Haematological manifestations of lupus

Lupus Science and Medicine

Volumn 2, Issue 1, 2015, Pages

  Fayyaz, Anum ^a,b,c   Igoe, Ann ^a,b,d   Kurien, Biji T ^a,b,c   Danda, Debashish ^a,e   James, Judith A ^a,b,c   Stafford, Haraldine A ^f   Scofield, R Hal ^a,b,c  

^a OKLAHOMA MEDICAL RESEARCH FOUNDATION   (United States)

^b UNIVERSITY OF OKLAHOMA COLLEGE OF MEDICINE   (United States)

^c VETERANS AFFAIRS MEDICAL CENTER   (United States)

^d The MetroHealth System   (United States)

^e Christian Medical Centre   (India)

^f UNIVERSITY OF IOWA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

AUTOANTIBODY; AZATHIOPRINE; B CELL ACTIVATING FACTOR; BELIMUMAB; CYCLOPHOSPHAMIDE; CYCLOSPORIN; DANAZOL; GLUCOCORTICOID; GRANULOCYTE COLONY STIMULATING FACTOR; HYDROXYCHLOROQUINE; IMMUNOGLOBULIN; IMMUNOSUPPRESSIVE AGENT; METHYLPREDNISOLONE; PREDNISONE; RITUXIMAB; RUXOLITINIB; VINBLASTINE; VINCRISTINE;

ANTIPHOSPHOLIPID SYNDROME; AUTOIMMUNE HEMOLYTIC ANEMIA; CLINICAL FEATURE; CLINICAL TRIAL (TOPIC); CORTICOSTEROID THERAPY; DISEASE ACTIVITY; DISEASE CLASSIFICATION; GENETICS; HEMATOLOGIC DISEASE; HEMOLYTIC ANEMIA; HOSPITAL PATIENT; HUMAN; IDIOPATHIC THROMBOCYTOPENIC PURPURA; INFECTION SENSITIVITY; KIDNEY DISEASE; LEUKOPENIA; LYMPHOCYTOPENIA; MEDLINE; MORBIDITY; MYELOFIBROSIS; NEUTROPENIA; PATHOGENESIS; PRIORITY JOURNAL; PROGNOSIS; PROPHYLAXIS; RANDOMIZED CONTROLLED TRIAL (TOPIC); REMISSION; REVIEW; SPLENECTOMY; SURVIVAL; SYSTEMIC LUPUS ERYTHEMATOSUS; THROMBOCYTE COUNT; THROMBOCYTOPENIA; THROMBOTIC THROMBOCYTOPENIC PURPURA; TREATMENT RESPONSE;

EID: 84949193256     PISSN: None     EISSN: 20538790     Source Type: Journal    
DOI: 10.1136/lupus-2014-000078     Document Type: Review

References (177)

1. Lie TH, Rothfield NF. An evaluation of the preliminary criteria for the diagnosis of systemic lupus erythematosus. Arthritis Rheum 1972;15:532-4.
2. Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982;25:1271-7.
3. Feletar M, Ibanez D, Urowitz MB, et al. The impact of the 1997 update of the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus: what has been changed? Arthritis Rheum 2003;48:2067-9.
4. Petri M, Orbai AM, Alarcon GS, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 2012;64:2677-86.
5. Katsanis E, Hsu E, Luke KH, et al. Systemic lupus erythematosus and sickle hemoglobinopathies: A report of two cases and review of the literature. Am J Hematol 1987;25:211-14.
6. Harvey AM, Shulman LE, Tumulty PA, et al. Systemic lupus erythematosus: review of the literature and clinical analysis of 138 cases. Medicine (Baltimore) 1954;33:291-437.
7. Arenas M, Abad A, Valverde V, et al. Selective inhibition of granulopoiesis with severe neutropenia in systemic lupus erythematosus. Arthritis Rheum 1992;35:979-80.
8. Haddy TB, Rana SR, Castro O. Benign ethnic neutropenia: what is a normal absolute neutrophil count? J Lab Clin Med 1999;133:15-22.
9. Starkebaum G, Price TH, Lee MY, et al. Autoimmune neutropenia in systemic lupus erythematosus. Arthritis Rheum 1978;21: 504-12.
10. Yamasaki K, Niho Y, Yanase T. Granulopoiesis in systemic lupus erythematosus. Arthritis Rheum 1983;26:516-21.
11. Rustagi PK, Currie MS, Logue GL. Complement-activating antineutrophil antibody in systemic lupus erythematosus. Am J Med 1985;78(6 Pt 1):971-7.
12. Kurien BT, Newland J, Paczkowski C, et al. Association of neutropenia in systemic lupus erythematosus (SLE) with anti-Ro and binding of an immunologically cross-reactive neutrophil membrane antigen. Clin Exp Immunol 2000;120:209-17.
