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Kidney light chain disease in patients with the acquired immunodeficiency syndrome.

Villaverde RV, Darioli V, Hirschel B, McKee TA, Lobrinus JA, Moll S - Clin Kidney J (2012)

Bottom Line: Light chain deposit disease (LCDD) is a rare condition caused by deposition of overproduced monoclonal light chains and has been frequently related to multiple myeloma or lymphocytic disorders.LCDD in association with human immunodeficiency virus (HIV) has only been described twice in the literature and is thought to result from HIV direct/indirect effects on B and T-cell populations, leading to chronic immune activation with paraprotein production.We report a renal LCDD case diagnosed at autopsy in a severely immunodepressed HIV patient and analyse renal histopathology of 18 HIV patients who had an autopsy in our department between 2000 and 2010.

View Article: PubMed Central - PubMed

Affiliation: Institute of Clinical Pathology, University Hospital, Geneva, Switzerland.

ABSTRACT
Light chain deposit disease (LCDD) is a rare condition caused by deposition of overproduced monoclonal light chains and has been frequently related to multiple myeloma or lymphocytic disorders. LCDD in association with human immunodeficiency virus (HIV) has only been described twice in the literature and is thought to result from HIV direct/indirect effects on B and T-cell populations, leading to chronic immune activation with paraprotein production. We report a renal LCDD case diagnosed at autopsy in a severely immunodepressed HIV patient and analyse renal histopathology of 18 HIV patients who had an autopsy in our department between 2000 and 2010.

No MeSH data available.


Related in: MedlinePlus

Renal microscopical examination. (A and B) (Light microscopy): Glomerular mesangial matrix expansion, Trichrome staining (original magnification ×65) and Periodic acid-Schiff staining (original magnification ×260). (C and D) (Immunofluorescence): Kappa light chain deposition within the mesangium and along the glomerular capillary loops. Lambda light chain negativity as a control (original magnification ×100). (E–G) (Electron microscopy) Granular dense osmiophilic deposits in mesangial and sub-endothelial areas (original magnification ×3400 and ×10 500 on Philips CM10).
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fig1: Renal microscopical examination. (A and B) (Light microscopy): Glomerular mesangial matrix expansion, Trichrome staining (original magnification ×65) and Periodic acid-Schiff staining (original magnification ×260). (C and D) (Immunofluorescence): Kappa light chain deposition within the mesangium and along the glomerular capillary loops. Lambda light chain negativity as a control (original magnification ×100). (E–G) (Electron microscopy) Granular dense osmiophilic deposits in mesangial and sub-endothelial areas (original magnification ×3400 and ×10 500 on Philips CM10).

Mentions: At autopsy, massive bilateral chronic pneumonia was diagnosed in the presence of P. jirovecii and Cytomegalovirus. An HIV lymphadenitis pattern B was identified. Polyclonal plasmacytosis was observed in the bone marrow. Infrequent microglial nodules compatible with an HIV encephalopathy were found in brain. Kidneys were macroscopically normal (left kidney weight: 190 g; right kidney weight: 160 g). Light microscopy showed a glomerular mesangial matrix expansion with segmental mesangial cell proliferation (Figure 1A and B). Diffuse and moderate interstitial fibrosis with tubular atrophy was also noted. No significant tubular lesions were observed. Immunofluorescence revealed kappa light chain deposition within the mesangium and along the glomerular capillary membranes (Figure 1C and D). No deposits were observed along the tubular basement membranes. Electron microscopy identified numerous dense granular osmiophilic deposits, localized in the mesangial and sub-endothelial areas (Figure 1E and F). A polymerase chain reaction (PCR) analysis performed on frozen renal tissue confirmed the presence of kappa monoclonality, establishing the diagnosis of renal kappa LCDD. It should be noted that we were not able to demonstrate kappa monoclonality in bone marrow and lymph nodes using immunohistochemistry and PCR analyses on formalin-fixed samples.


