How to avoid the mistake in diagnosing incipient critical disorder of haemostasis in an out-patient clinic

Česká verzia

Authors: P. Kessler
Authors place of work: Oddělení hematologie a transfuziologie Nemocnice Pelhřimov, p. o., přednosta prim. MUDr. Petr Kessler
Published in the journal: Vnitř Lék 2012; 58(7 a 8): 141-145
Category: 60th Birthday prof. MUDr. Miroslav Penka, CSc.

Summary

Critical disorders of haemostasis occur mostly in hospitalized patients; they are usually associated with a serious illness. However, a patient with an early phase of such disorder can visit the office of the general practitioner, hematologist or internist. Every patient with newly emerging bleeding symptoms should be thoroughly investigated with the aim of early detecting of developing critical disorder of haemostasis. The disseminated intravascular coagulation, microangiopathic hemolytic anemia (thrombotic thrombocytopenic purpura, hemolytic-uremic syndrome, pre-eclampsia, HELLP syndrome and catastrophic antiphospholipid syndrome), acute leukemia and heparin induced thrombocytopenia are the most important emergent haemostasis disturbances. In patients suffering from these disorders the bleeding tendency is often combined with thrombotic process affecting dominantly the microcirculation. Severe bleeding can be caused also by overdose of anticoagulants or by acquired inhibitors of coagulation factors.

Key words:
bleeding – thrombosis – microangiopathic hemolytic anemia – acute leukemia – heparin induced thrombocytopenia

Zdroje

1. Rodeghiero F, Stasi R, Gernsheimer T et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood 2009; 113: 2386–2393.

2. Webert K, Cook RJ, Sigouin CS et al. The risk of bleeding in thrombocytopenic patients with acute myeloid leukemia. Haematologica 2006; 91: 1530–1537.

3. Slichter SJ. Relationship between platelet count and bleeding risk in thrombocytopenic patients. Transfus Med Rev 2004; 18: 153–167.

4. Cines DB, Bussel JB, Liebman HA et al. The ITP syndrome: pathogenic and clinical diversity. Blood 2009; 113: 6511–6521.

5. Cohen YC, Djulbegovic B, Shamai-Lubovitz O et al. The bleeding risk and natural history of idiopathic thrombocytopenic purpura in patients with persistently low platelet counts. Arch Intern Med 2000; 160: 1630–1638.

6. Kitchens CS. Thrombocytopenia and thrombosis in disseminated intravascular coagulation (DIC). Hematology Am Soc Hematol Educ Program 2009; 20: 240–246.

7. Okabayashi T, Hanazaki K. Overwhelming postsplenectomy infection syndrome in adults – a clinically preventable disease. World J Gastroenterol 2008; 14: 176–179.

8. Wagenaar JF, Goris MG, Partiningrum DL et al. Coagulation disorders in patients with severe leptospirosis are associated with severe bleeding and mortality. Trop Med Int Health 2010; 15: 152–159.

9. Moake J. Thrombotic thrombocytopenia purpura (TTP) and other thrombotic microangiopathies. Best Pract Res Clin Haematol 2009; 22: 567–576.

10. Burns ER, Lou Y, Pathak A. Morphologic diagnosis of thrombotic thrombocytopenic purpura. Am J Hematol 2004; 75: 18–21.

11. Tsai HM. Thrombotic thrombocytopenic purpura: a thrombotic disorder caused by ADAMTS13 deficiency. Hematol Oncol Clin North Am 2007; 21: 609–632.

12. Kořístek Z, Žák P. Koagulopatie a diferenciační syndrom: hlavní komplikace úvodní léčby akutní promyelocytární leukemie. Vnitř Lék 2008; 54: 745–750.

13. Warkentin TE. Think of HIT. Hematology Am Soc Hematol Educ Program 2006; 18: 408–414.

14. Warkentin TE, Sheppard JI. Generation of platelet-derived microparticles and procoagulant activity by heparin-induced thrombocytopenia IgG/serum and other IgG platelet agonists: a comparison with standard platelet agonists. Platelets 1999; 10: 319–326.

15. Lubenow N, Kempf R, Eichner A et al. Heparin-Induced Thrombocytopenia: temporal pattern of thrombocytopenia in relation to initial use or re-exposure to heparin. Chest 2002; 122: 37–42.

16. Ortel TL. Heparin-induced thrombocytopenia: when a low platelet count is a mandate for anticoagulation. Hematology Am Soc Hematol Educ Program 2009: 225–232.

17. Cervera R, Bucciarelli S, Plasín MA et al. Catastrophic Antiphospholipid Syndrome(CAPS) Registry Project Group (European Forum On Antiphospholipid Antibodies). Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the “CAPS Registry”. J Autoimmun 2009; 32: 240–245.

18. Shetty S, Bhave M, Ghosh K. Acquired hemophilia A: Diagnosis, aetiology, clinical spectrum and treatment options. Autoimmun Rev 2010; 10: 311–316.

19. Kumar S, Pruthi RK, Nichols WL. Acquired von Willebrand disease. Mayo Clin Proc 2002; 77: 181–187.

20. Wiwanitkit V. Spectrum of bleeding in acquired factor V inhibitor: a summary of 33 cases. Clin Appl Thromb Hemost 2006; 12: 485–488.

21. Thompson CA, Kyle R, Gertz M et al. Systemic AL amyloidosis with acquired factor X deficiency: A study of perioperative bleeding risk and treatment outcomes in 60 patients. Am J Hematol 2010; 85: 171–173.

22. Chrobák L, Rozsíval V, Herout V. Heparinové antikoagulans u pacienta s Wegenerovou granulomatózou. Bratisl Lek Listy 1990; 91: 396–398.

23. Gumulec J, Kessler P, Procházka V et al. Krvácivé komplikace antikoagulační léčby. Vnitř Lék 2009; 55: 277–289.