13. Harmon DC, Weitzman SA, Stossel TP. The severity of immune neutropenia correlates with the maturational specificity of antineutrophil antibodies. Br J Haematol 1984;58:209-15.
14. Duckham DJ, Rhyne RL Jr, Smith FE, et al. Retardation of colony growth of in vitro bone marrow culture using sera from patients with Felty's syndrome, disseminated lupus erythematosus (SLE), rheumatoid arthritis, and other disease states. Arthritis Rheum 1975;18:323-33.
15. Gergely P. Severe neutropenia in systemic lupus erythematosus. Arthritis Rheum 1984;27:719.
16. Matsuyama W, Yamamoto M, Higashimoto I, et al. TNF-related apoptosis-inducing ligand is involved in neutropenia of systemic lupus erythematosus. Blood 2004;104:184-91.
17. Martinez-Banos D, Crispin JC, Lazo-Langner A, et al. Moderate and severe neutropenia in patients with systemic lupus erythematosus. Rheumatology (Oxford) 2006;45:994-8.
18. Sugimoto T, Soumura M, Tanaka Y, et al. Early morning neutropenia in a patient with systemic lupus erythematosus. Mod Rheumatol 2006;16:267-8.
19. Kondo H, Date Y, Sakai Y, et al. Effective simultaneous rhG-CSF and methylprednisolone "pulse" therapy in agranulocytosis associated with systemic lupus erythematosus. Am J Hematol 1994;46:157-8.
20. Euler HH, Harten P, Zeuner RA, et al. Recombinant human granulocyte colony stimulating factor in patients with systemic lupus erythematosus associated neutropenia and refractory infections. J Rheumatol 1997;24:2153-7.
21. Rivero SJ, Diaz-Jouanen E, Alarcon-Segovia D. Lymphopenia in systemic lupus erythematosus. Clinical, diagnostic, and prognostic significance. Arthritis Rheum 1978;21:295-305.
22. Bhuyan UN, Malaviya AN. Deficiency of T cells in blood and tissues and severity of infections in systemic lupus erythematosus. Indian J Med Res 1978;67:269-78.
23. Glinski W, Gershwin ME, Budman DR, et al. Study of lymphocyte subpopulations in normal humans and patients with systemic lupus erythematosus by fractionation of peripheral blood lymphocytes on a discontinuous Ficoll gradient. Clin Exp Immunol 1976;26:228-38.
24. Laurence J, Wong JE, Nachman R. The cellular hematology of systemic lupus erythematosus. In: Lahita RG, ed. Systemic Lupus erythematosus. 2nd edn. New York: Churchhill Livingston, 1992:771-806.
25. Stohl W, Crow MK, Kunkel HG. Systemic lupus erythematosus with deficiency of the T4 epitope on T helper/inducer cells. N Engl J Med 1985;312:1671-8.
26. Odendahl M, Jacobi A, Hansen A, et al. Disturbed peripheral B lymphocyte homeostasis in systemic lupus erythematosus. J Immunol 2000;165:5970-9.
27. Massardo L, Metz C, Pardo E, et al. Autoantibodies against galectin-8: Their specificity, association with lymphopenia in systemic lupus erythematosus and detection in rheumatoid arthritis and acute inflammation. Lupus 2009;18:539-46.
28. Chen W, Lin J. Lymphopenia relating to T-lymphocyte apoptosis in systemic lupus erythematosus. Clin Rheumatol 2011;30:1515-16.
29. Silva LM, Garcia AB, Donadi EA. Increased lymphocyte death by neglect-apoptosis is associated with lymphopenia and autoantibodies in lupus patients presenting with neuropsychiatric manifestations. J Neurol 2002;249:1048-54.
30. Keser G, Sequeira J, Khamashta MA, et al. Anti-Ro and lymphopenia in SLE. Lupus 1993;2:63.
31. Winfield JB, Mimura T. Pathogenetic significance of anti-lymphocyte autoantibodies in systemic lupus erythematosus. Clin Immunol Immunopathol 1992;63:13-16.
32. Noguchi M, Iwamori M, Hirano T, et al. Autoantibodies to T and B cell lines detected in serum samples from patients with systemic lupus erythematosus with lymphopenia and hypocomplementaemia. Ann Rheum Dis 1992;51:713-16.
33. Stafford HA, Chen AE, Anderson CJ, et al. Anti-ribosomal and 'P-peptide'-specific autoantibodies bind to T lymphocytes. Clin Exp Immunol 1997;109:12-19.
34. Emlen W, Niebur J, Kadera R. Accelerated in vitro apoptosis of lymphocytes from patients with systemic lupus erythematosus. J Immunol 1994;152:3685-92.
