Age: 61.0
Sex: F
Vaccine: HIBV, PPV, TD, HIBV, PPV, TD
Symptoms: Oedema, Proteinuria, Renal failure, Renal tubular disorder, Skin ulcer
Clinical narrative: Information has been received from the authors of the above entitled published literature article concerning a 61 year old female pt with a history of acute myelogenous leukaemia, induction chemotherapy with anthracycline and cytarabine and consolidation therapy with high dose cytarabine, relapsed AML and nonmyelaoablative hematopoietic stem cell transplantation (HSCT) from an unrelated donor (conditioning included fludarabine phosphate and busulfan, and graft versus host disease (GVHD) prophylaxis consisted of methotrexate, sirolimus, and tacrolimus. One year after HSCT the pt was vaccinated with a dose of Pneumococcal 23v polysaccharide vaccine. Concomitant suspect therapy included Hib conj vaccine (manufacturer unk), and diphtheria toxoid/tetanus toxoid. Her serum creatinine level at the time was 0.8mg/dl. After 5 days, the pt began to experience fatigue and progressive weight gain, and 14 days after immunization she had gained 10kg and had generalized edema. She has a serum creatinine level of 3.4 mg/gl, hypoalbuminenia of 0.28 mg/l, proteinuria of 4+, and no hematuria. Examination of urinary sediment showed fine granular casts. The pt was admitted to the hospital for a kidney biopsy and further evaluation. A Doppler renal ultrasound showed normal sized kidneys, normal vascular flow, and no hydronephrosis. A 24 hour urine collection contained 14g of urine protein. Light microscopy of the kidney biopsy showed evidence of tubular injury and a mild, but diffuse, interstitial inflammatory infiltrate consisting of mononuclear cells and scattered eosinophils. It also showed 15 glomeruli without evidence of segmental or global sclerosis, active inflammation, basement membrane changes, or thrombosis. No immune deposits or fibrin thrombi were identified by immunofluorescence and electron microscopy. Ultrastructural evaluation revealed severe injury of the glomerular visceral epithelial cells, including microvillous degeneration and diffuse foot process effacement. The fenestration's of the glomerular capillary endothelium were well preserved and there was no significant basement membrane remodeling. Minimal change disease, acute tubular injury, and mild active interstitial nephritis were diagnosed. The pt was diuresed and started on oral prednisone 60 mg day. The proteinuria and acute renal failure resolved rapidly. The urine protein creatine ratio decreased from 19.8 to 0.2 within 2 months of starting therapy. Within 3 weeks of the renal biopsy, the pt developed mildly elevated transaminases, dry eyes, and oral ulcerations. Mild chronic GVHD was diagnosed. Steroids were tapered and, ultimately, discontinued after 10 weeks. After 9 months, the pt remained in clinical remission from AML and the nephrotic syndrome. Her oral chronic GVHD responded to topical steroids, and her elevated transaminases have normalized. The authors felt that acute renal failure and minimal change nephrotic syndrome were related to therapies with Pneumococcal 23v polysaccharide vaccine, Hib vaccine unspecified, and diphtheria toxoid tetanus toxoid and commented. Several factors support vaccination as the cause of both MCD and ARF in the case presented here, symptoms began 5 days after vaccination, MCD has previously been associated with both vaccination and HSCT, and recent date indicate that the nephrotic syndrome might be more common than previously thought in nonmyelaoablative HST recipients. In the case presented here, we propose that simultaneous immunization with three different vaccines might have resulted in hyperstimulation of the recovering immune system, leading to dysregulated T lymphocyte activation and, subsequently, MCD. The fact that the pt developed chronic GVHD soon after MCD was diagnosed indicates that MCD might be an unusual manifestation of GVHD in HSCT recipients. Nevertheless, we cannot rule out the possibility that the association between vaccination and MCD is co incidental, because almost all HSCT recipients receive immunizations after successful engraftment and, to our knowledge, no other case of MCD has been reported in this context. No further information is available. A Copy of the published literature article is attached as further documentation of the pts experience.