Case 37-2010: A 16-year-old girl with confusion, anemia, and thrombocytopenia

New England Journal of Medicine

Volumn 363, Issue 24, 2010, Pages 2352-2361

  Binder, William D ^a,b   Traum, Avram Z ^a,b   Makar, Robert S ^a,b   Colvin, Robert B ^a,b  

^a MASSACHUSETTS GENERAL HOSPITAL   (United States)

^b HARVARD MEDICAL SCHOOL   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

AUTOANTIBODY; CREATINE KINASE; CREATINE KINASE ISOENZYME; ENALAPRIL; ERGOCALCIFEROL; HYDROMORPHONE; METOCLOPRAMIDE; MYCOPHENOLIC ACID 2 MORPHOLINOETHYL ESTER; NORETHISTERONE; ONDANSETRON; PREDNISONE; RITUXIMAB; TROPONIN I; UNCLASSIFIED DRUG; VON WILLEBRAND FACTOR CLEAVING PROTEINASE; VON WILLEBRAND FACTOR CLEAVING PROTEINASE ANTIBODY;

ADOLESCENT; AGITATION; ANEMIA; ARTICLE; CASE REPORT; CONFUSION; CREATINE KINASE BLOOD LEVEL; DIFFERENTIAL DIAGNOSIS; DIZZINESS; DRUG DOSE REDUCTION; DYSPNEA; FATIGUE; FEMALE; HEADACHE; HEART PALPITATION; HUMAN; HYPERTENSION; KIDNEY BIOPSY; LUPUS ERYTHEMATOSUS NEPHRITIS; MALAISE; MENTAL DISEASE; NAUSEA AND VOMITING; PARESTHESIA; PLASMAPHERESIS; PRIORITY JOURNAL; PROTEINURIA; THROMBOTIC THROMBOCYTOPENIC PURPURA; BLOOD; CENTRAL NERVOUS SYSTEM DISEASE; CLINICAL OBSERVATION; CLINICAL STUDY; DIAGNOSIS; DIAGNOSIS, MEASUREMENT AND ANALYSIS; KIDNEY; PATHOLOGY; THROMBOCYTOPENIA;

ADOLESCENT; ANEMIA; AUTOANTIBODIES; CENTRAL NERVOUS SYSTEM DISEASES; CONFUSION; DIAGNOSIS, DIFFERENTIAL; FEMALE; HUMANS; KIDNEY; LABORATORY TECHNIQUES AND PROCEDURES; LUPUS NEPHRITIS; PLASMA EXCHANGE; PURPURA, THROMBOTIC THROMBOCYTOPENIC; THROMBOCYTOPENIA;

EID: 78649895285     PISSN: 00284793     EISSN: 15334406     Source Type: Journal    
DOI: 10.1056/NEJMcpc0910929     Document Type: Article

References (38)