35. Amasaki Y, Kobayashi S, Takeda T, et al. Up-regulated expression of Fas antigen (CD95) by peripheral naive and memory T cell subsets in patients with systemic lupus erythematosus (SLE): A possible mechanism for lymphopenia. Clin Exp Immunol 1995;99:245-50.
36. Wenzel J, Gerdsen R, Uerlich M, et al. Lymphocytopenia in lupus erythematosus: close in vivo association to autoantibodies targeting nuclear antigens. Br J Dermatol 2004;150:994-8.
37. Nossent JC, Swaak AJ. Prevalence and significance of haematological abnormalities in patients with systemic lupus erythematosus. Q J Med 1991;80:605-12.
38. Porges AJ, Beattie SL, Ritchlin C, et al. Patients with systemic lupus erythematosus at risk for Pneumocystis carinii pneumonia. J Rheumatol 1992;19:1191-4.
39. Ginzler EM. Infections in systemic lupus erythematosus. In: Hahn BH. ed. DUBOIS's Lupus Erythematosus. 5th edn. Maryland: Williams & Wilkins, 1997:903-14.
40. Isenberg DA, Patterson KG, Todd-Pokropek A, et al. Haematological aspects of systemic lupus erythematosus: A reappraisal using automated methods. Acta Haematol 1982;67:242-8.
41. Mirzayan MJ, Schmidt RE, Witte T. Prognostic parameters for flare in systemic lupus erythematosus. Rheumatology (Oxford) 2000;39:1316-19.
42. Manzi S, Sanchez-Guerrero J, Merrill JT, et al. Effects of belimumab, a B lymphocyte stimulator-specific inhibitor, on disease activity across multiple organ domains in patients with systemic lupus erythematosus: combined results from two phase III trials. Ann Rheum Dis 2012;71:1833-8.
43. Kapouzas GA. Hematological and Lymphoid Abnomalities in SLE. In: Wallace DJ, Hahn BH, eds. DUBOIS' Lupus erythmatosus and Related Syndomes. 8th edn. Philadelphia: Elsevier, 2013:426-37.
44. Howe SE, Lynch DM. Platelet antibody binding in systemic lupus erythematosus. J Rheumatol 1987;14:482-6.
45. Drenkard C, Villa AR, Alarcon-Segovia D, et al. Influence of the antiphospholipid syndrome in the survival of patients with systemic lupus erythematosus. J Rheumatol 1994;21:1067-72.
46. Fabris F, Casonato A, Randi ML, et al. Clinical significance of surface and internal pools of platelet-associated immunoglobulins in immune thrombocytopenia. Scand J Haematol 1986;37:215-20.
47. Michel M, Chanet V, Dechartres A, et al. The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases. Blood 2009;114:3167-72.
48. Pujol M, Ribera A, Vilardell M, et al. High prevalence of platelet autoantibodies in patients with systemic lupus erythematosus. Br J Haematol 1995;89:137-41.
49. Ahmed AE, Peter JB, Shoenfeld YY. ANCA testing. New developments and clinical implications. Clin Rev Allergy Immunol 1998;16:303-11.
50. Fabris F, Steffan A, Cordiano I, et al. Specific antiplatelet autoantibodies in patients with antiphospholipid antibodies and thrombocytopenia. Eur J Haematol 1994;53:232-6.
51. Rupin A, Gruel Y, Poumier-Gaschard P, et al. Thrombocytopenia in systemic lupus erythematosus: Association with antiplatelet and anticardiolipin antibodies. Clin Immunol Immunopathol 1990;55:418-26.
52. Nojima J, Kuratsune H, Suehisa E, et al. Association between the prevalence of antibodies to beta(2)-glycoprotein I, prothrombin, protein C, protein S, and annexin V in patients with systemic lupus erythematosus and thrombotic and thrombocytopenic complications. Clin Chem 2001;47:1008-15.
53. Munoz-Rodriguez FJ, Reverter JC, Font J, et al. Prevalence and clinical significance of antiprothrombin antibodies in patients with systemic lupus erythematosus or with primary antiphospholipid syndrome. Haematologica 2000;85:632-7.
54. Abu-Shakra M, Gladman DD, Urowitz MB, et al. Anticardiolipin antibodies in systemic lupus erythematosus: clinical and laboratory correlations. Am J Med 1995;99:624-8.
55. Lopez-Soto A, Cervera R, Font J, et al. Isotype distribution and clinical significance of antibodies to cardiolipin, phosphatidic acid, phosphatidylinositol and phosphatidylserine in systemic lupus erythematosus: prospective analysis of a series of 92 patients. Clin Exp Rheumatol 1997;15:143-9.
56. Morgan M, Downs K, Chesterman CN, et al. Clinical analysis of 125 patients with the lupus anticoagulant. Aust N Z J Med 1993;23:151-6.
