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Renal Pathology Case History by Dr Bonsib
The patient is a 19-year old white female. She was first seen by her family physician because of edema with swelling of feet and lower extremities. Two days before she received vaccinations (hepatitis B and meningococcal) in preparation for college.
She was treated with a prednisone with transient resolution of the edema. The edema recurred 1 week later and was still present at a follow up visit 1 month later. She had gained 20 pounds and a large amount of protein was detected in her urine. She was referred to a nephrologist.
Physical examination showed only significant edema.
Laboratory findings showed normal renal function and a large amount of protein in the urine. There were no other laboratory abnormalities.
She patient was treated with prednisone 60mg/day and a renal biopsy was performed.
| Figure 1. The cortex appears normal; note absence of tubulo-interstitial disease. |
Figure
2. |
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| Figure 3.
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Renal biopsy findings:
LM: The sections and special stains contain 35 glomeruli per section. No completely sclerotic glomeruli are present. There is no glomerular hypercellularity or segmental lesion, and all glomeruli have open capillary loops with normal basement membranes. There is no tubular atrophy or interstitial fibrosis, and no interstitial inflammation or edema is noted. There are focal clusters of interstitial foam cells. All arterioles and arteries are normal.
IF: The tissue contains 10 glomeruli. There is no reaction for IgG, IgA, IgM, C3, C1q, or fibrin.
EM: Ultrastructural examination of 2 glomeruli shows diffuse effacement of podocyte foot processes. The capillary loop basement membranes are uniform and of normal thickness. There is no capillary loop sclerosis, hypercellularity, or electron dense deposit. The mesangial matrix is not expanded and no electron dense deposits are noted.
Morphologic diagnosis (not final diagnosis):
Minimal change disease
Clinical Course:
May, 2006
Prednisone with resolution of edema
Edema recurred 1 week later
June, 2006
Steroid taper but edema persists
July 14, 2006
Nephrology consult
Renal biopsy performed
July 27, 2006
Cervical lymphadenopathy noted
CT scan – mediastinal lymphadenopathy identified - regarded as reactive
August 22, 2006
Repeat CT - suspicious for Hodgkin’s disease
Lymph node biopsy performed
August 29, 2006
Hodgkin’s disease, nodular sclerosing type diagnosed
Chemotherapy initiated - doxorubicin, bleomycin, vinblastine, DTIC / q2 weeks
January, 2007
Hodgkin’s disease is in remission and proteinuria completely resolved.
Final diagnoses:
Minimal change disease secondary to Hodgkin’s disease
Major points:
I. There are many situations in which minimal change disease develops. In most cases there is no identifiable cause
Causes of Minimal Change Disease
No known casue – most common
Drugs, especially nonsteroidal antiinflammatory drugs
Neoplasms, especially lymphoproliferative disorders
Mutations of podocyte and slit diaphragm proteins: nephrin, podocin, a-actinnin 4
Superimposed on another renal disease: IgA-N, SLE, HIV
Autoimmune and hypersensitivity disorders, including vaccinations
II. Vaccination has been shown to cause proteinuria with minimal change disease in a small number of cases listed below.
Vaccinations and Nephrotic Syndrome
1st Author - yr
Vaccine
Ren biopsy
Onset - Labs
Treatment
Outcome
Kikuchi 2002
Pneumococcal
MCD/AIN
<1mo Crt 1.3/10.4gm
corticosteroids
Resol 32 days
Keilstein 2000
Influenza
MCD
4d -13.2 gm/65cc
none
Resol 6 wks
Islek 2000
Hep B
MCD
8d - 2 gm
prednisone
Resol 18 days
Ozdemir 1998
Hep B
MCD
17d - NRP
corticosteroids
Resol 20 days
Macario 1995
Hep B
MCD
‘after’ 2nd dose - 8 gm
corticosteroids
Resol 14 days
Carmeli 1993
Hep B
No bx
6 wks – 2+P / 1+ H
none
Resol ‘few days’
III. Cancer is also associated with proteinuria. In patients with minimal change disease, such as our patient, Hodgkin’s disease is most often the cause.
Glomerular Diseases in Hematopoietic Malignancies
(Data from Dabbs, et. al. Am J Med 80:63-70, 1986)
Glomerular lesion
Hodgkin’s disease
NH Lymphoma
CLL
Minimal change disease
40
5
2
Amyloidosis
39
3
Focal segmental glom scl
2
1
Membranous gn
4
8
4
Membranoproliferative gn
2
8
7
Other proliferative gn
5
3
Crescentic/AGBM
6
3
Unclassified gn
2
7
IV. Successful treatment of the
Hodgkin Disease results in disappearance of protein in the urine and
the patient’s edema.
This occurred with our
patient
Minimal Change Disease in Patients with Hodgkin’s Disease
1st Author
# Pts
Hodgkin disease in remission
Protein in the urine resolved
Audard
21
100% **
Eagen
26
100%
Stephan
5
100%
Contact the Renal Pathology Department of LSUHSC-SH for more information.
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