Case Reports Open access

Emergence of acute promyelocytic leukemia in a patient with granulomatosis with polyangiitis during treatment with cyclophosphamide: a rare case report

Authors

Published
19 December 2023
Section
Case Reports
DOI
10.4081/reumatismo.2023.1593

Abstract

Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease that affects multiple organs and causes inflammation, necrosis, and vasculitis in small blood vessels. Treatment for GPA involves achieving and maintaining remission. In recent studies, cyclophosphamide-based regimens have been linked to comorbidity hazards, including an increased risk of malignancies, especially hematological ones. Acute myeloid leukemia is the main hematologic malignancy that can complicate GPA. In this context, we report the case of a middle-aged woman with GPA who developed acute promyelocytic leukemia (APL) during maintenance with cyclophosphamide. She was treated with all-trans retinoic acid at 50 mg/day and arsenic trioxide at 10 mg/day, along with steroids. This case highlights the unique emergence of APL in a GPA patient during cyclophosphamide therapy. A single case has previously been reported on the development of APL in a patient with GPA while using azathioprine monotherapy for 2 years.

Keywords

References

  1. Bacon PA. The spectrum of Wegener's granulomatosis and disease relapse. N Engl J Med 2005; 352: 330-2. DOI: https://doi.org/10.1056/NEJMp048338
  2. Polychronopoulos VS, Prakash UB, Golbin JM, Edell ES, Specks U. Airway involvement in Wegener's granulomatosis. Rheum Dis Clin North Am 2007; 33: 755-75, vi. DOI: https://doi.org/10.1016/j.rdc.2007.09.004
  3. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum 2013; 65: 1-11. DOI: https://doi.org/10.1002/art.37715
  4. Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis 2016; 75: 1583-94. Erratum in: Ann Rheum Dis 2017; 76: 1480. Correction in: Ann Rheum Dis 2022; 81: e109. DOI: https://doi.org/10.1136/annrheumdis-2016-209133
  5. Faurschou M, Mellemkjaer L, Voss A, Keller KK, Hansen IT, Baslund B. Prolonged risk of specific malignancies following cyclophosphamide therapy among patients with granulomatosis with polyangiitis. Rheumatology 2014; 54: 1345-50. DOI: https://doi.org/10.1093/rheumatology/keu372
  6. Knight A, Hjorton K, Sundström C, Höglund M, Backlin C, Smedby KE, et al. Leukemia and myelodysplastic syndrome in granulomatosis with polyangiitis: subtypes, clinical characteristics, and outcome. J Rheumatol 2015; 42: 690-4. DOI: https://doi.org/10.3899/jrheum.141104
  7. Sumichika Y, Yokose K, Sato S, Saito K, Yoshida S, Matsumoto H, et al. Development of acute promyelocytic leukemia in a patient with granulomatosis with polyangiitis: a case report. Tohoku J Exp Med 2023; 259: 107-12. DOI: https://doi.org/10.1620/tjem.2022.J098
  8. Godman GC, Churg J. Wegener's granulomatosis: pathology and review of the literature. AMA Arch Pathol 1954; 58: 533-53.
  9. Kubaisi B, Abu Samra K, Foster CS. Granulomatosis with polyangiitis (Wegener's disease): an updated review of ocular disease manifestations. Intractable Rare Dis Res 2016; 5: 61-9. DOI: https://doi.org/10.5582/irdr.2016.01014
  10. Sainz de la Maza M, Foster CS. Necrotizing scleritis after ocular surgery. A clinicopathologic study. Ophthalmology 1991; 98: 1720-6. DOI: https://doi.org/10.1016/S0161-6420(91)32062-1
  11. Shang W, Ning Y, Xu X, Li M, Guo S, Han M, et al. Incidence of cancer in ANCA-associated vasculitis: a meta-analysis of observational studies. PLoS One 2015; 10: e0126016. DOI: https://doi.org/10.1371/journal.pone.0126016
  12. Wang ZY, Chen Z. Acute promyelocytic leukemia: from highly fatal to highly curable. Blood 2008; 111: 2505-15. DOI: https://doi.org/10.1182/blood-2007-07-102798
  13. Lee SW, Park YB. Classification of antineutrophil cytoplasmic antibody-associated vasculitis. J Rheum Dis 2019; 26: 156-64. DOI: https://doi.org/10.4078/jrd.2019.26.3.156
  14. Esper GJ, Johnson JS. Update on the treatment of Wegener's granulomatosis. Bull Rheum Dis 1999; 48: 1-4.
  15. De Groot K, Rasmussen N, Bacon PA, Tervaert JW, Feighery C, Gregorini G, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum 2005; 52: 2461-9. DOI: https://doi.org/10.1002/art.21142

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