57. Scofield RH, Bruner GR, Kelly JA, et al. Thrombocytopenia identifies a severe familial phenotype of systemic lupus erythematosus and reveals genetic linkages at 1q22 and 11p13. Blood 2003;101:992-7.
58. Love PE, Santoro SA. Antiphospholipid antibodies: Anticardiolipin and the lupus anticoagulant in systemic lupus erythematosus (SLE) and in non-SLE disorders. Prevalence and clinical significance. Ann Intern Med 1990;112:682-98.
59. Ryan WL, Hakenkamp K, Sullivan E. A new platelet function test. Thromb Res 1990;58:163-73.
60. Kaufman KM, Rankin J, Harley IT, et al. A genetic marker within the CD44 gene confirms linkage at 11p13 in African-American families with lupus stratified by thrombocytopenia, but genetic association with CD44 is not present. Genes Immun 2002;3(Suppl 1):S86-8.
61. Kumakura S, Murakawa Y. Clinical characteristics and treatment outcomes of autoimmune-associated hemophagocytic syndrome in adults. Arthritis Rheumatol 2014;66:2297-307.
62. Tsao BP, Grossman JM, Riemekasten G, et al. Familiality and co-occurrence of clinical features of systemic lupus erythematosus. Arthritis Rheum 2002;46:2678-85.
63. Piotrowski P, Lianeri M, Gasik R, et al. Monocyte chemoattractant protein-1-2518 A/G single nucleotide polymorphism might be associated with renal disease and thrombocytopenia of SLE. J Biomed Biotechnol 2010;2010:130265.
64. Mestanza-Peralta M, Ariza-Ariza R, Cardiel MH, et al. Thrombocytopenic purpura as initial manifestation of systemic lupus erythematosus. J Rheumatol 1997;24:867-70.
65. Miller MH, Urowitz MB, Gladman DD. The significance of thrombocytopenia in systemic lupus erythematosus. Arthritis Rheum 1983;26:1181-6.
66. Feinglass EJ, Arnett FC, Dorsch CA, et al. Neuropsychiatric manifestations of systemic lupus erythematosus: diagnosis, clinical spectrum, and relationship to other features of the disease. Medicine (Baltimore) 1976;55:323-39.
67. Alger M, Alarcon-Segovia D, Rivero SJ. Hemolytic anemia and thrombocytopenic purpura: Two related subsets of systemic lupus erythematosus. J Rheumatol 1977;4:351-7.
68. Mok CC, Lee KW, Ho CT, et al. A prospective study of survival and prognostic indicators of systemic lupus erythematosus in a southern Chinese population. Rheumatology (Oxford) 2000;39:399-406.
69. Ward MM, Pyun E, Studenski S. Long-term survival in systemic lupus erythematosus. Patient characteristics associated with poorer outcomes. Arthritis Rheum 1995;38:274-83.
70. Reveille JD, Bartolucci A, Alarcon GS. Prognosis in systemic lupus erythematosus. Negative impact of increasing age at onset, black race, and thrombocytopenia, as well as causes of death. Arthritis Rheum 1990;33:37-48.
71. Pistiner M, Wallace DJ, Nessim S, et al. Lupus erythematosus in the 1980s: A survey of 570 patients. Semin Arthritis Rheum 1991;21:55-64.
72. Massardo L, Martinez ME, Jacobelli S, et al. Survival of Chilean patients with systemic lupus erythematosus. Semin Arthritis Rheum 1994;24:1-11.
73. Swaak AJ, Nossent JC, Bronsveld W, et al. Systemic lupus erythematosus. I. Outcome and survival: Dutch experience with 110 patients studied prospectively. Ann Rheum Dis 1989;48:447-54.
74. Arnal C, Piette JC, Leone J, et al. Treatment of severe immune thrombocytopenia associated with systemic lupus erythematosus: 59 cases. J Rheumatol 2002;29:75-83.
75. Eyanson S, Passo MH, Aldo-Benson MA, et al. Methylprednisolone pulse therapy for nonrenal lupus erythematosus. Ann Rheum Dis 1980;39:377-80.
76. Marino C, Cook P. Danazol for lupus thrombocytopenia. Arch Intern Med 1985;145:2251-2.
77. Agnello V, Pariser K, Gell J, et al. Preliminary observations on danazol therapy of systemic lupus erythematosus: effects on DNA antibodies, thrombocytopenia and complement. J Rheumatol 1983;10:682-7.
78. Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V, et al. Successful therapy with danazol in refractory autoimmune thrombocytopenia associated with rheumatic diseases. Br J Rheumatol 1997;36:1095-9.
79. West SG, Johnson SC. Danazol for the treatment of refractory autoimmune thrombocytopenia in systemic lupus erythematosus. Ann Intern Med 1988;108:703-6.
