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Kidney Week

Abstract: PO0105

Angioimmunoblast T-Cell Lymphoma Masquerading as Type II Cryoglobulinemia and AKI: A Case Report

Session Information

Category: Acute Kidney Injury

  • 102 AKI: Clinical, Outcomes, and Trials

Authors

  • Xiangyang, Li, University of Hong Kong-Shenzhen Hospital, Shenzhen, Guangdong, China
  • He, Haiyan, University of Hong Kong-Shenzhen Hospital, Shenzhen, Guangdong, China
Introduction

Angioimmunoblastic T cell lymphoma (AITL), which belongs to the non-Hodgkin's lymphoma (NHL), is an infrequent hematological malignancy yet with variable and often atypical presentations. The presence of dysproteinemia, autoantibodies and systemic involvement in AITL have often led to a delay in diagnosis and/or misdiagnosis in practice.

Case Description

A 67-year old previously healthy housewife presented with 2-month history of intermittent joint pain affecting the right wrist and right hand, and a 3-day history bilateral lower limb edema. Her serum creatinine rose from 67 to 197 μmol/L and leg swelling appeared after a moderate fever prior to admission. The physical examinations at presentation were insignificant except for bilateral lower-leg pitting edema. Laboratory investigations after admission were notable for ascending serum creatinine levels, moderate anemia, marked hypocomplementemia with multiple autoantibodies of ANA, anti-cardiolipin-IgM and direct antiglobulin. The serum and urinary Immunofixation and serum cryoglobulinemia tests were all negative, while the serum κ to λ light chain ratio was depressed to 0.231. A renal biopsy conducted on day 9 revealed a diffuse proliferative glomerulonephritis with intracapillary pseudo-thrombi formation with orderly arranged microtubular structures of 20-35 nm in diameter in the subendothelial and mesangial area on electron microscopy. The patient developed on day 13 symmetrical finger-tip numbness and tingling with weaknesses in her hands and legs. A diagnosis of cryoglobulinemia complicated with cryoglobulinemic glomerulonephritis and polyneuropathy was made. She responded well with methylprednisolone (1500mg in divided pulse, maintained on 40 mg daily afterwards), intermittent hemodialysis, 5 alternate-day plasma exchange and 600mg rituximab (375mg/m2). 3 months later, however, generalized pruritic rash, weight loss, and significant groin lymphadenopathy emerged and progressed. An inguinal excisional lymph node biopsy performed at month 8 revealed AITL as the underlying disease.

Discussion

AITL and associated dysimmunity can give rise to multiorgan involvement, while the presence of autoantibodies, monoclonal gammopathy and cryoglobulinemia might conceal it as the underlying disorder. In various auto-immune diseases, it is advisable the clinicians take into consideration the multi-faceted lymphoma.