Journals A-Z
All Subjects
Free Journals
sciepub.com
American Journal of Medical Case Reports
ISSN (Print): 2374-2151 ISSN (Online): 2374-216X Website: https://www.sciepub.com/journal/ajmcr Editor-in-chief: Apply for this position
Open Access
Journal Browser
Go
Issue 10, Volume 2
Article Metrics
  • 23204
    Views
  • 17786
    Saves
  • 5
    Citations
  • 40
    Likes
Export Article
American Journal of Medical Case Reports. 2014, 2(10), 206-213
DOI: 10.12691/ajmcr-2-10-3
Open AccessCase Report

A Case Report of Thrombotic Thrombocytopenic Purpura Associated with Systemic Lupus Erythematosus: Overlapping Features

Ali Taha A. Hassan1, Mahmoud Ibrahim Yousef Elbadry1 and Mohammad Adel Tawfik2,

1Department of Internal Medicine, Sohag Faculty of Medicine, Sohag University, Sohag, Egypt

2Alhelal Health Insurance Hospital, Sohag, Egypt

Pub. Date: October 12, 2014
View Full Text Full Text PDF (257 KB) Full Text ePUB(715 KB)

Cite this paper:
Ali Taha A. Hassan, Mahmoud Ibrahim Yousef Elbadry and Mohammad Adel Tawfik. A Case Report of Thrombotic Thrombocytopenic Purpura Associated with Systemic Lupus Erythematosus: Overlapping Features. American Journal of Medical Case Reports. 2014; 2(10):206-213. doi: 10.12691/ajmcr-2-10-3

Abstract

The association between thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) is rare. Herein, we present a case with overlapping features between TTP and SLE. A 17-year old girl admitted to our hospital with an initial complaint of high grade fever, persistent headache, and microangiopathic hemolytic anaemia (negative Coombs test with schistocytes), thrombocytopenia with thrombotic event with central nervous system involvement and probably renal involvement. Also, our patient fulfilled the criteria for SLE- fever, hair loss, oral ulcers, central nervous system involvement, renal involvement, positive ANA, positive dsDNA and normal C3 and C4. The patient was rescued by extensive plasma exchange started promptly after the diagnosis. After 8 months of treatment, TTP recurred, successfully managed with plasma exchange, steroids and cyclophosphamide. It was difficult to discriminate TTP from SLE. Our case presented simultaneous features of both SLE and TTP who reinforce the importance of early diagnosis of TTP by the observation of fragmented RBCs and the intensive therapy, including plasma exchange and careful follow-up, not only to ensure diagnosis and treatment of a relapsed episode of TTP, but also to ensure proper management of the patient’s physical and mental health due to its high morbidity.

Keywords:
thrombotic thrombocytopenic purpura (TTP) systemic lupus erythematosus (SLE) and plasma exchange

Creative CommonsThis work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

Figures

Figure of 1

References:

[1]  S.K.Kwok, J.H.Ju, C.S.Cho, H.Y.Kim, and S.H.Park, “Thrombotic thrombocytopenic purpura in systemic lupus erythematosus: risk factors and clinical outcome: a single centre study,” Lupus 2009: 18(1): 16-21.
 
[2]  B. L¨ ammle, J. A. Kremer Hovinga, and L. Alberio, “Thrombotic thrombocytopenic purpura,” Journal of Thrombosis and Haemostasis, 2005:3(8): 1663-1675.
 
[3]  G.A.Rock, K.H.Shumak, N.A.Buskardetal., “Comparing of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura,” The New England Journal of Medicine, 1991: 325(6): 393=397.
 
[4]  M. P. Thomas and A. Wang, “Clinical problem solving. Taken out of context,” The New England Journal of Medicine, 2008:359: 2478-2482.
 
[5]  Moschkowitz E. Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed dis- ease. Proc NY Pathol Soc; 1924: 24: 21-4.
 
[6]  Enami T, Suzuki T, Ito S, Yoshimi A, et al. Successful Treatment of Refractory Thrombotic Thrombocytopenic Purpura with Cyclosporine and Corticosteroids in a Patient with Systemic Lupus Erythematosus and Antibodies to ADAMTS13. Jap J Intern Med; 2007:46 (13): 1033-1037.
 