80. Cervera H, Jara LJ, Pizarro S, et al. Danazol for systemic lupus erythematosus with refractory autoimmune thrombocytopenia or Evans' syndrome. J Rheumatol 1995;22:1867-71.
81. Moss C, Hamilton PJ. Thrombocytopenia in systemic lupus erythematosus responsive to dapsone. BMJ (Clinical research ed) 1988;297:266.
82. Maier-Moore JS, Koelsch KA, Smith K, et al. Antibody-secreting cell specificity in labial salivary glands reflects clinical presentation and serology in sjogren's syndrome patients. Arthritis Rheumatol 2014;66:3445-56.
83. ter Borg EJ, Kallenberg CG. Treatment of severe thrombocytopenia in systemic lupus erythematosus with intravenous gammaglobulin. Ann Rheum Dis 1992;51:1149-51.
84. Roldan R, Roman J, Lopez D, et al. Treatment of hemolytic anemia and severe thrombocytopenia with high-dose methylprednisolone and intravenous immunoglobulins in SLE. Scand J Rheumatol 1994;23:218-9.
85. Chandramouli NB, Rodgers GM. Prolonged immunoglobulin and platelet infusion for treatment of immune thrombocytopenia. Am J Hematol 2000;65:85-6.
86. Ahn YS, Harrington WJ, Seelman RC, et al. Vincristine therapy of idiopathic and secondary thrombocytopenias. N Engl J Med 1974;291:376-80.
87. Griffiths B, Emery P. The treatment of lupus with cyclosporin A. Lupus 2001;10:165-70.
88. Sekigawa I, Ogasawara H, Sugiyama M, et al. Extremely low dose treatment of cyclosporine for autoimmune diseases. Clin Exp Rheumatol 1998;16:352.
89. Manger K, Kalden JR, Manger B. Cyclosporin A in the treatment of systemic lupus erythematosus: results of an open clinical study. Br J Rheumatol 1996;35:669-75.
90. Roach BA, Hutchinson GJ. Treatment of refractory, systemic lupus erythematosus-associated thrombocytopenia with intermittent low-dose intravenous cyclophosphamide. Arthritis Rheum 1993;36:682-4.
91. Boumpas DT, Barez S, Klippel JH, et al. Intermittent cyclophosphamide for the treatment of autoimmune thrombocytopenia in systemic lupus erythematosus. Ann Intern Med 1990;112:674-7.
92. Park HJ, Kang MI, Kang Y, et al. Two cases of refractory thrombocytopenia in systemic lupus erythematosus that responded to intravenous low-dose cyclophosphamide. J Korean Med Sci 2013;28:472-5.
93. Dang Do AN, Umoren RA, Tarvin SE, et al. Systemic lupus erythematosus in a 3-month old male presenting as thrombocytopenia. Lupus 2011;20:527-30.
94. Chang HK. Successful treatment of refractory thrombocytopenia with mycophenolate mofetil in a patient with systemic lupus erythematosus. J Korean Med Sci 2005;20:883-5.
95. Hepburn AL, Narat S, Mason JC. The management of peripheral blood cytopenias in systemic lupus erythematosus. Rheumatology (Oxford) 2010;49:2243-54.
96. Lateef A, Lahiri M, Teng GG, et al. Use of rituximab in the treatment of refractory systemic lupus erythematosus: Singapore experience. Lupus 2010;19:765-70.
97. Garcia-Carrasco M, Mendoza-Pinto C, Sandoval-Cruz M, et al. Anti-CD20 therapy in patients with refractory systemic lupus erythematosus: A longitudinal analysis of 52 Hispanic patients. Lupus 2010;19:213-9.
98. Chen H, Zheng W, Su J, et al. Low-dose rituximab therapy for refractory thrombocytopenia in patients with systemic lupus erythematosus-a prospective pilot study. Rheumatology (Oxford) 2011;50:1640-4.
99. Gonzalez-Nieto JA, Martin-Suarez I, Quattrino S, et al. The efficacy of romiplostim in the treatment of severe thrombocytopenia associated to Evans syndrome refractory to rituximab. Lupus 2011;20:1321-3.
100. Liang Y, Zhang L, Gao J, et al. Rituximab for children with immune thrombocytopenia: A systematic review. PLoS ONE 2012;7:e36698.
101. Kumar S, Benseler SM, Kirby-Allen M, et al. B-cell depletion for autoimmune thrombocytopenia and autoimmune hemolytic anemia in pediatric systemic lupus erythematosus. Pediatrics 2009;123: e159-63.
102. Feinglass S, Deodhar A. Treatment of lupus-induced thrombocytopenia with recombinant human interleukin-11. Arthritis Rheum 2001;44:170-5.
