A child with acquired factor XIII deficiency: Case report and literature review

Haemophilia

Volumn 19, Issue 6, 2013, Pages 814-826

  Kessel, R ^a   Hu, C ^a   Shore Lesserson, L ^b   Rand, J ^b   Manwani, D ^a  

^a CHILDREN S HOSPITAL AT MONTEFIORE   (United States)

^b MONTEFIORE MEDICAL CENTER   (United States)

Author keywords

Bleeding diathesis; Factor XIII assays; Factor XIII deficiency

Indexed keywords

AMINOCAPROIC ACID; BLOOD CLOTTING FACTOR 13 CONCENTRATE; BLOOD CLOTTING FACTOR 13A; BLOOD CLOTTING FACTOR 13B; FIBRIN; FRESH FROZEN PLASMA; HEMOGLOBIN; METHYLPREDNISOLONE; NEUTRALIZING ANTIBODY; PENICILLIN DERIVATIVE; PREDNISOLONE; RITUXIMAB; STEROID;

ANTIBODY TITER; BLEEDING; BLOOD CLOTTING DISORDER; BLOOD CLOTTING FACTOR 13 DEFICIENCY; BLOOD VESSEL PERMEABILITY; BRAIN HEMORRHAGE; CASE REPORT; CHILD; CONSANGUINITY; CROSS LINKING; DISEASE COURSE; DRUG HALF LIFE; DRUG MEGADOSE; DRUG USE; EXOPHTHALMOS; EYELID EDEMA; FEMALE; FIBRINOGEN BLOOD LEVEL; FOLLOW UP; HUMAN; IMMUNOLOGICAL TOLERANCE; MEDICAL HISTORY; NUCLEAR MAGNETIC RESONANCE IMAGING; PHASE 2 CLINICAL TRIAL (TOPIC); PHASE 3 CLINICAL TRIAL (TOPIC); PRIORITY JOURNAL; PROGNOSIS; REVIEW; SCARLET FEVER; SCHOOL CHILD; STREPTOCOCCAL PHARYNGITIS; THROMBOCYTE AGGREGATION; THROMBOCYTE FUNCTION; THROMBOELASTOGRAPHY; TREATMENT RESPONSE;

BLEEDING DIATHESIS; FACTOR XIII ASSAYS; FACTOR XIII DEFICIENCY;

CHILD; FACTOR XIII; FACTOR XIII DEFICIENCY; FEMALE; FIBRINOLYSIN; FIBRINOLYTIC AGENTS; HEMATOMA; HUMANS; MAGNETIC RESONANCE IMAGING; PROGNOSIS; PROTEIN SUBUNITS; RECOMBINANT PROTEINS; TREATMENT OUTCOME;

EID: 84886640096     PISSN: 13518216     EISSN: 13652516     Source Type: Journal    
DOI: 10.1111/hae.12145     Document Type: Review

References (61)

