Les glomérulopathies associées aux néoplasies myéloprolifératives

Bardy, A.; Tiple, A.; Rabant, Marion; Kémény, Jean-Louis; Karoui, Khalil El; Hermet, M.; Guettrot-Imbert, G.; Deteix, P; Bay, Jacques‐Olivier; Aumaître, O.; André, M.

doi:10.1016/j.revmed.2013.04.014

Cited by 7 publications

(2 citation statements)

References 40 publications

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“…The largest series, published in 2011, included only 11 patients (PMF n=8, ET n=1, PV n=1, chronic myeloid leukemia n=1). In this series and in anecdotal case reports, focal segmental glomerulosclerosis (FSGS) was the main pathological finding 10,11,12 . Renal pathologies in patients with chronic CMML or essential thrombocytosis (ET) are far less described 12 .…”

Section: Introductionmentioning

confidence: 70%

Kidney Involvement in Patients With Chronic Myelomonocytic Leukemia or BCR-ABL–Negative Myeloproliferative Neoplasms

Béllière

Colombat

Kounde

et al. 2021

Kidney International Reports

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Introduction The identification of specific molecular signatures and the development of new targeted drugs have changed the paradigm of onco-nephrology, now allowing a multiscale approach of kidney involvement related to hematologic malignancies relying on combined hematologic and molecular assessments. In this study, we aimed to refine the spectrum of kidney disorders associated with chronic myelomonocytic leukemia (CMML) or BCR-ABL–negative myeloproliferative neoplasms (MPNs), 2 very rare conditions scarcely described. Methods Case series. Patients with myeloid neoplasms who were referred to Toulouse University Hospital Nephrology Unit and were diagnosed with acute kidney injury (AKI), chronic kidney disease (CKD), or urine abnormalities were retrospectively included. Results Eighteen patients (males n =13, CMML n =8, essential thrombocytosis [ET] n =7, polycythemia vera [PV] n =1, and myelofibrosis n =2) developed kidney disease 7.7±2 years after the diagnosis of the malignancy. Twelve patients had AKI at presentation. Eight patients had glomerular presentation (high-range proteinuria 33%, microscopic hematuria 56%). Kidney biopsy ( n =14) showed various patterns, including pauci-immune glomerulosclerosis ( n =5), extramedullary hematopoiesis ( n =6), or tubular atrophy and interstitial fibrosis with polymorphic inflammation ( n =8). Immunostaining of CD61 confirmed the infiltration of megakaryocytes within glomeruli or interstitium in 5 of 8 patients. Other pictures of glomerulopathy were identified in 3 patients (IgA nephropathy n =2, AA amyloidosis n =1). Massive kidney infiltration by CMML was identified in 1 patient. After a mean follow-up of 24±6 months, malignancy was considered as stable in 11 patients (61%), but 22% of patients had progressed to end-stage renal failure. The remaining had persistently reduced kidney function. No correlation between the malignancy and the renal presentation and outcomes could be identified. Conclusions Kidney complications of CMML/MPN are heterogenous, and kidney biopsy may help to identify new molecular targets to prevent the development of kidney fibrosis.

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Section: Introductionmentioning

confidence: 70%

Kidney Involvement in Patients With Chronic Myelomonocytic Leukemia or BCR-ABL–Negative Myeloproliferative Neoplasms

Béllière

Colombat

Kounde

et al. 2021

Kidney International Reports

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“…Patients with MPN develop a distinct glomerular lesion, recently described as MPN-related glomerulopathy, which differs in morphology and pathology from those caused by other hematologic neoplasms [ 4 , 6 ]. MPN-related glomerulopathy is the first glomerular injury that has been associated with myeloid neoplasms [ 7 ].…”

Section: Introductionmentioning

confidence: 99%

Primary myelofibrosis associated glomerulopathy: significant improvement after therapy with ruxolitinib

