Paraneoplastic immunoglobulin A nephropathy and associated focal segmental glomerulosclerosis in asymptomatic low volume B-cell lymphoma - a case report.

BACKGROUND: Paraneoplastic glomerulonephritis is rare in haematological malignancies and tends to manifest as minimal change disease, membranous glomerulonephritis or membranoproliferative glomerulonephritis. We present the first report of immunoglobulin A nephropathy and associated focal segmental glomerulosclerosis in a patient with asymptomatic low grade B-cell lymphoma. CASE PRESENTATION: A 53 year old gentleman presented with nephrotic range proteinuria (urine protein creatinine ratio of 662 mg/mmol) on a background of type 2 diabetes mellitus (glycosylated haemoglobin: < 6%), hypertension, obesity (body mass index: 47.6 kg/m2) and degenerative spine disease. Bone marrow biopsy diagnosed a low grade B-cell lymphoma and renal biopsy was consistent with immunoglobulin A nephropathy. Lymphoma treatment with six cycles of cyclophosphamide/ rituximab/ prednisolone led to normalisation of urinary protein excretion (urine protein creatinine ratio: 14 mg/mmol at 26 months post-chemotherapy). CONCLUSION: Paraneoplastic immunoglobulin A nephropathy can occur with a broad range of haematological malignancies regardless of stage. This case illustrates the importance of meticulous haematological system work-up for patients presenting with immunoglobulin A nephropathy. Recognition of paraneoplastic immunoglobulin A nephropathy and early diagnosis of associated malignancy can be life-saving.

A rare case of a pericardial mass: Myelosarcoma from relapsed acute monoblastic leukaemia without bone marrow involvement.

INTRODUCTION: A 67-year-old male presented with progressive exertional dyspnoea, night sweats and 25 kg of weight loss over 2 years. He was febrile, 38.0°C, hypotensive and tachycardiac. Pulsus paradoxus was present. His electrocardiogram showed electrical alternans. He had previously had acute myeloid leukaemia treated with three cycles of chemotherapy. RESULTS: The patient was found to have a 3.6 cm pericardial effusion with features consistent with tamponade and three cardiac masses (largest 10 × 9 × 5 cm) and mediastinal lymphadenopathy. He had 825 mL of pericardial fluid drained from which cytology was consistent with extramedullary leukaemia. A bone marrow aspirate and trephine was normal. CONCLUSION: The findings suggested extramedullary recurrence of leukaemia in the pericardium, without bone marrow involvement.

Outcomes after major or bidirectional ABO-mismatched allogeneic hematopoietic progenitor cell transplantation after pretransplant isoagglutinin reduction with donor-type secretor plasma with or without plasma exchange.

BACKGROUND: Major ABO mismatch in hematopoietic progenitor cell transplantation (HPCT) is associated with a range of immunohematologic consequences including progenitor cell infusion (PCI)-related hemolysis, delayed red blood cell engraftment, and pure red cell aplasia (PRCA). Although pretransplant (recipient) isoagglutinin reduction may be associated with decreased immunohematologic complications in this setting, there is no consensus with respect to strategies for isoagglutinin reduction. STUDY DESIGN AND METHODS: This observational study assessed the efficacy of a standardized pretransplant isoagglutinin reduction strategy incorporating donor-type secretor plasma infusions with or without plasma exchange to prevent PCI-associated hemolysis and PRCA in major or bidirectional ABO-mismatched peripheral blood HPCT. All major or bidirectional ABO-mismatched HPCTs performed between 1999 and 2010 were identified from an institutional database. Immunohematologic outcomes were determined retrospectively by review of individual medical records. RESULTS: In total 110 major or bidirectional ABO-mismatched HPCTs had been performed. No patient developed hemolysis after PCI. With respect to PRCA incidence, 16 patients (15%) were excluded due to early mortality and three (3%) due to incomplete data; of the remaining 91 patients, five (5%) developed PRCA. Patients with PRCA had significantly higher pretransplant isoagglutinin titers (p = 0.0001) compared to those who did not develop PRCA. CONCLUSIONS: Use of a standardized pretransplant isoagglutinin reduction strategy including donor-type secretor plasma infusions is both safe and efficient in preventing PCI-associated hemolysis and is associated with low rates of posttransplant PRCA.