Two cases of IgA nephropathy after splenectomy: spleen as a silent culprit in the pathogenesis of IgA nephropathy

Excerpt

A 52-year-old man was admitted to the hematology clinic with purpuric rash on his lower extremities and abdominal pain. He had a 15-year history of hypertension and diabetes. Three years prior to this admission he was also diagnosed with lymphoplasmacytic lymphoma (LPL) and LPL-related autoimmune hemolytic anemia (AIH). After six cycles of the BORID regimen (Bortezomib, Rituximab, and Dexamethasone) and splenectomy, he remained in complete remission for two and a half years. Upon present admission serum creatinine level was 0.91 mg/dL and blood workup revealed autoimmune hemolytic anemia. Skin biopsy of the purpuric lesions demonstrated IgA and C3 deposition in the small vessel walls of the dermis by direct immunofluorescence (IF) examination. Pulse methylprednisolone (MP) 1 g/day for three days and two weeks of IVIG (Intravenous immunoglobulin) 2 mg/kg were administered and anemia and rash resolved. Bone marrow biopsy and positron emission tomography (PET-CT) showed no signs of lymphoplasmacytic lymphoma recurrence. Two weeks after discharge, the patient received the first dose of the BNT162b2 mRNA COVID-19 vaccine. On the fifteenth day post vaccination, he was re-admitted with a complaint of purpuric rash, similar to the previous episode. Serum creatinine level had increased to 1.74 mg/dL from 0.8 mg/dL, and abdominal ultrasonography showed no overt pathology. Urinalysis revealed 132 red blood cells/High-power field, and UPCR (Spot urine protein-to-creatinine ratio) was 3.28 g/g. He was hospitalized, and his creatinine level rose to 4.1 mg/dL on the third day of hospitalization. A renal biopsy was performed and showed 4 of 24 globally sclerotic glomeruli, and cellular crescents in 12 glomeruli. Endocapillary proliferation was also observed. Mesangial granular accumulation of IgA (+++) and C3 (+) was observed by IF (Fig. 1A, B). A biopsy of the purpuric lesions was also compatible with IgA vasculitis, similar to the previous biopsy. Pulse steroids and cyclophosphamide were started. After 3 weeks, his creatinine level decreased to 1.54 mg/dL under 1 mg/kg of methylprednisolone. He is still being followed-up and his serum creatinine level is 1.8 mg/dL after discontinuing immunosuppressives over a year ago.

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