A wide variety of conditions can present with acute thrombocytopenia, ranging from those that are relatively benign and self-limiting to those that require urgent therapeutic intervention. Initial decision-making relies on a good history and the results of some simple investigations. Although a precise diagnosis of the underlying cause might often not be possible in the acute setting, supportive treatments should be provided to all patients. This article describes a practical approach to the diagnosis and initial management of patients presenting with acute thrombocytopenia, with the aid of a case vignette.

[download-attachments container="div" style="list" display_icon="1" display_count="1" display__size="0"]

References

1. Tan GC, Stalling M, Dennis G, et al. Pseudothrombocytopenia due to platelet clumping: a case report and brief review of the literature. Case Rep Hematol 2016; 2016: 3036476.
2. Nagler M, Keller P, Siegrist D, Alberio L. A case of EDTAdependent pseudothrombocytopenia: simple recognition of an underdiagnosed and misleading phenomenon. BMC Clin Pathol 2014; 14: 19.
3. Arnold DM. Bleeding complications in immune thrombocytopenia. Hematology Am Soc Hematol Educ Program 2015; 2015:237-42.
4. Cohen YC, Djulbegovic B, Shamai-Lubovitz O, Mozes B. The bleeding risk and natural history of idiopathic thrombocytopenic purpura in patients with persistently low platelet counts. Arch Intern Med 2000; 160(11): 1630-8.
5. Neunert C, Noroozi N, Norman G, et al. Severe bleeding events in adults and children with primary immune thrombocytopenia: a systematic review. J Thromb Haemost 2015;13(3):457-64.
6. Velo-Garcia A, Castro SG, Isenberg DA. The diagnosis and management of the haematological manifestations of lupus. J Autoimmun 2016; 74:139-60.
7. Fayyaz A, Igoe A, Kurien BT, et al. Haematological manifestations of lupus. Lupus Sci Med 2015;2(1): e000078.
8. Hepburn AL, Narat S, Mason JC. The management of peripheral blood cytopenias in systemic lupus erythematosus. Rheumatology (Oxford) 2010;49(12):2243-54.
9. Palta A, Dhiman P. Thrombocytopenia in pregnancy. J Obstet Gynaecol 2016;36(2):146-52.
10. Appel GB. Thrombotic microangiopathies: similar presentations, different therapies. Cleve Clin J Med 2017;84(2):114-30.
11. George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med 2014;371(7):654-66.
12. Scully M, Hunt BJ, Benjamin S, et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol 2012;158(3):323-35.
13. Boral BM, Williams DJ, Boral LI. Disseminated intravascular coagulation, Am J Clin Pathol 2016;146(6):670-680. s
14. Lenting PJ, Christophe OD, Denis CV. von Willebrand factor biosynthesis, secretion and clearance: connecting the far ends. Blood 2015;125(13):2019-28.
15. 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. New Engl J Med 1991;325(6):393-7.
16. Nolasko LH, Turner NA, Bernardo A, et al. Hemolytic uremic syndrome-associated Shiga toxins promote endothelial-cell secretion and impair ADAMTS13 cleavage of unusually large von Willebrand factor multimers. Blood 2005;106(13): 4199-209.
17. Laurence J, Haller H, Mannucci PM, et al. Atypical haemolytic uremic syndrome (aHUS): essential aspects of an accurate diagnosis. Clin Adv Hematol Oncol 2016; 14 Suppl 11(11): 2-15.
18. Mitaka H, Yamada Y, Hamada O, et al. Malignant hypertension with thrombotic microangiopathy. Intern Med 2016;55(16):2277-80.
19. Thomas MR, Robinson S, Scully MA. How we manage thrombotic microangiopathies in pregnancy? Br J Haematol 2016;173(6);821-30.
20. Aster RH, Curtis BR, McFarland JG, Bougie DW. Druginduced immune thrombocytopenia: pathogenesis, diagnosis and management. J Thromb Haemost 2009; 7: 911-18.
21. Stasi R. How to approach thrombocytopenia. Hematology Am Soc Hematol Educ Program 2012; 2012:191-7.
22. Salter BS, Weiner MM, Trinh MA, et al. Heparin-induced thrombocytopenia: a comprehensive clinical review. J Am Coll Cardiol 2016;67(21):2519-32.
23. Venkata C, Kashyap R, Farmer JC, Afessa B. Thrombocytopenia in adult patients with sepsis: incidence, risk factors and its association with clinical outcome. J Intensive care 2013; 1(1): 9.
24. Franchini M, Veneri D, Lippi G. Thrombocytopenia and infections. Expert Rev Hematol 2017; 10(1): 99-106.
25. Taylor SM, Molyneux ME, Simel DL, et al. Does this patient have malaria? JAMA 2010;304(18):2048-56.
26. Kularatne SA. Dengue fever. BMJ 2015; 351: h4661. Schram AM, Berliner N. How I treat hemophagocytic lymphohistiocytosis in the adult patient? Blood 2015; 125(9):2908-14.
27. Bradbury C, Murray J. Investigating an incidental finding of thrombocytopenia. BMJ 2013; 346: f11.
28. Altintas A, Ozel A, Okur N, 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.
29. Suresh E. Systemic lupus erythematosus: diagnosis for the nonspecialist. Br J Hosp Med (London) 2007; 68(10): 538-41.
30. Michel M, Chanet V, Dechartres A, et al. The spectrum of Evans syndrome in adults: new insights into the disease based on the analysis of 68 cases. Blood 2009;114(15):3167-72.
31. Ziakas PD, Giannouli S, Zintzaras E, et al. Lupus thrombocytopenia: clinical implications and prognostic significance. Ann Rheum Dis 2005; 64(9): 1366-9.
32. 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(4): 323-39.
33. Miller MH, Urowitz MB, Gladman DD. The significance of thrombocytopenia in systemic lupus erythematosus. Arthritis Rheum 1983; 26(10): 1181-6.
34. Kaufman RM, Djulbegovic B, Gernsheimer T, et al. Platelet transfusion: a clinical practice guideline from the AABB. Ann Intern Med 2015; 162(3): 205-13.
35. Desborough MJ, Smethurst PA, Estcourt LJ, Stanworth SJ. Alternatives to allogenic platelet transfusion. Br J Haematol 2016; 175(3): 381-92.
36. Ortel TL, Erkan D, Kitchens CS. How I treat catastrophic thrombotic syndromes? Blood 2015;126(11):1285-93.
37. George JN. How I treat patients with thrombotic thrombocytopenic purpura: 2010. Blood 2010; 116(20): 4060-9.
38. Scully M, Goodship T. How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemia syndrome? Br J Haematol 2014;164(6):759-66.
39. Clark WF, Hildebrand A. Attending rounds: microangiopathic haemolytic anaemia with renal insufficiency. J Am Soc Nephrol 2012; 7(2): 342-7.
40. Levi M, Toh CH, Thachil J, Watson HG. Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology. Br J Haematol 2009; 145(1): 24-33.
41. Neunert CE. Current management of immune thrombocytopenia. Hematology Am Soc Hematol Educ Program 2013; 2013: 276-82.