1. Karimi M, Bereczky Z, Cohan N, Muszbek L. Factor XIII deficiency. Semin Thromb Hemost 2009; 35: 426-38. Epub 2009/07/15.
2. Komaromi I, Bagoly Z, Muszbek L. Factor XIII: novel structural and functional aspects. J Thromb Haemost 2011; 9: 9-20. Epub 2010/10/01.
3. Inbal A, Lubetsky A, Krapp T et al. Impaired wound healing in factor XIII deficient mice. Thromb Haemost 2005; 94: 432-7. Epub 2005/08/23.
4. Dardik R, Loscalzo J, Inbal A. Factor XIII (FXIII) and angiogenesis. J Thromb Haemost 2006; 4: 19-25. Epub 2005/09/01.
5. Inbal A, Muszbek L. Coagulation factor deficiencies and pregnancy loss. Semin Thromb Hemost 2003; 29: 171-4. Epub 2003/04/24.
6. Kohler HPIA, Seitz R, Ariens AS, Muszbek L. On behalf of the factor XIII and Fibrinogen SSC Subcommittee of the ISTH diagnosis and classification of factor XIII deficiencies. J Thromb Haemost 2011; 9: 1404-6.
7. Ichinose A. Pathophysiology and regulation of factor XIII. Thromb Haemost 2001; 86: 57-65.
8. Ajzner E, Schlammadinger A, Kerenyi A et al. Severe bleeding complications caused by an autoantibody against the B subunit of plasma factor XIII: a novel form of acquired factor XIII deficiency. Blood 2009; 113: 723-5. Epub 2008/10/29.
9. Lorand L, Graham RM. Transglutaminases: crosslinking enzymes with pleiotropic functions. Nat Rev Mol Cell Biol 2003; 4: 140-56. Epub 2003/02/04.
10. Lorand L. Hemorrhagic Disorders of Fibrin Stabilization. London: John Wiley & Sons Ltd, 1977.
11. Tosetto A, Rodeghiero F, Gatto E, Manotti C, Poli T. An acquired hemorrhagic disorder of fibrin crosslinking due to IgG antibodies to FXIII, successfully treated with FXIII replacement and cyclophosphamide. Am J Hematol 1995; 48: 34-9. Epub 1995/01/01.
12. Standen G, Birchall J, Morse C, Chamberlain-Webber R. A large bruise. Lancet 1999; 353: 1934. Epub 1999/06/17.
13. Shires L, Gomperts ED, Bradlow BA. An acquired inhibitor to factor XIII A case report. S Afr Med J 1979; 56: 70-2. Epub 1979/07/14.
14. Seiving BHP, Stenberg P, Nilsson IM. A reversed activity staining procedure for detection of an acquired antibody against factor XIII in a girl with factor XIII deficiency. Br J Haematol 1992; 82: 414-6.
15. Rosenberg RD, Colman RW, Lorand L. A new haemorrhagic disorder with defective fibrin stabilization and cryofibrinogenaemia. Br J Haematol 1974; 26: 269-84. Epub 1974/02/01.
16. Ragaz SKG, Furlan M, Beck EA. Bleeding disorder with abnormal wound healing, acid-soluble clots and normal factor XIII. Thromb Haemost 1976; 36: 537-41.
17. Petri M, Ellman L, Carey R. Acquired factor XIII deficiency with chronic myelomonocytic leukemia. Ann Intern Med 1983; 99: 638-9. Epub 1983/11/01.
18. Otis PT, Feinstein DI, Rapaport SI, Patch MJ. An acquired inhibitor of fibrin stabilization associated with isoniazid therapy: clinical and biochemical observations. Blood 1974; 44: 771-81. Epub 1974/12/01.
19. Nijenhuis AV, van Bergeijk L, Huijgens PC, Zweegman S. Acquired factor XIII deficiency due to an inhibitor: a case report and review of the literature. Haematologica 2004; 89: ECR14. Epub 2004/05/12.
20. Nakazawa MIR, Tanoue K, Arai M. Successful gastrectomy in a patient with inhibitor activities to factor XIII and phospholipid-mediated clotting time [abstract]. Thromb Haemost 1997; 35: PS1-137.
21. Nakamura S, Kato A, Sakata Y, Aoki N. Bleeding tendency caused by IgG inhibitor to factor XIII, treated successfully by cyclophosphamide. Br J Haematol 1988; 68: 313-9. Epub 1988/03/01.