[7]  James N. George, Sara K. Vesely, Judith A. James. Overlapping features of thronnbotic thronnbocytopenic purpura and systemic lupus erythematosus. Southern Medical Association 2007; 512-512.
 
[8]  Brunner HI, Freedman M, Silverman ED. Close relationship between systemic lupus erythematosus and thrombotic thrombocytopenic purpura in childhood. Arthritis Rheum 1999; 42(11): 2346-55.
 
[9]  Aleem A, Al-Sugair S. Thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus. Acta Haematol 2006; 115(1-2): 68-73.
 
[10]  Binder WD, Traum AZ, Makar RS, Colvin RB. Case records of the Massachusetts General Hospital. Case 37-2010. A 16-year-old girl with confusion, anemia, and thrombocytopenia. N Engl J Med 2010; 363(24): 2352-61.
 
[11]  James N. George and Zayd L. Al-Nour. “Diagnostic and therapeutic challenges in the thrombotic thrombocytopenic purpura and hemolytic uremic syndromes”. American Society of Hematology; 2012: 604-609.
 
[12]  Rock GA, Shumak KH, Buskard NA et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med 1991:325: 393-397.
 
[13]  Lewis EJ, Hunsicker LG, Lan SP, Rohde RD, Lachin JM A controlled trial of plasmapheresis therapy in severe lupus nephritis. The Lupus Nephritis Collaborative Study Group. N Engl J Med 1992:326: 1373-1379.
 
[14]  D.R.Terrell, S.K.Vesely, J.A.K.Hovinga, B.L¨ ammle, and J.N. George, “Different disparities of gender and race among the thrombotic thrombocytopenic purpura and hemolytic-uremic syndromes,” American Journal of Hematology, 2010: 85, no. 11, pp. 844-847.
 
[15]  C.Engelfriet,M.Overbeeke,andA.vondemBorne,”Autoimmune hemolytic anemia,” Seminars in Hematology, 1992: vol. 29, no.1, pp. 3-12.
 
[16]  E. Biagi, G. Assali, F. Rossi, M. Jankovic, B. Nicolini, and A. Balduzzi, “A persistent severe autoimmune hemolytic anemia despite apparent direct antiglobulin test negativization,” Haematologica, 1999: vol. 84, no. 11, pp. 1043-1045.
 
[17]  T. Kamesaki, T. Oyamada, M. Omine, K. Ozawa, and E. Kajii, “Cut-off value of red-blood-cell-bound IgG for the diagnosis of Coombs-negative autoimmune hemolytic anemia,” American Journal of Hematology, 2009: vol. 84, no. 2, pp. 98-101.
 
[18]  H. I. Brunner,M. Freedman, and E. D. Silverman, “Close relationship between systemic lupus erythematosus and thrombotic thrombocytopenic purpura in childhood,” Arthritis and Rheumatism, 1999: vol. 42, no. 11, pp. 2346-2355.
 
[19]  R. Asherson, “The catastrophic antiphospholipid syndrome,” Journal of Rheumatology, 1992: vol. 19, no. 4, pp. 508-512.
 
[20]  L. Kerr, H. Spiera, and L. Aledort, “Acute disseminated intravascular coagulation as a complication of systemic lupus erythematosus,” New York State Journal of Medicine, 1987: vol. 87, no. 3, pp. 181-183.
 
[21]  S. Hirohata and T. Miyamoto, “Elevated levels of interleukin-6 in cerebrospinal fluid from patients with systemic lupus erythematosus and central nervous system involvement,” Arthritis and Rheumatism, 1990: vol. 33, no. 5, pp. 644-649,.
 
[22]  Y. Fujimura and M.Matsumoto, “Registry of 919 patients with thrombotic microangiopathies across Japan: database of Nara Medical University during 1998-2008,” Internal Medicine, 2010: vol. 49, no. 1, pp. 7-15.
 
[23]  P. Coppo, M. Schwarzinger, M. Buffet et al., “Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: the French TMA reference center experience,” PLoS One, 2010: vol. 5, no. 4, Article ID e10208.
 
[24]  J. L.Moake, “Von willebrand factor, ADAMTS-13, and thrombotic thrombocytopenic purpura,” Seminars in Hematology 2004: vol. 41, no. 1, pp. 4-14.
 
