Immune Thrombocytopenia as the Initial Manifestation of Pediatric Systemic Lupus Erythematosus: Case Reports
DOI:
https://doi.org/10.3889/oamjms.2022.8174Keywords:
Immune thrombocytopenia, Systemic lupus erythematosus, ANA, ChildAbstract
BACKGROUND: Immune thrombocytopenia (ITP) can precede the onset of systemic lupus erythematosus (SLE) by months to years.
CASE PRESENTATION: A 12-year-old girl weighing 46 kg came to the hospital with the complaint of 12 days-menstrual bleeding. The patient is weak, pale. Eyes, ENT, heart, lungs, abdomen: within normal limits, no petechiae. Laboratorium: Hemoglobin (Hb) 4.6 g/dL, leukocytes 12,930/uL, platelets 11,000/uL, hematocrit 15%, Diff Count: normal. Red blood cell (RBC) 1.59 million/uL, mean corpuscular volume (MCV) 94.3fL, mean corpuscular hemoglobin (MCH) 28.9pg, MCH concentration 30.7%, RDW-CV 14.6%. Corrected-reticulocytes 5.16%, Ret-He 22.6, IPF 54.17%. Peripheral blood smears normochromic, normocytic, blast not found, platelets are rare. The diagnosis is menometrorrhagia with anemia due to bleeding caused by ITP. The patient was given PRC and platelet transfusion, methylprednisolone. Three months later, the patient had another prolonged menstruation, hair loss, no petechiae, or purpura. Laboratorium: Hb 8.2 g/dL, leukocytes 7800/uL, platelets 6000/uL, RBC 1.59 million/uL, MCV 94.3fL, MCH 28.9pg, corrected reticulocytes 5.08%, Ret-He 24.6, IPF 54.5%. ANA test positive, Anti dsDNA-NcX 190.2 IU/ml. The diagnosis is SLE. During the last 16 months, the patient took 10 mg prednisone with a platelet count >150,000/uL.
CONCLUSION: In every case of ITP in a child, consider the possibility of SLE.Downloads
Metrics
Plum Analytics Artifact Widget Block
References
Yong M, Schoonen WM, Li L, Kanas G, Coalson J, Mowat F, et al. Epidemiology of paediatric immune thrombocytopenia in the general practice research database. Br J Haematol. 2010;149(6):855-64. https://doi.org/10.1111/j.1365-2141.2010.08176.x PMid:20377590 DOI: https://doi.org/10.1111/j.1365-2141.2010.08176.x
Zhu FX, Huang JY, Ye Z, Wen QQ, Wei JC. Risk of systemic lupus erythematosus in patients with idiopathic thrombocytopenic purpura: A population-based cohort study. Ann Rheum Dis. 2020;79(6):793-9. https://doi.org/10.1136/annrheumdis-2020-217013 PMid:32241798 DOI: https://doi.org/10.1136/annrheumdis-2020-217013
Neunert C, Lim W, Crowther M, Cohen A, Solberg L Jr., Crowther MA, et al. American Society of Hematology. The American society of hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood. 2011;117(16):4190-207. https://doi.org/10.1182/blood-2010-08-302984 PMid:21325604 DOI: https://doi.org/10.1182/blood-2010-08-302984
Provan D, Stasi R, Newland AC, Blanchette VS, Bolton- Maggs P, Bussel JB, et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood. 2010;115(2):168-86. https://doi.org/10.1182/blood-2009-06-225565 PMid:19846889 DOI: https://doi.org/10.1182/blood-2009-06-225565
Sandoval C, Visintainer P, Ozkaynak MF, Tugal O, Jayabose S. Clinical features and treatment outcomes of 79 infants with immune thrombocytopenic purpura. Pediatr Blood Cancer. 2004;42(1):109-12. https://doi.org/10.1002/pbc.10458 PMid:14752803 DOI: https://doi.org/10.1002/pbc.10458
Jung JY, Rum OA, MD, Kim JK, Park M. Clinical course and prognostic factors of childhood immune thrombocytopenia: Single center experience of 10 years. Korean J Pediatr. 2016;59(8):335-40. https://doi.org/10.3345/kjp.2016.59.8.335 PMid:27610182 DOI: https://doi.org/10.3345/kjp.2016.59.8.335