Rajasekaran

Ngo²,

Abdelrahim

et al. 2015

BMC Nephrol

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BackgroundPrimary myelofibrosis (PMF) is a type of myeloproliferative neoplasm (MPN) characterized by the predominant proliferation of megakaryocytes and granulocytes in the bone marrow, leading to the deposition of fibrous tissue, and by a propensity toward extramedullary hematopoiesis. Renal involvement in PMF is rare, but kidney tissue samples from these patients reveal MPN-related glomerulopathy, a recently discovered condition, in the late stages of the disease.Case presentationWe present the first case described in the medical literature of a patient with early renal glomerular involvement in PMF/MPN. A 60-year-old man with stage 4 chronic kidney disease and a recent diagnosis of PMF (within 4 weeks of presentation at our renal division) presented with generalized body swelling, acute kidney injury, and massive nephrotic-range proteinuria. Kidney biopsy was performed to determine the etiology of the patient’s renal dysfunction and revealed early renal glomerular involvement that was histologically characteristic of MPN-related glomerulopathy. Early diagnosis and prompt medical management returned the patient’s kidney functionality to the levels seen on initial presentation at our hospital.ConclusionLarge studies with long follow-up durations are necessary to identify and categorize the risk factors for the development of MPN-related glomerulopathy, to standardize therapeutic regimens, and to determine whether aggressive management of the myelofibrosis slows the progression of kidney disease.

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“Unusual Cause Of Tophi With Renal Thrombotic Microangiopathy”

Sahu¹,

Law²,

Kumar³

et al. 2015

Indian J Hematol Blood Transfus

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Chronic neutrophilic leukemia (CNL) is a rare entity amongst myeloproliferative neoplasms (MPNs). The classical presentation of CNL is with splenomegaly, mature neutrophilic leucocytosis and hyperuricemia. We herein report a case who presented with symptoms of acute gouty arthritis. Physical examination showed typical red, tender tophi in the right hand, right foot and both pinnae suggesting an acute episode of gout. During evaluation, moderate splenomegaly, mature neutrophilia, hyperuricemia and subnephrotic range range proteinuria were noted. Bone marrow examination and kidney biopsy was done. Final diagnosis of CNL with acute gouty arthritis and chronic renal thrombotic microangiopathy (TMA) was made. Although hyperuricemia is a common finding in MPNs but presentation of our case with symptoms of acute tophi and chronic TMA is atypical. Case reportA-25-year-old gentleman with no previous comorbid illnesses presented to us in July 2010 with complaints of pain and swelling in multiple joints since the last 6 years. For past few years he used to have acute episodes of excruciating pain in the right great toe associated with redness of the overlying skin. General physical examination showed reddish, tender tophi in both pinnae, left 3rd metacarpal and right 1st metatarsal joints (Fig. 1a-c). X ray of the affected area showed erosion of the medial aspect of the right 1st metatarsal joint (Fig. 1d). Biochemical analysis showed elevated uric acid (15.6 mg/dL) with normal liver and renal function tests. Aspirate from left metacarpal swelling showed extensive deposition of needle shaped crystals confirming the diagnosis of gouty tophus (Fig. 2a, b). Abdominal examination was remarkable for splenomegaly 8 cm below the left costal margin. A complete blood count was performed that showed haemoglobin of 123 gm/L, platelet count of 320 9 10 9 /L and total white blood cell count of 26 9 10 9 /L with 83 % neutrophils, 10 % lymphocytes, 4 % basophils, 2 % eosinophils and 1 % monocytes on differential count. The neutrophils were morphologically normal and there were no immature cells in the peripheral blood. No evidence of chronic infections or occult malignancies that may be responsible for neutrophilia were identified on extensive investigation. A possibility of an MPN was considered in view of the symptom complex of hyperuricemia in addition to neutrophilic leukocytosis and splenomegaly. As depicted in the Table 1, our patient full filled all the criteria's required to make a diagnosis of chronic neutrophilic leukemia (CNL). Bone marrow examination was hypercellular with granulocytic and megakaryocytic hyperplasia without dysplasia and with a significantly high leucocytic alkaline phosphate score 318 (normal range: 40-100) (Fig. 3a-d). BCR-ABL transcripts and JAK2V617F mutation were negative by polymerase chain reaction.The acute episode of gout was managed with indomethacin and colchicine. Once the acute episode settled, he was started on allopurinol for hyperuricemia and hydroxyurea (1500 mg/day) as a cytoreducti...

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Les glomérulopathies associées aux néoplasies myéloprolifératives

Cited by 7 publications

References 40 publications

Kidney Involvement in Patients With Chronic Myelomonocytic Leukemia or BCR-ABL–Negative Myeloproliferative Neoplasms

Kidney Involvement in Patients With Chronic Myelomonocytic Leukemia or BCR-ABL–Negative Myeloproliferative Neoplasms

Primary myelofibrosis associated glomerulopathy: significant improvement after therapy with ruxolitinib

“Unusual Cause Of Tophi With Renal Thrombotic Microangiopathy”

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