[25]  A.A.Shah, J.P.Higgins, and E.F.Chakravarty, “Thrombotic microangiopathic hemolytic anemia in a patient with SLE: diagnostic difficulties,” Nature Clinical Practice Rheumatology, 2007: vol. 3, no. 6, pp. 357-362.
 
[26]  Kremer Hovinga JA, Vesely SK, Terrell DR, La¨mmle B, George JN. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood. 2010; 115(8): 1500-1511.
 
[27]  De la Rubia J, Contreras E, Del Río-Garma J. Thrombotic thrombocytopenic purpura. Med Clin. 2011; 136: 534-40.
 
[28]  George JN. How I treat patients with thrombotic thrombocytopenic purpura - 2010. Blood. 2010; 116(20):4060-4069.
 
[29]  Hovinga JA, Vesely SK, Terrell DR, La ¨mmle B, George JN. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood; 2010:115(8): 1500-1511.
 
[30]  Som S, Deford CC, Kaiser ML, et al. Decreasing frequency of plasma exchange complications in patients treated for thrombotic thrombocytopenic purpura-hemolytic uremic syndrome, 1996-2011.
 
[31]  Caramazza D, Quintini G, Abbene I, Malato A, Sacullo G, Lo Coco L, et al. Relapsing or refractory idiopathic thrombotic thrombocytopenic purpura–hemolytic uremic syndrome: the role of rituximab. Transfusion. 2010; 50: 2753-60.
 
[32]  P.Cacoub, N.Limal, D.S` ene, I. Guichard, and J. C. Piette, “Rituximab for the treatment of thrombotic thrombocytopenic purpura in systemic lupus erythematosus,” Lupus, 2008: vol. 17, no. 1, pp. 69-71.
 
[33]  A. Hundae, S. Peskoe, E. Grimsley, and S. Patel, “Rituximab therapy for refractory thrombotic thrombocytopenic purpura and autoimmune-mediated thrombocytopenia in systemic lupus erythematosus,” Southern Medical Journal, 2008: vol. 101, no. 9, pp. 943-944.
 
[34]  P. Letchumanan, H. J. Ng, L. H. Lee, and J. Thumboo, “Acomparison of thrombotic thrombocytopenic purpura in an inception cohort of patients with and without systemic lupus erythematosus,” Rheumatology, 2009: vol. 48, no. 4, pp. 399-403.
 
[35]  C. Pagnoux, J. M. Korach, and L. Guillevin, “Indications for plasma exchange in systemic lupus erythematosus in 2005,” Lupus, 2005: vol. 14, no. 11, pp. 871-877.
 
[36]  C. Cerdas-Quesada, “A life-threatening case of autoimmune hemolytic anemia successfully treated by plasma-exchange,” Transfusion and Apheresis Science, 2010: vol. 42, no. 3, pp. 235-237.
 
[37]  K. Hamasaki Æ T. Mimura Æ H. Kanda Æ K. Kubo,K. Setoguchi Æ T. Satoh Æ Y. Misaki Æ K. Yamamoto,” Systemic lupus erythematosus and thrombotic thrombocytopenic purpura: a case report and literature review” Clin Rheumatol; 2003: 22: 355-358.
 
[38]  Alpert LI (1968) Thrombotic thrombocytopenic purpura and systemic lupus erythematosus. J Mount Sinai Hosp NY 35: 165-173
 
[39]  Dekker A, O’Brien ME, Cammarata RJ (1974) The associationof thrombotic thrombocytopenic purpura with systemic lupus erythematosus. Am J Med Sci 1974:267: 243-249.
 
[40]  Oen K, Petty RE, Scoroeder ML, Briggs EJ, Bishop AJ “Thrombotic thrombocytopenic purpura in a girl with systemic lupus erythematosus.” J Rheumatol 1980:7: 727-729.
 
[41]  Cecere FA, Yoshimoya S, Pope RM “Fatal thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus”. Arthritis Rheum1981: 24: 550-553.
 
[42]  Gatenby PA, Smith H, Kirwan P, Leuer CS “Systemic lupus erythematosus and thrombotic thrombocytopenic pur- pura. A case report and review of literature”. J Rheumatol 8: 504-550, 1981.
 