103. Coon WW. Splenectomy for cytopenias associated with systemic lupus erythematosus. Am J Surg 1988;155:391-4.
104. Gruenberg JC, VanSlyck EJ, Abraham JP. Splenectomy in systemic lupus erythematosis. Am Surg 1986;52:366-70.
105. Jacobs P, Wood L, Dent DM. Splenectomy and the thrombocytopenia of systemic lupus erythematosus. Ann Intern Med 1986;105:971-2.
106. Hall S, McCormick JL Jr, Greipp PR, et al. Splenectomy does not cure the thrombocytopenia of systemic lupus erythematosus. Ann Intern Med 1985;102:325-8.
107. Rivero SJ, Alger M, Alarcon-Segovia D. Splenectomy for hemocytopenia in systemic lupus erythematosus. A controlled appraisal. Arch Int Med 1979;139:773-6.
108. Hakim AJ, Machin SJ, Isenberg DA. Autoimmune thrombocytopenia in primary antiphospholipid syndrome and systemic lupus erythematosus: The response to splenectomy. Semin Arthritis Rheum 1998;28:20-5.
109. Alarcon-Segovia D. Splenectomy has a limited role in the management of lupus with thrombocytopenia. J Rheumatol 2002;29:1-2.
110. Wall BA, Weinblatt ME, Agudelo CA. Plasmapheresis in the treatment of resistant thrombocytopenia in systemic lupus erythematosus. South Med J 1982;75:1277-8.
111. Sakamoto H, Takaoka T, Usami M, et al. Apheresis: clinical response to patients unresponsive to conventional therapy. Trans Am Soc Artif Intern Organs 1985;31:704-10.
112. Sthoeger Z, Sthoeger D, Green L, et al. The role of anticardiolipin autoantibodies in the pathogenesis of autoimmune hemolytic anemia in systemic lupus erythematosus. J Rheumatol 1993;20:2058-61.
113. Sturfelt G, Nived O, Norberg R, et al. Anticardiolipin antibodies in patients with systemic lupus erythematosus. Arthritis Rheum 1987;30:382-8.
114. Lang B, Straub RH, Weber S, et al. Elevated anticardiolipin antibodies in autoimmune haemolytic anaemia irrespective of underlying systemic lupus erythematosus. Lupus 1997;6:652-5.
115. Barker RN, de Sa Oliveira GG, Elson CJ, et al. Pathogenic autoantibodies in the NZB mouse are specific for erythrocyte band 3 protein. Eur J Immunol 1993;23:1723-6.
116. Kay MM, Marchalonis JJ, Hughes J, et al. Definition of a physiologic aging autoantigen by using synthetic peptides of membrane protein band 3: localization of the active antigenic sites. Proc Natl Acad Sci U S A 1990;87:5734-8.
117. Ruiz-Arguelles A, Llorente L. The role of complement regulatory proteins (CD55 and CD59) in the pathogenesis of autoimmune hemocytopenias. Autoimmun Rev 2007;6:155-61.
118. Costallat GL, Appenzeller S, Costallat LT. Evans syndrome and systemic lupus erythematosus: clinical presentation and outcome. Joint Bone Spine 2012;79:362-4.
119. Gomard-Mennesson E, Ruivard M, Koenig M, et al. Treatment of isolated severe immune hemolytic anaemia associated with systemic lupus erythematosus: 26 cases. Lupus 2006;15:223-31.
120. Abdwani R, Mani R. Anti-CD20 monoclonal antibody in acute life threatening haemolytic anaemia complicating childhood onset SLE. Lupus 2009;18:460-4.
121. Tamimoto Y, Horiuchi T, Tsukamoto H, et al. A dose-escalation study of rituximab for treatment of systemic lupus erythematosus and Evans' syndrome: immunological analysis of B cells, T cells and cytokines. Rheumatology (Oxford) 2008;47:821-7.
122. Scheinberg M, Hamerschlak N, Kutner JM, et al. Rituximab in refractory autoimmune diseases: Brazilian experience with 29 patients (2002-2004). Clin Exp Rheumatol 2006;24:65-9.
123. Moschkowitz E. Hyaline thrombosis of the terminal arterioles and capillaries: A hitherto undescribed disease. Proc Natl Acad Sci U S A 1924;24:21-4.
124. Singer K, Bornstein FP, Wile SA. Thrombotic thrombocytopenic purpura; hemorrhagic diathesis with generalized platelet thromboses. Blood 1947;2:542-54.
125. Amorosi E, Ultmann J. Thrombotic thrombocytopenic purpura: report of 16 cases and review of the literature. Medicine 1966;45:139.
126. George JN, Vesely SK, James JA. Overlapping features of thrombotic thrombocytopenic purpura and systemic lupus erythematosus. South Med J 2007;100:512-14.