Makis A, Gkoutsias A, Palianopoulos T, Pappa E, Papapetrou E, Tsaousi C, Hatzimichael E, et al. Prognostic factors for immune thrombocytopenia outcome in Greek children: A retrospective single-centered analysis. Adv Hematol. 2017;2017:7878605. https://doi.org/10.1155/2017/7878605 PMid:29362564 DOI: https://doi.org/10.1155/2017/7878605
Petri M, Orbai AM, Alarcón GS, Gordon C, Merrill JT, Fortin PR, et al. Derivation and validation of the systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012;64(8):2677-86. https://doi.org/10.1002/art.34473 PMid:22553077 DOI: https://doi.org/10.1002/art.34473
Audia S, Mahevas M, Samson M, Godeau B, Bonnotte B. Pathogenesis of immune thrombocytopenia. Autoimmun Rev. 2017;16(6):620-32. https://doi.org/10.1016/j.autrev.2017.04.012 PMid:28428120 DOI: https://doi.org/10.1016/j.autrev.2017.04.012
Macchi L, Rispal P, Clofent-Sanchez G, Pellegrin JL, Nurden P, Leng B, et al. Antiplatelet antibodies in patientswithsystemiclupuserythemato susandtheprimaryantiphospholipid antibody syndrome: Their relationship with the observed thrombocytopenia. Br J Haematol 1997;98(2):336-41. https://doi.org/10.1046/j.1365-2141.1997.2243038.x PMid:9266930 DOI: https://doi.org/10.1046/j.1365-2141.1997.2243038.x
Karpatkin S, Strick N, Karpatkin MB, Siskind GW. Cumulative experience in the detection of antiplatelet antibody in 234 patients with idiopathic thrombocytopenic purpura, systemic lupus erythematosus and other clinical disorders. Am J Med. 1972;52(6):776-85. https://doi.org/10.1016/0002-9343(72)90084-8 PMid:5063961 DOI: https://doi.org/10.1016/0002-9343(72)90084-8
Michel M, Lee K, Piette JC, Fromont P, Schaeffer A, Bierling P, et al. Platelet autoantibodies and lupus-associated thrombocytopenia. Br J Haematol. 2002;119(2):354-8. https://doi.org/10.1046/j.1365-2141.2002.03817.x PMid:12406068 DOI: https://doi.org/10.1046/j.1365-2141.2002.03817.x
Ishikawa A, Okada J, Kondo H. Antiplatelet antibodies in sera from patients with systemic lupus erythematosus by immunoblot analysis. Nihon Rinsho Meneki Gakkai Kaishi. 1995;18(1):20-8. https://doi.org/10.2177/jsci.18.20 PMid:7553035 DOI: https://doi.org/10.2177/jsci.18.20
Kuwana M, Kaburaki J, Okazaki Y, Miyazaki H, Ikeda Y. Two types of autoantibody-mediated thrombocytopenia in patients with systemic lupus erythematosus. Rheumatology (Oxford). 2006;45(7):851-4. https://doi.org/10.1093/rheumatology/kel010 PMid:16418192 DOI: https://doi.org/10.1093/rheumatology/kel010
Scofield RH, Bruner GR, Kelly JA, Kilpatrick J, Bacino D, Nath SK, et al. Thrombocytopenia identifies a severe familial phenotype of systemic lupus erythematosus and reveals genetic linkages at 1q22 and 11p13. Blood. 2003;101(3):992-7. https://doi.org/10.1182/blood-2002-04-1003 PMid:12393658 DOI: https://doi.org/10.1182/blood-2002-04-1003
Fayyaz A, Igoe A, Kurien BT, Danda D, James JA, Stafford HA, et al. Haematological manifestations of lupus. Lupus Sci Med. 2015;2(1):e000078. https://doi.org/10.1136/lupus-2014-000078 PMid:25861458 DOI: https://doi.org/10.1136/lupus-2014-000078
Hakim AJ, Machin SJ, Isenberg DA. Autoimmune thrombocytopenia in primary antiphospholipid syndrome and systemic lupus erythematosus: The response to splenectomy. Semin Arthritis Rheum. 1998;28(1):20-5. https://doi.org/10.1016/s0049-0172(98)80024-3 PMid:9726332 DOI: https://doi.org/10.1016/S0049-0172(98)80024-3
