stenosis and Bartter's syndrome, and it is rarely associated with nephritic syndrome, nephrosclerosis, chronic glomerulonephritis (GN) and membranous nephropathy. Although cases of erythrocytosis with concomitant GN have occasionally been reported, there are few reports regarding PV. Focal segmental glomerulosclerosis (FSGS) is one of the most frequent forms of GN. However, its association with PV has rarely been described. We report here on one patient with concomitant PV and FSGS along with a review of the previously reported literature.

Keyword

Polycythemia vera; Glomerulonephritis; Focal segmental glomerulosclerosis

MeSH Terms

Bartter Syndrome
Erythrocytes
Glomerulonephritis
Glomerulonephritis, Membranous
Glomerulosclerosis, Focal Segmental*
Hematopoietic Stem Cells
Humans
Kidney Transplantation
Myeloproliferative Disorders
Nephrosclerosis
Polycythemia Vera*
Polycythemia*
Renal Artery Obstruction

Figure

  • Fig. 1 Bone marrow biopsy. Bone marrow biopsy section shows hypercelluar marrow with erythroid and megakaryocytic hyperplasia (H&E stain, ×200).

  • Fig. 2 Renal biopsy. Light microscopy revealed sharply demarcated segmental lesions (arrow) located in the glomerular tuft. Epithelial cells did not show any proliferation and there were no foamy endothelial cells (Masson's Trichrome stain, ×250).


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