Kidney light chain disease in patients with the acquired immunodeficiency syndrome.

Villaverde RV, Darioli V, Hirschel B, McKee TA, Lobrinus JA, Moll S - Clin Kidney J (2012)

Renal microscopical examination. (A and B) (Light microscopy): Glomerular mesangial matrix expansion, Trichrome staining (original magnification ×65) and Periodic acid-Schiff staining (original magnification ×260). (C and D) (Immunofluorescence): Kappa light chain deposition within the mesangium and along the glomerular capillary loops. Lambda light chain negativity as a control (original magnification ×100). (E–G) (Electron microscopy) Granular dense osmiophilic deposits in mesangial and sub-endothelial areas (original magnification ×3400 and ×10 500 on Philips CM10).
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4400465&req=5

fig1: Renal microscopical examination. (A and B) (Light microscopy): Glomerular mesangial matrix expansion, Trichrome staining (original magnification ×65) and Periodic acid-Schiff staining (original magnification ×260). (C and D) (Immunofluorescence): Kappa light chain deposition within the mesangium and along the glomerular capillary loops. Lambda light chain negativity as a control (original magnification ×100). (E–G) (Electron microscopy) Granular dense osmiophilic deposits in mesangial and sub-endothelial areas (original magnification ×3400 and ×10 500 on Philips CM10).
Mentions: At autopsy, massive bilateral chronic pneumonia was diagnosed in the presence of P. jirovecii and Cytomegalovirus. An HIV lymphadenitis pattern B was identified. Polyclonal plasmacytosis was observed in the bone marrow. Infrequent microglial nodules compatible with an HIV encephalopathy were found in brain. Kidneys were macroscopically normal (left kidney weight: 190 g; right kidney weight: 160 g). Light microscopy showed a glomerular mesangial matrix expansion with segmental mesangial cell proliferation (Figure 1A and B). Diffuse and moderate interstitial fibrosis with tubular atrophy was also noted. No significant tubular lesions were observed. Immunofluorescence revealed kappa light chain deposition within the mesangium and along the glomerular capillary membranes (Figure 1C and D). No deposits were observed along the tubular basement membranes. Electron microscopy identified numerous dense granular osmiophilic deposits, localized in the mesangial and sub-endothelial areas (Figure 1E and F). A polymerase chain reaction (PCR) analysis performed on frozen renal tissue confirmed the presence of kappa monoclonality, establishing the diagnosis of renal kappa LCDD. It should be noted that we were not able to demonstrate kappa monoclonality in bone marrow and lymph nodes using immunohistochemistry and PCR analyses on formalin-fixed samples.

Bottom Line: Light chain deposit disease (LCDD) is a rare condition caused by deposition of overproduced monoclonal light chains and has been frequently related to multiple myeloma or lymphocytic disorders.LCDD in association with human immunodeficiency virus (HIV) has only been described twice in the literature and is thought to result from HIV direct/indirect effects on B and T-cell populations, leading to chronic immune activation with paraprotein production.We report a renal LCDD case diagnosed at autopsy in a severely immunodepressed HIV patient and analyse renal histopathology of 18 HIV patients who had an autopsy in our department between 2000 and 2010.

View Article: PubMed Central - PubMed

Affiliation: Institute of Clinical Pathology, University Hospital, Geneva, Switzerland.

ABSTRACT
Light chain deposit disease (LCDD) is a rare condition caused by deposition of overproduced monoclonal light chains and has been frequently related to multiple myeloma or lymphocytic disorders. LCDD in association with human immunodeficiency virus (HIV) has only been described twice in the literature and is thought to result from HIV direct/indirect effects on B and T-cell populations, leading to chronic immune activation with paraprotein production. We report a renal LCDD case diagnosed at autopsy in a severely immunodepressed HIV patient and analyse renal histopathology of 18 HIV patients who had an autopsy in our department between 2000 and 2010.

No MeSH data available.


Related in: MedlinePlus