22. Milner GR, Holt PJ, Bottomley J, Maciver JE. Practolol therapy associated with a systemic lupus erythematosus-like syndrome and an inhibitor to factor XIII. J Clin Pathol 1977; 30: 770-3. Epub 1977/08/01.
23. Miesbach WBM, von Auer CH, Scharrer I eds. Case report of a FXIII inhibitor in a 77-year-old patient. 34th Hemophilia Symposium, Heidelberg: Springer Medizin Verlag, 2004.
24. McDevitt NB, McDonagh J, Taylor HL, Roberts HR. An acquired inhibitor to factor XIII. Arch Intern Med 1972; 130: 772-7. Epub 1972/11/01.
25. Luo Y, Zhang G, Zuo W, Zheng W, Dai C. Acquired factor XIII inhibitor in monoclonal gammopathy of undetermined significance: characterization and cross-linked fibrin ultrastructure. Ann Hematol 2010; 89: 833-4. Epub 2009/12/03.
26. Lorand L, Velasco PT, Rinne JR, Amare M, Miller LK, Zucker ML. Autoimmune antibody (IgG Kansas) against the fibrin stabilizing factor (factor XIII) system. Proc Natl Acad Sci U S A 1988; 85: 232-6. Epub 1988/01/01.
27. Lorand L, Velasco PT, Murthy SN, Lefebvre P, Green D. Autoimmune antibody in a hemorrhagic patient interacts with thrombin-activated factor XIII in a unique manner. Blood 1999; 93: 909-17. Epub 1999/01/28.
28. Lorand L, Velasco PT, Hill JM, Hoffmeister KJ, Kaye FJ. Intracranial hemorrhage in systemic lupus erythematosus associated with an autoantibody against actor XIII. Thromb Haemost 2002; 88: 919-23. Epub 2003/01/17.
29. Lorand L, Maldonado N, Fradera J, Atencio AC, Robertson B, Urayama T. Haemorrhagic syndrome of autoimmune origin with a specific inhibitor against fibrin stabilizing factor (factor XIII). Br J Haematol 1972; 23: 17-27. Epub 1972/07/01.
30. Lopaciuk S, Bykowska K, McDonagh JM et al. Difference between type I autoimmune inhibitors of fibrin stabilization in two patients with severe hemorrhagic disorder. J Clin Invest 1978; 61: 1196-203. Epub 1978/05/01.
31. Lim W, Moffat K, Hayward CP. Prophylactic and perioperative replacement therapy for acquired factor XIII deficiency. J Thromb Haemost 2004; 2: 1017-9. Epub 2004/05/14.
32. Lewis JHSI, Ellis LD, Bayer WL. An acquired inhibitor to coagulation factor 13. Johns Hopkins Med J 1967; 120: 401-7.
33. Krumdieck R, Shaw DR, Huang ST, Poon MC, Rustagi PK. Hemorrhagic disorder due to an isoniazid-associated acquired factor XIII inhibitor in a patient with Waldenstrom's macroglobulinemia. Am J Med 1991; 90: 639-45. Epub 1991/05/01.
34. Heriksson PMJ, Villa M. Type I autoimmune inhibitor of factor XIII in a patient with congenital factor XIII deficiency. Thromb Haemost 1983; 50: 272.
35. Gregory TF, Cooper B. Case report of an acquired factor XIII inhibitor: diagnosis and management. Proc (Bayl Univ Med Cent) 2006; 19: 221-3. Epub 2007/01/26.
36. Graham JE, Yount WJ, Roberts HR. Immunochemical characterization of a human antibody to factor XIII. Blood 1973; 41: 661-9. Epub 1973/05/01.
37. Godal HC. An inhibitor to fibrin stabilizing factor (FSF, factor XIII). Scand J Haematol 1970; 7: 43-8. Epub 1970/01/01.
38. Gailani D. An IgG inhibitor against coagulation factor XIII: resolution of bleeding after plasma immunoadsorption with staphylococcal protein A. Am J Med 1992; 92: 110-2. Epub 1992/01/01.
39. Fukue H, Anderson K, McPhedran P, Clyne L, McDonagh J. A unique factor XIII inhibitor to a fibrin-binding site on factor XIIIA. Blood 1992; 79: 65-74. Epub 1992/01/01.
40. Fear JD, Miloszewski KJ, Losowsky MS. An acquired inhibitor of factor XIII with a qualitative abnormality of fibrin cross-linking. Acta Haematol 1984; 71: 304-9. Epub 1984/01/01.
