Primary Kidney Allograft Dysfunction Due to Myeloma-Cast
Nephropathy: A Case Report
A. Perkowska-Ptasinska, M. Glyda, M. Paczkowski, and M. Durlik
ABSTRACT
Cast nephropathy is a rare event among renal transplants, usually associated with multiple
myleoma or light chain nephropathy. Herein we have reported primary graft dysfunction early
posttransplantation due to cast nephropathy, associated with thrombotic microangiopathy.
INTRODUCTION
T
HERE IS AN INCREASING number of patients with
multiple mycloma/light chain deposition disease who
have undergone renal transplantation. In the majority of
them, the monoclonal protein-associated nephropathy oc-
curs or recurs in the transplant.
1–4
Thrombotic microangi-
opathy (TMA) is a common complication among kidney
transplant patients. It may accompany an acute transplant
rejection, or a manifestation of cyclosporine, CsA or Pro-
graf toxicity.
5,6
CASE REPORT
This 55-year-old man developed cast nephropathy early posttrans-
plant. The patient had been dialyzed for 1 year due to end-stage
native kidney insufficiency of unknown reason. On August 2003, he
received a kidney allograft from a three HLA-mismatched, cadav-
eric, 57-year-old female donor, who died due to intracranial
hemorrhage. The donor and recipient were negative for HIV,
hepatitis C virus, and hepatitis B virus infections. Apart from mild
anemia, no changes in recipient peripheral blood morphology were
observed at the time of transplantation.
The initial immunosuppression consisted of CsA, azathioprine
(AZA), and prednisolone (Pred) in typical doses. On day 16 there
was conversion of AZA to mycophenolate mofetil with subsequent
withdrawal of CsA on day 21, followed by the introduction of
Rapamune. There were no clinical signs of CsA toxicity during its
administration, and the drug dosage was controlled by the trough
and C
2
blood level measurements. Three weeks after transplant the
patient was still oliguric and dialysis-dependent. The lowest post-
transplant serum creatinine concentration was 8.9 mg/dL. There
was no erythrocyturia on urinalysis. The urine protein content was
25 mg/dL. The patient displayed good blood pressure control
(140/80 mm Hg), a heart rate of 70 beats per minute. The lipid
profile analysis revealed triglyceride of 90 mg/dL and total choles-
terol of 210 mg/dL (high-density lipoprotein: 84 mg/dL, low-density
lipoprotein: 79 mg/dL). The graft biopsy revealed scattered acido-
philic, PAS-positive casts engulfed by multinucleated giant cells,
marked reduction in arterial lumine caused by the proliferative,
and focally myxoid changes within the intima, as well as fine,
acidophilic granular arteriolar wall thickenings associated with the
presence of a few scattered intrawall fragmented erythrocytes. The
immunomorphologic testing showed positive staining for light
chains lambda, and only weak staining for immunoglobulin 6 and
Clq within the casts. There were no C4d deposits in the graft. On
the basis of microscopic and immunomorphologic evaluations, we
rendered an initial diagnosis of cast nephropathy due to light chain
gammopathy in possible association with mild thrombotic micro-
angiopathy within the graft. Trepanobiopsy as well as blood and
urine immunofixation tests revealed changes consistent with mul-
tiple myeloma IIB. During the post-transplantation period INR
and activated partial thromboplastin time remained within normal
limits, as well as serum LDH and bilirubin concentration. At 6
weeks after transplantation the patient was still dialysis-dependent,
despite the urine volume of 2.5 L/d. On posttransplant day 51 the
graft was removed due to its constant dysfunction.
DISCUSSION
We do not know the reason for the native kidney insuffi-
ciency in this case. Considering the early, posttransplanta-
tion occurrence of cast nephropathy, an association be-
tween unrecognized lymphoproliferative disease and native
kidney insufficiency seems probable. The reported litera-
ture incidence of thromboembolic events among patients
with multiple myeloma is high. It has been attributed to
impaired fibrinolysis, mostly secondary to increased PAI-1
activity, the influence of monoclonal proteins with fibrin
structure, procoagulant autoantibody production, and ef-
fects of inflammatory cytokines on endothelium.
7–9
More-
over, injury to the endothelium, either by neoplastic cells or
From the Department of Transplantation Medicine and Ne-
phrology (A.P.-P., M.D.), Transplantation Institute, Warsaw Med-
ical University, Warsaw, Poland, and Department of Transplan-
tology (M.G., M.P.), District Hospital, Poznan, Poland.
Address reprint requests to Agnieszka Perkowska-Ptasinska,
Transplantation Institute, Warsaw Medical University, Nowogrodzka
59, 02-006 Warsaw, Poland. E-mail: aggape@poczta.onet.pl
© 2007 by Elsevier Inc. All rights reserved. 0041-1345/07/$–see front matter
360 Park Avenue South, New York, NY 10010-1710 doi:10.1016/j.transproceed.2007.01.089
Transplantation Proceedings, 39, 1683–1684 (2007) 1683