Nephrotic syndrome in primary myelofibrosis with renal extramedullary hematopoiesis and glomerulopathy in the JAK inhibitor era
| Autor: | Giorgio Barbatelli, Emidio Nunzi, Rachele Brugnano, Franca Falzetti, Gioia Fiorucci, Rachele Del Sordo, Carla Covarelli, Angelo Sidoni |
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| Rok vydání: | 2017 |
| Předmět: |
renal failure
Pathology medicine.medical_specialty 030232 urology & nephrology Case Report myelofibrosis Extramedullary hematopoiesis urologic and male genital diseases 03 medical and health sciences 0302 clinical medicine renal biopsy Glomerulopathy medicine Myelofibrosis Myeloproliferative neoplasm Kidney medicine.diagnostic_test nephrotic syndrome business.industry Glomerulonephritis General Medicine medicine.disease medicine.anatomical_structure Nephrology 030220 oncology & carcinogenesis Renal biopsy proteinuria business Nephrotic syndrome |
| Zdroj: | Clinical Nephrology. Case Studies |
| ISSN: | 0301-0430 |
| DOI: | 10.5414/cncs109100 |
| Popis: | Primary myelofibrosis (PMF) is an uncommon form of myeloproliferative neoplasm (MPN) characterized by a proliferation of predominantly megakaryocytes and granulocytes in the bone marrow that, in fully-developed disease, is associated with reactive deposition of fibrous connective tissue, extramedullary hematopoiesis (EMH), and splenomegaly. Kidney involvement is rare and clinically presents with proteinuria, nephrotic syndrome, and renal insufficiency. Renal damage can be due to EMH and glomerulopathy. Renal EMH presents three patterns: infiltration of the interstitium with possible renal failure caused by functional damage of parenchyma and vessels, infiltration of capsule and pericapsular adipose tissue, and sclerosing mass-like lesions that can cause hydronephrosis and hydroureter with obstructive uropathy and renal failure. Glomerulopathy associated with PMF is rarely described, ranging from 1 month to 18 years from diagnosis of the neoplasm to renal biopsy. It is characterized by expansion and hypercellularity mesangial, segmental sclerosis, features of chronic thrombotic microangiopathy (TMA), and intracapillary hematopoietic cells infiltrating in absence of immune-mediated glomerulonephritis. We present a nephrotic syndrome in PMF-related glomerulopathy, associated with EMH, without renal failure, in a patient under treatment for 2 years with JAK2 inhibitor ruxolitinib. Despite treatment, the patient died 7 months after renal biopsy. Nephrologists still know very little about this topic and there is no homogeneous data about incidence, pathogenesis, and optimal treatment of this poor prognostic PMF-associated nephrotic syndrome. We focus on data in the literature in the hope of stimulating hematologists, nephrologists, pathologists to future studies about the natural history of renal involvement, useful for optimal management of this rare pathology. |
| Databáze: | OpenAIRE |
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