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2015 ; 4
(2
): 174-179
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gab.com Text
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English Wikipedia
A case of acute renal failure of multiple myeloma due to monoclonal type I
cryoglobulinemia with thrombotic microangiopathy
#MMPMID28509095
Okazaki M
; Yaomura T
; Tsuboi T
; Mizuno S
; Nakamura T
; Hasegawa T
; Katayama M
; Nakamura H
; Iida H
; Saito T
; Maruyama S
; Matsuo S
CEN Case Rep
2015[Nov]; 4
(2
): 174-179
PMID28509095
show ga
The term cryoglobulinemia (CG) is used to refer to vasculitis due to so-called
mixed cryoglobulins containing immune complexes. Although most cases of
monoclonal CG, called type I CG, are asymptomatic, purpura, skin ulcers, and
renal failure develop in some cases. Hematological disorders are the underlying
diseases in most cases, on which the therapeutic strategies available and the
prognosis of patients depends. We here report a case of a 47-year-old man who had
pain in both his ankles, with palpable purpura and epistaxis, and presented with
acute renal failure. Monoclonal immunoglobulin (Ig) G-? protein was detected and
cryoglobulin was also positive. Renal biopsy revealed emboli with a fibrillar
structure in the glomeruli and renal tubule lumina. The complication of
thrombotic microangiopathy (TMA) occurred during the course. Therefore, plasma
exchange and hemodialysis were added to methylprednisolone pulse therapy. The
treatment was successful, dissipating the purpura. However, the purpura relapsed
and renal dysfunction progressed when the administration of oral steroids was
tapered. Bone marrow biopsy was performed again, which indicated an increase in
abnormal plasma cells. The patient was finally diagnosed as multiple myeloma.
Then, bortezomib-dexamethasone therapy was initiated. This is the first case of
type I CG with monoclonal IgG complicated by TMA during the course; it provides
insight into the pathogenesis of renal dysfunction associated with type I CG.