Abstracts Clinical Lymphoma, Myeloma & Leukemia September 2023 S394 MPN-409 Associations of JAK2 Mutant Allele Burden With Occurrence of CKD (Chronic Kidney Disease) and Dynamics of Kidney Function Over Time in Patients With Chronic Myeloproliferative Neoplasms Marko Lucijanic MD, PhD1, Petra Veic MD2, Ivona Aric Mag. Biol. Mol.3, Katarina Marija Tupek Ing. Med. Lab.3, Ena Soric MD1, Anica Sabljic MD1, Josipa Vlasac Glasnovic MD1, Tajana Stoos-Veic MD, PhD4, Ivan Krecak MD, PhD5,6, Rajko Kusec Md, PhD1,2,3 1Hematology Department, University Hospital Dubrava, Zagreb, Croatia. 2Internal Medicine Department, School of Medicine University of Zagreb, Zagreb, Croatia. 3Clinical Department of Laboratory Diagnostics, University Hospital Dubrava, Zagreb, Croatia. 4Pathology and Cytology Department, University Hospital Dubrava, Zagreb, Croatia. 5Internal Medicine Department, General Hospital Sibenik-Knin County, Sibenik, Croatia. 6University of Rijeka, Rijeka, Croatia Context: CKD is common in patients with chronic myeloproliferative neoplasms (MPNs). The two diseases may be causally related through development of MPN-related glomerulopathy. Objective: To evaluate associations of JAK2V617F mutant-allele-burden (MAB) with occurrence of CKD and dynamics of kidney function over time. Design: Retrospective cohort study Setting: Tertiary hematology referral center Patients or Other Participants: 230 patients with JAK2-V617F-mutated MPN (98 polycythemia vera, 94 essential thrombocythemia, 20 primary myelofibrosis, 18 other) were evaluated from 2006 to 2022. Interventions: MAB was determined using the quantitative real-time polymerase chain reaction (Applied Biosystems, California, USA). Main Outcome Measures: Occurrence of CKD defined by CKDEPI formula as estimated glomerular filtration rate <60 ml/min/1.73 m2 present for >3 months. Increase or decrease in serum-creatinine values at 6- and 12-months in comparison to baseline. Results: Median age was 67 years; 50.2% females; median MAB, 26.3%. A total of 24.9% patients had CKD, which did not significantly differ between MPN subsets (P=0.140). Patients presenting with higher MAB stratified at the median had a higher frequency of CKD at baseline (32.6% vs 16.7%, P=0.012). In the multivariate logisticregression model adjusted for clinically meaningful variables higher MAB stratified at the median remained significantly associated with CKD (OR 2.81, P=0.024) independently of older age (OR 1.11, P<0.001) and arterial hypertension (OR 2.96, P=0.015). Patients with higher MAB rather than lower significantly differed regarding the dynamics of kidney function at 6-months (mean 2% worsening vs 7% improvement, P=0.032) and 12-months (mean 11% worsening vs 8% improvement, P=0.007). In the multivariate logistic-regression model adjusted for clinically meaningful variables higher MAB remained significantly associated with worsening of kidney function at 6-months (OR 3.37, P=0.038) independently of older age (OR 1.05, P=0.024) and ACE inhibitor use (OR 0.21, P=0.024). In the similar model evaluating kidney-function dynamics at 12 months higher MAB remained the only significantly associated factor (OR 9.79, P=0.026). Conclusions: MPN patients with higher MAB seem to have a higher occurrence of concomitant CKD and demonstrate unfavorable dynamics of kidney function over time. Although causal relationship cannot be inferred due to limitations of the study design, our observations support the view that biology of MPN and CKD might be intertwined. Keywords: MPN, JAK2, chronic kidney disease MPN-421 A Case of Rosai‑Dorfman Disease, Associated With KRAS 13C, POLE, and NDE1 Mutations, Masquerading as Chronic Myelomonocytic Leukemia: Complex Management Paradigm Ekaterina Proskuriakova MD1, Leo Shunyakov MD2, Dhan Shrestha MD1, Iulia Kovalenko MD3, Pam Khosla MD4 1Department of Internal Medicine, Mount Sinai Hospital, Chicago, IL, USA. 2Department of Oncology/Hematology, Citizens Memorial Hospital, Springfield Central Care, Bolivar, MO, USA. 3Department of Internal Medicine, UPMC, Harrisburg, PA, USA. 4Department of Internal Medicine, Division of Hematology and Oncology, Mount Sinai Hospital, Chicago, IL, USA Context: Rosai–Dorfman disease (RDD) might cause a diagnostic and treatment challenge due to its rarity, overlap, and cooccurrence with other hematologic neoplasms. Background: RDD is a rare myeloproliferative disorder of histiocytes with an incidence of 1:200,000, with around 100 new cases diagnosed annually in the United States. Initial management includes steroids and rituximab. Lenalidomide is considered second line. Here we report a patient with initial partial response to rituximab and to steroids subsequently treated in combination with lenalidomide. Case Presentation: A 57-year-old healthy man presented with progressive fatigue for 6 months and painful progressive lymphadenopathy of 1 year. He had normal, complete blood count and lactate dehydrogenase (LDH). His exam was remarkable for 2cm left cervical and 4cm right inguinal lymph nodes; diffuse rash in the trunk and upper extremities. Biopsy of the inguinal lymph node showed sinus histiocytosis suggestive for RDD. Flow cytometry was unrevealing for any immunophenotypic abnormalities. Positronemission tomography showed hypermetabolic lymphadenopathy above and below diaphragm, hypermetabolic bone marrow. The patient was started on rituximab with the subsequent addition of steroids. He had a partial response, with resolution of the rash and lymphadenopathy. However, restaging scans 2 years later showed worsening lymphadenopathy and splenomegaly. Lenalidomide was initiated, with complete resolution of lymphadenopathy on restaging scans after almost 2 years. However, splenomegaly persisted, and the patient developed significant pancytopenia, despite holding lenalidomide for >4 weeks. The patient had 2 episodes of transient respiratory distress, in the setting of diffuse bilateral lung infiltrates, which responded with high dose steroids within 24 hours. His peripheral blood smear showed 67% monocytes, rare, phagocytized red blood cells with ferritin of 420 and normal LDH. Bone marrow
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