[43]  Musio F, Bohen EM, Yuan CM, Welch PG “Review of thrombotic thrombocytopenic purpura in the setting of systemic lupus erythematosus”. Semin Arthritis Rheum 1998:28: 1-19.
 
[44]  Becker RC, Giuliani M, Weick JK T “hrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus”. Cleveland Clin Q 1985: 52: 409-415.
 
[45]  Dixit R, Krieg AM, Atkinson JP “Thrombotic thrombocytopenic purpura developing during pregnancy in a C2-deficient patient with a history of systemic lupus erythematosus”. Arthritis Rheum 1985: 28: 341-344.
 
[46]  Gelfand J, Truong L, Stern L, Pirani CL, Appel GB “Thrombotic thrombocytopenic purpura syndrome in systemic lupus erythematosus: treatment with plasma infusion”. Am J Kidney Dis 1985:6: 154-160.
 
[47]  Fox DA, Faix JD, Coblyn J, Fraser P, Smith B, Weinblatt ME “Thrombotic thrombocytopenic purpura and systemic lupus erythematosus. Ann Rheum Dis 1986:45: 319-322.
 
[48]  Itoh Y, Sekine H, Hosono O et al. “Thrombotic thrombocytopenic purpura in two patients with systemic lupus erythematosus: clinical significance of anti-platelet antibodies”. Clin Immunol Immunopathol. 1990: 57: 125-136.
 
[49]  Jonsson OG, Fink CW “Systemic lupus erythematosus presenting as thrombotic thrombocytopenic purpura”. J Rheumatol 1990: 17: 973 974.
 
[50]  Hess DC, Sethi K, Awad E “Thrombotic thrombocytopenic purpura in systemic lupus erythematosus and antiphospholipid antibodies: effective treatment with plasma exchange and immunosuppression”. J Rheumatol. 1992: 19: 1474-1478.
 
[51]  Simeon Aznar CP, Cuenca-Luque R, Fonollosa-Pla V, Bosch Gil JA “Thrombotic thrombocytopenic purpura preceding systemic lupus erythematosus”. Ann Rheum Dis 1992.: 51: 396-398.
 
[52]  Stricker RB, Davis JA, Gershow J, Yamamoto KS, Kiprov DD “Thrombotic thrombocytopenic purpura complicating systemic lupus erythematosus. Case report and literature review from the plasmapheresis era”. J Rheumatol 1992:19: 1469-1473.
 
[53]  Braun J, Sieper J, Schwarz A et al. “Widespread vasculopathy with hemolytic uremic syndrome, perimyocarditis and cystic pancreatitis in a young woman with mixed connective tissue disease”. Rheumatol Int 1993:13: 31-36.
 
[54]  Porta C, Bobbio-Pallavicini E, Centurioni R, Caporali R, Montecucco CM “Thrombotic thrombocytopenic purpura in systemic lupus erythematosus. J Rheumatol 20: 1625-1626, 1993.
 
[55]  Bray VJ, West SG, Kristo DA Simultaneous presentation of thrombotic thrombocytopenic purpura and systemic lupus erythematosus. South Med J 1994:87: 827-830.
 
[56]  Jain R, Chartash E, Susin M, Furie R “Systemic lupus erythematosus complicated by thrombotic microangiopathy”. Semin Arthritis Rheum 1994:24: 173-182.
 
[57]  Nesher G, Hanna VE, Moore TL, HershM, Osborn TG “Thrombotic microangiopathic hemolytic anemia in systemic lupus erythematosus”. Semin Arthritis Rheum 1994.:24: 165-172.
 
[58]  Di Pietro G, Menicucci A, Venturini S, Avanti G “A case of thrombotic thrombocytopenic purpura preceding onset of systemic lupus erythematosus”. J Clin Apheresis 1996:11: 51-52.
 
[59]  Kaloterakis A, Vaiopoulos G, Filiotoy A et al. “ Concurrent development of thrombotic thrombocytopenic purpura and systemic lupus erythematosus in a male patient. Clin Exp Rheumatol: 1996:14: 581-582.
 
[60]  Lim GT, Kim SS, Park SH et al. “Thrombotic throm bocytopenic purpura-like syndrome associated with systemic lupus erythematosus. J Korean Med Sci 1992:7: 66-70.
 
