Abstract
Background: Castleman disease (CD) is a rare and heterogeneous lymphoproliferative disorder with
a wide variety of clinical presentations and outcomes. Human herpesvirus-8 (HHV-8) related CD
corresponds to the most common subtype of the multicentric Castleman disease (MCD). However,
if HHV-8 is negative, POEMS (peripheral neuropathy, organomegaly, endocrinopathy, monoclonal
gammopathy, and skin changes) associated with MCD or idiopathic MCD are the cause in a
subgroup of patients. Considering the rarity of POEMS and MCD association, we herein describe a
patient with a typical presentation based on clinical, laboratory and tissue biopsy data.
Case Presentation: We report a diabetic patient who presented with asthenia, edema, skin lesions
manifested by scarring in chiropodactyls, multiple lymph node enlargement in the neck, armpits
and inguinal areas, splenomegaly, severe anemia, thrombocytopenia, and mixed polyneuropathy.
Hematuria and proteinuria were detected. The patient developed progressive renal failure
requiring dialysis. Renal biopsy showed mesangial expansion with mesangial hypercellularity, and
lymphoplasmacytoid cells focally distributed in tubules and interstitium, which were compatible
with acute tubulointerstitial nephritis. In immunofluorescence, no deposits of IgG, IgA, IgM,
C1q, C3 or fibrinogen were found, and kappa and lambda were also negative. Lymph node biopsy
revealed lymphoid tissue with follicular hyperplasia, sinusoidal and medullary infiltration of plasma
cells. Immunohistochemistry confirmed positivity for B lymphocytes, T lymphocytes, and plasma
cells in sub-capsular and para-follicular areas. The patient was diagnosed as POEMS-associated
MCD variant, and chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone
was started. The patient did not recover renal function and remained dialysis-dependent.
Conclusions: To date, the renal involvement in MCD and POEMS syndrome seems to be uncommon
as reported in few case series. Its pathophysiology is not well understood. In the spectrum of MCD,
decreased renal function may have impact in patient survival. Early diagnosis and treatment are
needed to control the systemic manifestations, and most importantly to avoid chronic organ damage