No abstract

Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is among the spectrum of monoclonal protein-associated renal diseases, with only about 15 case reports in children. We report a 7-year-old boy with biopsy-proven crescentic PGNMID who progressed to end-stage renal disease within a few months of presentation. He then received a renal transplant with his grandmother as a donor. Proteinuria was detected at 27 months post-transplant and an allograft biopsy revealed a recurrent disease.

Introduction: Polyomavirus nephropathy (PVN) is now being frequently encountered in renal transplant recipients receiving highly potent immunosuppressive drugs and has emerged as an important cause of allograft loss. In this study, we tried to study the clinical and morphological features while incorporating the latest Banff 2018 classification of PVN and correlating it with graft outcomes. Materials and Methods: This was a retrospective study including ten patients with biopsy-proven PVN. The risk factors, clinical, histomorphological, and immunohistochemical features of all the patients were studied. We scored the intrarenal polyomavirus load and Banff interstitial fibrosis as described by Banff 2018 working group. Results: There were 6 male and 4 female patients and the mean age at the time of biopsy was 42.5 ± 10.8 years. All patients were on triple immunosuppression and the mean transplant duration to the time of diagnosis was 6.98 ± 4 months. The mean serum creatinine at the time of biopsy was 2.73 ± 1.12 mg/dl. A prior history of antibody-mediated rejection was present in two patients. All ten biopsies showed tubular epithelial basophilic, intranuclear inclusions suggestive of BK virus which was confirmed by positivity for SV40 antigen on immunohistochemistry (IHC). As per the Banff 2018 classification, seven biopsies were categorized as Class 2 and three were class 3. On follow-up, three patients went into graft loss, five patients had persistent graft dysfunction, and two expired. Conclusion: PVN is an important cause of renal dysfunction and premature allograft loss. Light microscopy for viral cytopathic changes aided by IHC with SV40 is essential for the diagnosis of PVN. The Banff scheme of classification is helpful in predicting the prognosis. It is important to diagnose PVN and differentiate it from rejection for appropriate management.

Bee stings usually result in mild allergic reactions; however, mass envenomation can cause severe complications such as rhabdomyolysis, hemolysis, shock, or multi-organ damage. Rhabdomyolysis can result in acute renal failure either by tubular obstruction by myoglobin casts or by direct cytotoxic injury. We present a case of a 12-year-old female child who presented with sudden onset anuria and hypertension following mass envenomation by bees. A renal biopsy was performed, the microscopic evaluation of which revealed tubular injury, with associated intratubular pigmented casts. The casts stained positive for myoglobin immunohistochemical stain, thus confirming a diagnosis of myoglobin cast nephropathy. The patient was given IV steroids and underwent seven sessions of hemodialysis, following which there was complete recovery of renal function.

Mixed phenotype acute leukaemia (MPAL) is a diverse group of leukemia of ambiguous lineage diagnosed when blasts in peripheral blood and/or bone marrow have antigens of more than one lineage or a mosaic of blasts belonging to more than one lineage. Retrospective analysis of 218 consecutive cases of acute leukaemia diagnosed by multiparametric flow cytometry (FCM) was done. MPAL cases were identified in accordance with European Group for the Immunological Classification of Leukaemias Criteria and World Health Organization 2008/2016 guidelines for lineage assignment. Nine out of 218 (4.1%) cases were classified as MPAL. Eight out of nine patients (88.8%) were male and 4/9 (44.4%) were \ 20 years of age. There were three cases of B/T and T/myeloid MPAL each. Two cases were B/myeloid MPAL and one case was chronic myeloid leukaemia (CML) in B/myeloid blast crisis. B/myeloid MPAL and CML in B/myeloid blast crisis cases were Philadelphia chromosome positive. The latter case had a complex karyotype as well. Seven cases were treated with acute lymphoblastic leukaemia treatment regimen; two of them achieved complete remission (CR). The patient with CML in B/myeloid blast crisis was treated with imatinib based regimen, attained CR, underwent allogenic bone marrow stem cell transplantation, but developed graft versus host disease. Five patients died due to complications of febrile neutropenia early in the course of treatment (62.5%). The last patient (B/T MPAL) refused therapy and was lost to follow-up. Early accurate diagnosis of MPAL requires FCM. It may be misdiagnosed if a limited panel of antibodies is used.