[61]  Myung SJ, Yoo B, Lee KH et al. “A case of systemic lupus erythematosus developing two years after remission of thrombotic thrombocytopenic purpura. Korean J Intern Med 1996: 11: 178-182.
 
[62]  Caramaschi P, Riccetti MM, Pasini AF, Savarin T, Biasi D, Todeschini G “Systemic lupus erythematosus and thrombotic thrombocytopenic purpura”. Report of three cases and review of the literature. Lupus 1998:7: 37-41.
 
[63]  Jorfen M et al. “Fulminant thrombotic thrombocytopenic purpura in systemic lupus erythematosus”. Scand J Rheumatol 1998: 27: 76-77.
 
[64]  Perez-Sanchez I, Anguita J, Pintado T “Use of cyclophosphamide in the treatment of thrombotic thrombocytopenic purpura complicating systemic lupus erythematosus: report of two cases”. Ann Hematol 1999:78: 285-287.
 
[65]  Musa MO et al. “Fulminant thrombotic thrombocytopenic purpura in two patients with systemic lupus erythematosus and phospholipid antibodies”. Eur J Haematol 2000:64: 433-443.
 
[66]  Nanke Y, Akama H, Yamanaka H, Hara M, Kamatani N”Progressive appearance of overlap syndrome together with autoantibodies in a patient with fatal thrombotic microangiopathy”. Am J Med Sci, 2000: 320: 348-351.
 
[67]  Gungor T, Furlan M, Lammle B, Kuhn F, Seger RA “Acquired deficiency of von Willebrand factor-cleaving protease in a patient suffering from acute systemic lupus erythematosus”. (Letter) Rheumatology 40: 940-942, 2001.
 
[68]  Sakarcan A, Stallworth J “ Systemic lupus erythematosus and thrombotic thrombocytopenic purpura: a case and review”. Pediatr Nephrol 2001:16: 672-674.
 
[69]  Vaidya S, Abul-ezz S, Lipsmeyer E (2001) Thrombotic thrombocytopenic purpura and systemic lupus erythematosus. Scand J Rheumatol. 30: 308–3101. Musio F, Bohen EM, Yuan CM, Welch PG “Review of thrombotic thrombocytopenic purpura in the setting of sys- temic lupus erythematosus”. Semin Arthritis Rheum 1998:28: 1-19.
 
[70]  Pratish George, Jasmine Das, Basant Pawar, Naveen Kakkar “Thrombotic thrombocytopenic purpura and systemic lupus erythematosus: Successful management of a rare presentation”. Indian J Crit Care Med 2008: Vol 12: 129-131.
 
[71]  Risa Yamada, Kazuhisa Nozawa,b Takashi Yoshimine, Yoshinari Takasaki, Hideoki Ogawa, Kenji Takamori, and Iwao Sekigawa,” A Case of Thrombotic Thrombocytopenia Purpura Associated with Systemic Lupus Erythematosus: Diagnostic Utility of ADAMTS-13 Activity” SAGE-Hindawi Access to Research. Autoimmune Diseases. 2011: Volume, Article ID 483642, 1-6.
 
[72]  Selma Siham El Khayat, Ghislaine Medkouri, Armel Mbourou Etomba, Mohamed Zamd, et al.” Thrombotic Thrombocytopenic Purpura and Systemic Lupus Erythematosus: a Rare and Life-threatening Association” Arab Journal of Nephrology and Transplantation. 2012; 5(2): 103-5.
 
[73]  Lucia M. A. Campos, Maria Silvia Spadoni, Cintia M. Michelin” Thrombotic thrombocytopenic purpura at presentation of juvenile systemic lupus erythematosus patients” Rev Bras Reumatol; 2013:53(1): 120-126.
 
[74]  C. Porta, E. Bobbio-Pallavicini, R. Centurioni et al., “Thrombotic thrombocytopenic purpura in systemic lupus erythematosus,” Journal of Rheumatology, 1993: vol. 20, no. 9, pp. 1625-1626.
 
[75]  F. Musio, E. M. Bohen, C. M. Yuan, and P. G. Welch, “Review of thrombotic thrombocytopenic purpura in the setting of systemic lupus erythematosus,” Seminars in Arthritis and Rheumatism, 1998: vol. 28, no. 1, pp. 1-19.
 
Manuscript template