Zhao H, Li S, Yang R. Thrombocytopenia in patients with systemic lupus erythematosus: Significant in the clinical implication and prognosis. Platelets. 2010;21(5):380-5. https://doi.org/10.3109/09537101003735564 PMid:20433308 DOI: https://doi.org/10.3109/09537101003735564
Hazzan R, Mukamel M, Yacobovich J, Yaniv I, Tamary H. Risk factors for future development of systemic lupus erythematosus in children with idiopathic thrombocytopenic purpura. Pediatr Blood Cancer. 2006;47(5):657-9. https://doi.org/10.1002/pbc.20970 PMid:16933242 DOI: https://doi.org/10.1002/pbc.20970
Altintas A Ozel A, Okur N, Okur N, Cil T, Pasa S, et al. Prevalence and clinical significance of elevated antinuclear antibody test in children and adult patients with idiopathic thrombocytopenic purpura. J Thromb Thrombolysis. 2007;24(2):163-8. https://doi.org/10.1007/s11239-007-0031-y PMid:17436144 DOI: https://doi.org/10.1007/s11239-007-0031-y
Dou X, Yang R. Current and emerging treatments for immune thrombocytopenia, Expert Rev Hematol. 2019;12(9):723-32. https://doi.org/10.1080/17474086.2019.1636644 PMid:31237783 DOI: https://doi.org/10.1080/17474086.2019.1636644
Despotovic JM.Emerging therapies in immune thrombocytopenia. The Hematologist. 2018;15(4). https://doi.org/10.1182/hem.V15.4.8728 DOI: https://doi.org/10.1182/hem.V15.4.8728
Zufferey A, Kapur R, Semple JW. Pathogenesis and therapeutic mechanisms in immune thrombocytopenia (ITP). J Clin Med. 2017;6(2):16. https://doi.org/10.3390/jcm6020016 PMid:28208757 DOI: https://doi.org/10.3390/jcm6020016
Liua S, Shan N. DNA methylation plays an important role in immune thrombocytopenia. Int Immunopharmacol. 2020;83:106390. https://doi.org/10.1016/j.intimp.2020.106390 PMid:32193101 DOI: https://doi.org/10.1016/j.intimp.2020.106390
Arnal C, Piette JC, Leone J, Taillan B, Hachulla E, Roudot- Thoraval F, et al. Treatment of severe immune thrombocytopenia associated with systemic lupus erythematosus: 59 cases. J Rheumatol. 2002;29(1):75-83. PMid:11824975
Kado R, McCune WJ. Treatment of primary and secondary immune thrombocytopenia. Curr Opin Rheumatol. 2019;31(3):213-22. https://doi.org/10.1097/BOR.0000000000000599 PMid:30920453 DOI: https://doi.org/10.1097/BOR.0000000000000599
Mohammadpour F, Kargar M, Hadjibabaie M. The role of hydroxychloroquine as a steroid-sparing agent in the treatment of immune thrombocytopenia: A review of the literature. J Res Pharm Pract. 2018;7(1):4-12. https://doi.org/10.4103/jrpp.JRPP_17_60 PMid:29755993
Jung JH, Soh MS, Ahn YH, Um YJ, Jung JY, Suh CH, et al. Thrombocytopenia in systemic lupus erythematosus: Clinical manifestations, treatment, and prognosis in 230 patients. Medicine (Baltimore). 2016;95(6):e2818. DOI: https://doi.org/10.1097/MD.0000000000002818
Quartuccio L, Sacco S, Franzolini N, Perin A, Ferraccioli G, de Vita S. Efficacy of cyclosporin-A in the long-term management of thrombocytopenia associated with systemic lupus erythematosus. Lupus. 2006;15(2):76-9. https://doi.org/10.1191/0961203306lu2266oa PMid:16539277 DOI: https://doi.org/10.1191/0961203306lu2266oa
Li Y, Feng X. Efficacy and safety of tacrolimus in systemic lupus erythematosus patients with refractory thrombocytopenia: A retrospective study. Lupus. 2018;27(1):60-5. https://doi.org/10.1177/0961203317711011 PMid:28566017 DOI: https://doi.org/10.1177/0961203317711011
Downloads
Published
How to Cite
Issue
Section
Categories
License
Copyright (c) 2022 Harapan Parlindungan Ringoringo (Author)
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
http://creativecommons.org/licenses/by-nc/4.0