127. Porta C, Caporali R, Montecucco C. Thrombotic thrombocytopenic purpura and autoimmunity: A tale of shadows and suspects. Haematologica 1999;84:260-9.
128. Furlan M, Robles R, Galbusera M, et al. von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 1998;339:1578-84.
129. Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 1998;339:1585-94.
130. Devinsky O, Petito CK, Alonso DR. Clinical and neuropathological findings in systemic lupus erythematosus: The role of vasculitis, heart emboli, and thrombotic thrombocytopenic purpura. Ann Neurol 1988;23:380-4.
131. Stricker RB, Davis JA, Gershow J, et al. Thrombotic thrombocytopenic purpura complicating systemic lupus erythematosus. Case report and literature review from the plasmapheresis era. J Rheumatol 1992;19:1469-73.
132. Yuen LK, Lai WM, Tong PC, et al. Recurrent thrombotic thrombocytopenic purpura in a young boy with systemic lupus erythematosus. J Clin Rheumatol 2007;13:224-8.
133. Brunner HI, Freedman M, Silverman ED. Close relationship between systemic lupus erythematosus and thrombotic thrombocytopenic purpura in childhood. Arthritis Rheum 1999;42:2346-55.
134. Vaidya S, Abul-ezz S, Lipsmeyer E. Thrombotic thrombocytopenic purpura and systemic lupus erythematosus. Scand J Rheumatol 2001;30:308-10.
135. Zheng T, Chunlei L, Zhen W, et al. Clinical-pathological features and prognosis of thrombotic thrombocytopenic purpura in patients with lupus nephritis. Am J Med Sci 2009;338:343-7.
136. Letchumanan P, Ng HJ, Lee LH, et al. A comparison of thrombotic thrombocytopenic purpura in an inception cohort of patients with and without systemic lupus erythematosus. Rheumatology (Oxford) 2009;48:399-403.
137. Moake JL. Thrombotic thrombocytopenic purpura today. Hospital practice (1995) 1999;34:53-9.
138. 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;325:393-7.
139. Kafle P, Malakoff GL. Coexistence of systemic lupus erythematosus and thrombotic thrombocytopenic purpura: A case report. Tenn Med 2012;105:37-8.
140. Aziz AR, Mohammadian Y, Ruby C, et al. Systemic lupus erythematosus presenting with pancytopenia due to bone marrow myelofibrosis in a 22-year-old male. Clin Adv Hematol Oncol 2004;2:467-9; discussion 69-70.
141. Burkhardt R. [Cytology and histology of the human bone marrow]. Hamatologie und Bluttransfusion 1968;4:11-29.
142. Daly HM, Scott GL. Myelofibrosis as a cause of pancytopenia in systemic lupus erythematosus. J Clin Pathol 1983;36:1219-22.
143. Lau KS, White JC. Myelosclerosis associated with systemic lupus erythematosus in patients in West Malaysia. J Clin Pathol 1969;22:433-8.
144. Wanitpongpun C, Teawtrakul N, Mahakkanukrauh A, et al. Bone marrow abnormalities in systemic lupus erythematosus with peripheral cytopenia. Clin Exp Rheumatol 2012;30:825-9.
145. Feng CS, Ng MH, Szeto RS, et al. Bone marrow findings in lupus patients with pancytopenia. Pathology 1991;23:5-7.
146. Pereira RM, Velloso ER, Menezes Y, et al. Bone marrow findings in systemic lupus erythematosus patients with peripheral cytopenias. Clin Rheumatol 1998;17:219-22.
147. Aharon A, Levy Y, Bar-Dayan Y, et al. Successful treatment of early secondary myelofibrosis in SLE with IVIG. Lupus 1997;6:408-11.
148. Gotlib J. JAK inhibition in the myeloproliferative neoplasms: lessons learned from the bench and bedside. Hematology Am Soc Hematol Educ Program 2013;2013:529-37.
149. Verstovsek S, Mesa RA, Gotlib J, et al. A double-blind, placebo-controlled trial of ruxolitinib for myelofibrosis. N Engl J Med 2012;366:799-807.
150. Harrison C, Kiladjian JJ, Al-Ali HK, et al. JAK inhibition with ruxolitinib versus best available therapy for myelofibrosis. N Engl J Med 2012;366:787-98.
151. Verstovsek S, Mesa RA, Gotlib J, et al. Efficacy, safety and survival with ruxolitinib in patients with myelofibrosis: results of a median 2-year follow-up of COMFORT-I. Haematologica 2013;98:1865-71.
152. Frohlich K, Holle JU, Aries PM, et al. Successful use of bortezomib in a patient with systemic lupus erythematosus and multiple myeloma. Ann Rheum Dis 2011;70:1344-5.
153. Quartuccio L, Rupolo M, Michieli M, et al. Efficacy and tolerability of repeated cycles of a once-weekly regimen of bortezomib in lupus. Rheumatology (Oxford) 2014;53:381-2.
