Ras-Associated Autoimmune Leukoproliferative Disorder Manifesting in an Adult Patient as a Lupus-Like Syndrome and Demonstrating Bone Marrow and Peripheral Blood Findings Reminiscent of Chronic Myelomonocytic Leukemia
Restricted accessResearch articleFirst published online 2026
Ras-Associated Autoimmune Leukoproliferative Disorder Manifesting in an Adult Patient as a Lupus-Like Syndrome and Demonstrating Bone Marrow and Peripheral Blood Findings Reminiscent of Chronic Myelomonocytic Leukemia
Ras-associated autoimmune leukoproliferative disorder (RALD) is a rare, clonally derived leukoproliferative disorder that often affects the pediatric population and can show a variety of systemic, autoimmune symptoms. We describe an adult RALD patient clinically presenting with a lupus-like syndrome and demonstrating bone marrow and peripheral blood findings reminiscent of chronic myelomonocytic leukemia (CMML). This challenging presentation of a rare disease highlights the difficulty in recognizing RALD in adults, a distinction that can have significant treatment implications.
CalvoKRPriceSBraylanRC, et al.JMML and RALD (Ras-associated autoimmune leukoproliferative disorder): common genetic etiology yet clinically distinct entities. Blood. 2015;125(18):2753‐2758. doi:10.1182/blood-2014-11-567917.
2.
WangWZhouYZhongL, et al.RAS-associated autoimmune leukoproliferative disease (RALD) manifested with early-onset SLE-like syndrome: a case series of RALD in Chinese children. Pediatr Rheumatol. 2019;17(1):55. doi:10.1186/s12969-019-0346-1.
3.
NevenQBoulangerCBruwierA, et al.Clinical spectrum of Ras-associated autoimmune leukoproliferative disorder (RALD). J Clin Immunol. 2021;41(1):51‐58. doi:10.1007/s10875-020-00883-7.
4.
OliveiraJBBidèreNNiemelaJE, et al.NRAS mutation causes a human autoimmune lymphoproliferative syndrome. Proc Natl Acad Sci USA. 2007;104(21):8953‐8958. doi:10.1073/pnas.0702975104.
5.
RodriguezDGLuqueGRFioraMBAndreoliVSanchezAVBasquieraAL. Does sustained monocytosis always mean CMML? A case of Ras-associated autoimmune leukoproliferative disorder. The Hematologist. 2024;21(6). doi:10.1182/hem.V21.6.202461.
6.
PriceSShawPASeitzA, et al.Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations. Blood. 2014;123(13):1989‐1999. doi:10.1182/blood-2013-10-535393.
7.
SavageSANiewischMR. Dyskeratosis congenita and related telomere biology disorders. 2009 Nov 12 [Updated 2023 Jan 19]. In: AdamMPBickSMirzaaGMPagonRAWallaceSEAmemiyaA, eds. GeneReviews(®)[Internet]. University of Washington, Seattle; 1993-2026.
8.
ShenWKerrCMPrzychozenB, et al.Impact of germline CTC1 alterations on telomere length in acquired bone marrow failure. Br J Haematol. 2019;185(5):935‐939. doi:10.1111/bjh.15862.
9.
KarczewskiKJFrancioliLCTiaoG, et al.The mutational constraint spectrum quantified from variation in 141,456 humans. Nature. 2020;581(7809):434‐443. doi:10.1038/s41586-020-2308-7.
10.
LawSMAkizukiSMorinobuAOhmuraK. A case of refractory systemic lupus erythematosus with monocytosis exhibiting somatic KRAS mutation. Inflamm Regen. 2022;42(1):10. doi:10.1186/s41232-022-00195-w.
11.
SunCZhaoLXiaJ, et al.Diffuse large B-cell lymphoma in a Ras-associated autoimmune leukoproliferative disorder (RALD). Ann Hematol. 2025;104(11):6109‐6113. doi:10.1007/s00277-025-06555-z.
12.
XieWFanGWiszniewskaJPressRDYangF. Histopathological and molecular findings in a patient with Ras-associated autoimmune leukoproliferative disorder. Hum Pathol Reports. 2022;29:300670. doi:10.1016/j.hpr.2022.300670.
13.
GiacamanABauzá AlonsoASalinas SanzJA, et al.Cutaneous involvement in an 8-year-old boy with Ras-associated autoimmune leucoproliferative disorder (RALD). Clin Exp Dermatol. 2018;43(8):913‐916. doi:10.1111/ced.13668.
14.
TranTANGrowWBChangCC. Superficial and deep cutaneous involvement by RAS-associated autoimmunne leukoproliferative disease (RALD cutis): a histologic mimicker of histiocytoid sweet syndrome. Am J Dermatopathol.2019;41(8):606‐610. doi:10.1097/DAD.0000000000001332.
15.
KhouryJDSolaryEAblaO, et al.The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: myeloid and histiocytic/dendritic neoplasms. Leukemia. 2022;36(7):1703‐1719. doi:10.1038/s41375-022-01613-1.
16.
ArberDAOraziAHasserjianRP, et al.International Consensus Classification of Myeloid Neoplasms and Acute Leukemias: integrating morphologic, clinical, and genomic data. Blood. 2022;140(11):1200‐1228. doi:10.1182/blood.2022015850.
17.
OsmanAEGRetsAPatelAB. KRAS mutations, autoimmunity and female sex in chronic myelomonocytic leukemia. Leuk Res.2024;138:107466. doi:10.1016/j.leukres.2024.107466.