1. Goldstein JN, Greer DM. Rapid focused neurological assessment in the emergency department and ICU. Emerg Med Clin North Am 2009;27:1-16.
2. Lehman RK, Mink J. Altered mental status. Clin Pediatr Emerg Med 2008;9:68-75.
3. Cuchacovich R, Gedalia A. Pathophysiology and clinical spectrum of infections in systemic lupus erythematosus. Rheum Dis Clin North Am 2009;35:75-93.
4. Chávez-Bueno S, McCracken GH Jr. Bacterial meningitis in children. Pediatr Clin North Am 2005;52:795-810.
5. Muscal E, Brey RL. Neurologic manifestations of systemic lupus erythematosus in children and adults. Neurol Clin 2010;28:61-73.
6. Greenberg BM. The neurologic manifestations of systemic lupus erythematosus. Neurologist 2009;15:115-21.
7. Marcus N, Scheuerman O, Hoffer V, Zilbershot-Fink E, Reiter J, Garty BZ. Stupor in an adolescent following Yom Kippur fast, due to late-onset ornithine transcarbamylase deficiency. Isr Med Assoc J 2008;10:395-6.
8. Ziakas PD, Giannouli S, Zintzaras E, Tzioufas AG, Voulgarelis M. Lupus thrombocytopenia: clinical implications and prognostic significance. Ann Rheum Dis 2005;64:1366-9.
9. Schmugge M, Revel-Vilk S, Hiraki L, Rand ML, Blanchette VS, Silverman ED. Thrombocytopenia and thromboembolism in pediatric systemic lupus erythematosus. J Pediatr 2003;143:666-9.
10. Benseler SM, Silverman ED. Systemic lupus erythematosus. Rheum Dis Clin North Am 2007;33:471-98.
11. Jaffey PB, Feldman HA. The clinical spectrum of thrombotic thrombocytopenic purpura. Emerg Med 2005;37:36-44.
12. Weening JJ, D'Agati VD, Schwartz MM, et al. The classification of glomerulonephritis in systemic lupus erythematosus revisited. Kidney Int 2004;65:521-30.
13. Glassock RJ. Prophylactic anticoagulation in nephrotic syndrome: a clinical conundrum. J Am Soc Nephrol 2007;18:2221-5.
14. Terrell DR, Williams LA, Vesely SK, Lämmle B, Hovinga JA, George JN. The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency. J Thromb Haemost 2005;3:1432-6.
15. Coppo P, Bengoufa D, Veyradier A, et al. Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement. Medicine (Baltimore) 2004;83:233-44.
16. Vesely SK, George JN, Lammle B, et al. ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood 2003;102:60-8.
17. Tsai HM. The molecular biology of thrombotic microangiopathy. Kidney Int 2006;70:16-23.
18. Furlan M, Robles R, Solenthaler M, Wassmer M, Sandoz P, Lammle B. Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. Blood 1997;89:3097-103.
19. 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.
20. Furlan M, Robles R, Solenthaler M, Lämmle B. Acquired deficiency of von Willebrand factor-cleaving protease in a patient with thrombotic thrombocytopenic purpura. Blood 1998;91:2839-46.
21. Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 1998;339:1585-94.
22. Sadler JE. Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura. Blood 2008;112:11-8.
23. Moake JL. Thrombotic microangiopathies. N Engl J Med 2002;347:589-600.
24. George JN. Thrombotic thrombocytopenic purpura. N Engl J Med 2006;354:1927-35.
25. Rock GA, Shumak KH, Buskard NA, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. N Engl J Med 1991;325:393-7.
26. Swisher KK, Terrell DR, Vesely SK, Kremer Hovinga JA, Lämmle B, George JN. Clinical outcomes after platelet transfusions in patients with thrombotic thrombocytopenic purpura. Transfusion 2009;49:873-87.
27. Allford SL, Hunt BJ, Rose P, Machin SJ. Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias. Br J Haematol 2003;120:556-73.
28. George JN. How I treat patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Blood 2000; 96:1223-9.
29. Zheng XL, Kaufman RM, Goodnough LT, Sadler JE. Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura. Blood 2004;103:4043-9.
30. Tsai HM, Li A, Rock G. Inhibitors of von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura. Clin Lab 2001;47:387-92.
31. Kokame K, Nobe Y, Kokubo Y, Okayama A, Miyata T. FRETS-VWF73, a first fluorogenic substrate for ADAMTS13 assay. Br J Haematol 2005;129:93-100.
32. Chemnitz J, Draube A, Scheid C, et al. Successful treatment of severe thrombotic thrombocytopenic purpura with the monoclonal antibody rituximab. Am J Hematol 2002;71:105-8.
33. Galbusera M, Bresin E, Noris M, et al. Rituximab prevents recurrence of thrombotic thrombocytopenic purpura: a case report. Blood 2005;106:925-8.
34. Zheng X, Pallera AM, Goodnough LT, Sadler JE, Blinder MA. Remission of chronic thrombotic thrombocytopenic purpura after treatment with cyclophosphamide and rituximab. Ann Intern Med 2003;138:105-8.
35. Bennett CL, Kim B, Zakarija A, et al. Two mechanistic pathways for thienopyridine-associated thrombotic thrombocytopenic purpura: a report from the SERF-TTP Research Group and the RADAR Project. J Am Coll Cardiol 2007;50:1138-43.
36. Bell WR, Braine HG, Ness PM, Kickler TS. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome - clinical experience in 108 patients. N Engl J Med 1991;325:398-403.
37. Harkness DR, Byrnes JJ, Lian EC, Williams WD, Hensley GT. Hazard of platelet transfusion in thrombotic thrombocytopenic purpura. JAMA 1981;246:1931-3.
38. Ridolfi RL, Bell WR. Thrombotic thrombocytopenic purpura: report of 25 cases and review of the literature. Medicine (Baltimore) 1981;60:413-28.