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Dr_Seethalekeshmy_V_Hematology_Forum
1. Spectrum of monoclonal gammopathies
encountered in renal biopsies –
A tertiary care center experience from
India
Dr Seethalekshmy NV, Dr Smitha NV , Dr Bindhu MR, Dr Annie Jojo
Amrita Institute of Medical Sciences
KOCHI 41, KERALA, INDIA
2.
3. Introduction
• Monoclonal gammopathies include malignancies
(multiple myeloma,Walderstroms
macroglobulinemia) as well as premalignant entities
like MGUS .
• Spectrum of renal lesions in monoclonal
gammopathies has expanded in addition to cast
nephropathy and amyloidosis.
• Many cases of monoclonal gammopathies are
detected first on investigations of renal dysfunction
and renal biopsy play an important role in
identification of them especially with the use of
immunofluorescence for kappa and lambda light
chains in addition to the routine panel.
4. Monoclonal gammopathies and renal lesions-
AIMS, Kochi study
Aims and objectives
1) To analyse the spectrum of lesions encountered in renal biopsies with
documented monoclonal gammopathies
2) To analyse the clinical and pathological findings of these cases
Materials and methods
•Retrospective study of renal biopsy
•Period: 3years from Jan 2014 to Dec 2016
Inclusion criteria
•All renal biopsy cases in which monoclonal gammopathy was demonstrated
by IF/Free light chain assays/Electrophoresis.IgA nephropathies are excluded
as they usually shows a lambda restriction
5. Observations Renal biopsy Diagnosis
• No renal biopsy cases in 3 years:
5279
• No of cases with monoclonality
by Immunofluorescence/SPE :
123 (2.33% of all renal biopsies )
• 72 Males and 51 Females
• Age range 34 -80 yrs
• 2/3 rd
of case in 6th
and 7th
decade
6. CAST NEPHROPATHY
• 51 cases
• 33 Males,18 Females
• 44 cases with
immunofluorescence study
• 24 lambda and 20 kappa
restricted
• 2 cases with amyloidogenic
casts.
• 3 cases had light chain
deposition disease in tubular
basement membrane also
• Low urine protein on dipstick
and a dispropotionately high
24hr urine protein should
alert the clinician to look for
paraprotein in urine
Casts with lambda light chain restriction
on immunofluorescence study
Lambda kappa
Amyloidogenic casts with congored positivity
7. AMYLOIDOSOS
• 49 cases
• 24 males, 25 females
• Age range:42-76yrs
• 35 lambda ,12 kappa
and 2 IgG lamda
restricted
• Studies show lambda
light chains are
preferentially linked
with amyloidosis
• Majority presented
with nephrotic range
proteinuria.
H&E PAS
MT SILVER
Congo red
Polarisation
UV fluorescence
IF Lambda LC
8. MONOCLONAL Ig/LIGHT
CHAIN DEPOSITION
DISEASE(MIDD/LCDD)
• 12 cases
• 6 males 6 females
• Age range 34 -72yrs
• 11 LCDD and (9 kappa
and 2 lambda
restricted )
• 1 MIDD with IgG
kappa restriction
• 4 cases had associated
cast nephropathy also
H&E PAS
PAS
SILVER
KAPPA
LAMBDA
9. Proliferative Glomerulo
nephritis with monoclonality
• 10 cases
• 6male 4 female
• Age range:31 -80yrs
• 4 IgG Kappa, 1 IgG
lambda, 4 lambda and
1 kappa light chain
restricted
• 9 had MPGN pattern
• 1 with DPGN pattern
• 1 Crescentic GN
• Will miss the diagnosis
if Immunofluorescence
panel does not include
light chains.
H&E
H&E
IgG C 3 C1q
kappa Lambda
Proliferative glomerulonephritis with
IgG kappa restriction
11. Summary
• A significant proportion of monoclonal gammopathies show renal
involvement.
• Clinical presentations vary with the severity and nature of the renal
involvement.
• Diagnosis of monoclonal gammopathy was present upfront only in 1/5th
of our cases. A suspicious A/G reversal was present in only 25% cases
• Although cast nephropathy and amyloidosis are well known entities in
the spectrum of renal manifestations in plasma cell dyscrasias, newer
morphological patterns are increasingly described in this group of late.
• A routine use of kappa and light chains in the immunofluorescence
panel is important in all cases of renal biopsies to pick up these lesions
which are being encountered in many unusual morphological and
clinical scenarios.
• Entity of monoclonal gammopathy of renal significance is gaining
importance due to the need of initiation of early therapy and its definite
advantages for prevention of malignancies and increased survival
advantages in transplants