41. Daly HM, Carson PJ, Smith JK. Intracerebral haemorrhage due to acquired factor XIII inhibitor-successful response to factor XIII concentrate. Blood Coagul Fibrinolysis 1991; 2: 507-14. Epub 1991/08/01.
42. Ahmad F, Solymoss S, Poon MC, Berube C, Sullivan AK. Characterization of an acquired IgG inhibitor of coagulation factor XIII in a patient with systemic lupus erythematosus. Br J Haematol 1996; 93: 700-3. Epub 1996/06/01.
43. Ishida F, Okubo K. Spontaneous regression of the inhibitor against the coagulation factor XIII A subunit in acquired factor XIII deficiency. Thromb Haemost 2010; 104: 1284-5.
44. Matsuoka MMT, Onodera T, Ieko M et al. Hemorrhagic-acquired factor XIII deficiency associated with tocilizumab for treatment of rheumatoid arthritis. Int J Hematol 2012; 96: 781-5. Epub Oct 16.
45. Lodemann PKH, Held T, Ivaskevicius V et al. Acquired deficiency of coagulation factor XIII - possible evidence for a new link between coagulation and infection from a case. Ann Hematol 2013; 92: 427-9. Epub Sept 28.
46. Franchini MFF, Crestani S, Bonfanti C. Acquired FXIII inhibitors: a systemic review. J Thromb Thrombolysis 2012. Epub Oct 14.
47. Ichinose A, Souri M. As many as 12 cases with haemorrhagic acquired factor XIII deficiency due to its inhibitors were recently found in Japan. Thromb Haemost 2011; 105: 925-7.
48. Hsieh L, Nugent D. Factor XIII deficiency. Haemophilia 2008; 14: 1190-200. Epub 2009/01/15.
49. Jennings I, Kitchen S, Woods TA, Preston FE. Problems relating to the laboratory diagnosis of factor XIII deficiency: a UK NEQAS study. J Thromb Haemost 2003; 1: 2603-8. Epub 2003/12/17.
50. Bolton-Maggs PHBFEJ, Hillarp A, Jennings I, Kohler HP. Difficulties and pitfalls in teh laboratory diagnosis of bleeding disorders. Haemophilia 2012; 18(Suppl. 4): 66-72.
51. Lawrie AS, Green L, Mackie IJ, Liesner R, Machin SJ, Peyvandi F. Factor XIII-an under diagnosed deficiency-are we using the right assays? J Thromb Haemost 2010; 8: 2478-82. Epub 2010/08/24.
52. Ajzner EML. Kinetic spectrophotometric factor XIII activity assays: the subtraction of plasma blank is not omissible. J Thromb Haemost 2004; 2: 2075-7.
53. HF Bunn JA. Pathophysiology of Blood Disorders. New York: The McGraw Hill Companies Inc., 2011: 342.
54. Nielsen VG, Gurley WQ Jr, Burch TM. The impact of factor XIII on coagulation kinetics and clot strength determined by thrombelastography. Anesth Analg 2004; 99: 120-3. Epub 2004/07/30.
55. Lusher J, Pipe SW, Alexander S, Nugent D. Prophylactic therapy with Fibrogammin P is associated with a decreased incidence of bleeding episodes: a retrospective study. Haemophilia 2010; 16: 316-21. Epub 2009/12/19.
56. Inbal AOJ, Carcao M, Rosholm A, Tehranchi R, Nugent D. Recombinant factor XIII: a safe and novel treatment for congenital factor XIII deficiency. Blood 2012; 119: 5111-7.
57. Klamroth RWS, Kuhlman M, Gottstein S, Oldenburg J eds. Case report of an acquired inhibitor against Factor XIII. 37th Hemophillia Symposium, Berlin, Heidelberg: Springer-Verlag, 2008.
58. Fardella PCG, Gonzalez N, Cuneo M. An acquired inhibitor to factor XIII (case report). Blood 1999; 94: 95b.
59. Nugent DJ. Prophylaxis in rare coagulation disorders - factor XIII deficiency. Thromb Res 2006; 118(Suppl. 1): S23-8. Epub 2006/04/18.
60. Anwar R, Miloszewski KJ. Factor XIII deficiency. Br J Haematol 1999; 107: 468-84. Epub 1999/12/03.
61. Rivard GE, St Louis J, Lacroix S, Champagne M, Rock G. Immunoadsorption for coagulation factor inhibitors: a retrospective critical appraisal of 10 consecutive cases from a single institution. Haemophilia 2003; 9: 711-6. Epub 2004/01/31.