154. Trivedi T, Franek BS, Green SL, et al. Osteopontin alleles are associated with clinical characteristics in systemic lupus erythematosus. Journal of biomedicine & biotechnology 2011;2011:802581.
155. Amengual O, Atsumi T, Komano Y, et al. A polymorphism in human platelet antigen 6b and risk of thrombocytopenia in patients with systemic lupus erythematosus. Arthritis Rheum 2007;56:2803-5.
156. Nolsoe RL, Kelly JA, Pociot F, et al. Functional promoter haplotypes of the human FAS gene are associated with the phenotype of SLE characterized by thrombocytopenia. Genes Immun 2005;6:699-706.
157. Namjou B, Choi CB, Harley IT, et al. Evaluation of TRAF6 in a large multiancestral lupus cohort. Arthritis Rheum 2012;64:1960-9.
158. Jeon JY, Kim HA, Kim SH, et al. Association between variable number tandem repeats within the 3' flanking region of the interleukin-6 gene and systemic lupus erythematosus in Korean patients. Lupus 2011;20:1161-5.
159. Jeon JY, Kim KY, Kim HA, et al. The interleukin 6 receptor alpha gene polymorphisms are associated with clinical manifestations of systemic lupus erythematosus in Koreans. Int J Immunogenet 2013;40:356-60.
160. Chan HC, Ke LY, Chang LL, et al. Suppressor of cytokine signaling 1 gene expression and polymorphisms in systemic lupus erythematosus. Lupus 2010;19:696-702.
161. Kim HA, Chun HY, Kim SH, et al. C-reactive protein gene polymorphisms in disease susceptibility and clinical manifestations of Korean systemic lupus erythematosus. J Rheumatol 2009;36:2238-43.
162. Warchol T, Lianeri M, Wudarski M, et al. Catalase-262C>T polymorphism in systemic lupus erythematosus in Poland. Rheumatol Int 2008;28:1035-9.
163. Hong CH, Lee JS, Lee HS, et al. The association between fcgammaRIIIB polymorphisms and systemic lupus erythematosus in Korea. Lupus 2005;14:346-50.
164. Agarwal BR, Bhalla K, Dalvi R, et al. Myelofibrosis secondary to SLE and its reversal on steroid therapy. Indian Pediatr 1995;32:1207-10.
165. el Mouzan MI, Ahmad MA, al Fadel Saleh M, et al. Myelofibrosis and pancytopenia in systemic lupus erythematosus. Acta Haematol 1988;80:219-21.
166. Foley-Nolan D, Martin MF, Rowbotham D, et al. Systemic lupus erythematosus presenting with myelofibrosis. J Rheumatol 1992;19:1303-4.
167. Fukuya H, Hirose W, Masuda T, et al. [A case of systemic lupus erythematosus presenting with myelofibrosis as a cause of pancytopenia]. Ryumachi [Rheumatism] 1994;34:773-8.
168. Inoue Y, Matsubara A, Okuya S, et al. Myelofibrosis and systemic lupus erythematosus: reversal of fibrosis with high-dose corticosteroid therapy. Acta Haematol 1992;88:32-6.
169. Kaelin WG Jr, Spivak JL. Systemic lupus erythematosus and myelofibrosis. Am J Med 1986;81:935-8.
170. Kiss E, Gal I, Simkovics E, et al. Myelofibrosis in systemic lupus erythematosus. Leuk Lymphoma 2000;39:661-5.
171. Konstantopoulos K, Terpos E, Prinolakis H, et al. Systemic lupus erythematosus presenting as myelofibrosis. Haematologia 1998;29:153-6.
172. Matsouka C, Liouris J, Andrianakos A, et al. Systemic lupus erythematosus and myelofibrosis. Clin Rheumatol 1989;8:402-7.
173. Nanji AA, Jetha N. Myelofibrosis as a cause of pancytopenia in systemic lupus erythematosus. J Clin Pathol 1984;37:714.
174. Paquette RL, Meshkinpour A, Rosen PJ. Autoimmune myelofibrosis. A steroid-responsive cause of bone marrow fibrosis associated with systemic lupus erythematosus. Medicine (Baltimore) 1994;73:145-52.
175. Pillai A, Gautam M, Williamson H, et al. Multisystem failure due to three coexisting autoimmune diseases. Intern Med 2009;48:837-42.
176. Ramakrishna R, Kyle PW, Day PJ, et al. Evans' syndrome, myelofibrosis and systemic lupus erythematosus: role of procollagens in myelofibrosis. Pathology 1995;27:255-9.
177. Raut DS, Saha S. Myelofibrosis secondary to SLE and its reversal on steroid therapy. Indian Pediatr 1996;33:522-3; author reply 23-4.