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Polyangiitis with granulomatosis as a paraneoplastic syndrome of B-cell lymphoma of the lacrimal gland.

Wills Sanín B, Bolivar YR, Carvajal JJ, Quintero GE, Andrade R - Case Rep Hematol (2014)

Bottom Line: The current pathophysiological hypotheses explaining the relationship between a lymphoproliferative disease and an autoimmune paraneoplastic disorder are discussed.Conclusion.Tumor-associated segmental necrotizing glomerulopathy is a very rare manifestation of glomerular diseases.

View Article: PubMed Central - PubMed

Affiliation: Departamento de Critical Care, Hospital Universitario Fundación Santa Fe de Bogotá, Calle 119 No. 7-75, Bogotá, Colombia.

ABSTRACT
Introduction. The clinical course of an autoimmune paraneoplastic syndrome parallels the natural history of the primary malignancy. In most cases, such paraneoplastic are syndromes hardly distinguishable from idiopathic autoimmune diseases. A case of polyangiitis with granulomatosis as a paraneoplastic syndrome in a patient with B-cell Lymphoma of the lacrimal gland has not yet been reported. Case Presentation. We present the case of a male patient with a B-cell Lymphoma of the lacrimal gland, who debuted with symptoms similar to rheumatoid arthritis and acute renal failure, secondary to polyangiitis with granulomatosis. The current pathophysiological hypotheses explaining the relationship between a lymphoproliferative disease and an autoimmune paraneoplastic disorder are discussed. Conclusion. Tumor-associated segmental necrotizing glomerulopathy is a very rare manifestation of glomerular diseases. Some atypical clinical features should increase the suspicion of an underlying tumor, in which case it is essential to treat the primary neoplasia, in order to control the autoimmune manifestations.

No MeSH data available.


Related in: MedlinePlus

Orbit (a) histological appearance with diffuse lymphoid infiltrate H&E stain 10x and (b) immunohistochemistry study showing uniform B-cell population CD20, 40x.
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fig2: Orbit (a) histological appearance with diffuse lymphoid infiltrate H&E stain 10x and (b) immunohistochemistry study showing uniform B-cell population CD20, 40x.

Mentions: An orbital MRI was ordered to study the ophthalmological symptoms (Figure 1), which showed changes suggestive of a chronic, granulomatous inflammation in the right lacrimal gland. Histopathological studies (Figures 2 and 3) revealed a predominantly B, lymphoid, and small-cell infiltrate. Phenotypically, the cells were CD20-, CD43-, and CD23-positive with excess of lambda stain on the B-cells; without expression of CD10 and CYCLIN D, the proliferative activity, as measured by Ki67, was estimated to be 10%. This morphological and phenotypical pattern, with a predominance of lambda expression, favored a marginal zone B-cell lymphoma of the lacrimal gland. Extension studies, including a thoracoabdominal CT and bone marrow biopsy, were negative for tumor involvement. A course of R-CVP (rituximab, cyclophosphamide, vincristine, and prednisone) chemotherapy was initiated.


Polyangiitis with granulomatosis as a paraneoplastic syndrome of B-cell lymphoma of the lacrimal gland.

Wills Sanín B, Bolivar YR, Carvajal JJ, Quintero GE, Andrade R - Case Rep Hematol (2014)

Orbit (a) histological appearance with diffuse lymphoid infiltrate H&E stain 10x and (b) immunohistochemistry study showing uniform B-cell population CD20, 40x.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4281442&req=5

fig2: Orbit (a) histological appearance with diffuse lymphoid infiltrate H&E stain 10x and (b) immunohistochemistry study showing uniform B-cell population CD20, 40x.
Mentions: An orbital MRI was ordered to study the ophthalmological symptoms (Figure 1), which showed changes suggestive of a chronic, granulomatous inflammation in the right lacrimal gland. Histopathological studies (Figures 2 and 3) revealed a predominantly B, lymphoid, and small-cell infiltrate. Phenotypically, the cells were CD20-, CD43-, and CD23-positive with excess of lambda stain on the B-cells; without expression of CD10 and CYCLIN D, the proliferative activity, as measured by Ki67, was estimated to be 10%. This morphological and phenotypical pattern, with a predominance of lambda expression, favored a marginal zone B-cell lymphoma of the lacrimal gland. Extension studies, including a thoracoabdominal CT and bone marrow biopsy, were negative for tumor involvement. A course of R-CVP (rituximab, cyclophosphamide, vincristine, and prednisone) chemotherapy was initiated.

Bottom Line: The current pathophysiological hypotheses explaining the relationship between a lymphoproliferative disease and an autoimmune paraneoplastic disorder are discussed.Conclusion.Tumor-associated segmental necrotizing glomerulopathy is a very rare manifestation of glomerular diseases.

View Article: PubMed Central - PubMed

Affiliation: Departamento de Critical Care, Hospital Universitario Fundación Santa Fe de Bogotá, Calle 119 No. 7-75, Bogotá, Colombia.

ABSTRACT
Introduction. The clinical course of an autoimmune paraneoplastic syndrome parallels the natural history of the primary malignancy. In most cases, such paraneoplastic are syndromes hardly distinguishable from idiopathic autoimmune diseases. A case of polyangiitis with granulomatosis as a paraneoplastic syndrome in a patient with B-cell Lymphoma of the lacrimal gland has not yet been reported. Case Presentation. We present the case of a male patient with a B-cell Lymphoma of the lacrimal gland, who debuted with symptoms similar to rheumatoid arthritis and acute renal failure, secondary to polyangiitis with granulomatosis. The current pathophysiological hypotheses explaining the relationship between a lymphoproliferative disease and an autoimmune paraneoplastic disorder are discussed. Conclusion. Tumor-associated segmental necrotizing glomerulopathy is a very rare manifestation of glomerular diseases. Some atypical clinical features should increase the suspicion of an underlying tumor, in which case it is essential to treat the primary neoplasia, in order to control the autoimmune manifestations.

No MeSH data available.


Related in: MedlinePlus