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TTP-HUS Publications

1. These articles describe The Oklahoma TTP-HUS Registry:

George JN, Vesely SK, Terrell DR: The Oklahoma thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) registry: a community perspective of patients with clinically diagnosed TTP-HUS. Seminars in Hematol 41: 60-67, 2004

George JN: The Tibor Greenwalt Award Lecture. The Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome Registry: a program for patient care, education, and research. Transfusion 44: 1384-1392, 2004. [Full Text]

Johnson KK, Duvall D, Gilcher RO, Smith JW, Forsberg JE, Terrell DR, Vesely SK, George JN: The Oklahoma thrombotic thrombocytopenic purpura-hemolytic uremic syndrome registry: a community service. The Journal of the Oklahoma State Medical Association 100: 273-278, 2007.

Johnson KK, Terrell DR, Vesely SK, George JN: The Oklahoma thrombotic thrombocytopenic purpura-hemolytic uremic syndrome registry: a resource for medical education. The Journal of the Oklahoma State Medical Association 100: 309-314, 2007.

George JN, Kremer Hovinga JA, Terrell DR, Vesely SK, Lammle B:  The Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome Registry: the Swiss connection.  European J Haematol 80: 277-286, 2008. [Full Text]

George JN, Terrell DR, Swisher KK, Vesely SK:  Lessons learned from The Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome Registry.  J Clin Apheresis 23: 129-137, 2008. 

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George JN: Evaluation and management of the thrombotic thrombocytopenic purpura and hemolytic uremic syndromes. Experience of The Oklahoma TTP-HUS Registry, 1989-2007. Kidney International 75: S8-S10, 2009. [Full Text]

George JN: Overview of the pathogenesis of the thrombotic thrombocytopenic purpura and hemolytic uremic syndromes. Experience of The Oklahoma TTP-HUS Registry, 1989-2007. Kidney International 75: S52-S54, 2009. [Full Text]

Kremer Hovinga JA, Vesely SK, Terrell DR, Lämmle B, George JN: Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood 115: 1500-1511, 2010. [Full Text]

2. These articles describe the patient support group organized by The Oklahoma TTP-HUS Registry. This group has met 3 times every year for 15 years. Its continuing success documents its benefit for our patients.

Howard MA, Duvall D, Terrell DR, Christopher A, Thomas I, Holloway N, Vesely SK, George JN: A support group for patients who have recovered from thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: the six year experience of the Oklahoma TTP-HUS Study Group. J Clin Apheresis 18: 16-20, 2003. [Full Text]

Ambadwar P, Duvall D, Wolf NJ, Terrell DR, Vesely SK, George JN: Support groups for patients who have recovered from thrombotic thrombocytopenic purpura. J Clin Apheresis 23: 168-169, 2008. [Full Text]

3. These articles describe the incidence of TTP-HUS in Oklahoma, standardized for the US population. There are multiple gender and race disparities among the different clinical categories of TTP-HUS.

Terrell DR, Williams LA, Vesely SK, Lammle B, Kremer Hovinga JA, George JN: the incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS13 deficiency. J Thromb Haemost 3: 1432-1436, 2005. [Full Text]

Terrell DR, Vesely SK, Kremer Hovinga JA, Lämmle B, George JN: Distinct disparities of gender and race among the thrombotic thrombocytopenic purpura and hemolytic-uremic syndromes. Am J Hematol 85(11): 844-847, 2010. [Full Text]

4. A summary of current understanding and treatment of TTP-HUS

George JN: How I treat thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS). Blood 96: 1223-1229, 2000. [Full Text]

George JN: Thrombotic thrombocytopenic purpura. New Eng J Med 354: 1927-1935, 2006. [Full Text]

George JN:  Clinical insights from observations on ADAMTS13 deficiency in liver disease.  Thrombosis and Haemostasis 99: 987-988, 2008. [Full Text]

Lammle B, Kremer Hovinga JA, George JN:  Acquired thrombotic thrombocytopenic purpura: ADAMTS13 activity, anti-ADAMTS13 autoantibodies and risk of recurrent disease.  Haematologica 93: 172-177, 2008. [Full Text]

George JN: How I treat patients with thrombotic thrombocytopenic purpura: 2010. Blood 116(20): 4060-4069, 2010. [Full Text]

5. Although plasma exchange is essential treatment for patients with TTP-HUS, the risk for serious complication is great.

Rizvi MA, Vesely SK, George JN, Chandler L, Duvall D, Smith JN, Gilcher RO: Plasma exchange complications in 71 consecutive patients treated for clinically suspected thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Transfusion 40:896-901, 2000. [Full Text]

McMinn JR, Thomas IA, Terrell DR, Duvall D, Vesely SK, George JN: Complications of plasma exchange in TTP-HUS: a study of 78 additional patients. Transfusion 43: 415-416, 2003. [Full Text]

Howard MA, Williams LA, Terrell DR, Duvall D, Vesely SK, George JN: Complications of plasma exchange in patients treated for clinically suspected thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. III. An additional study of 54 consecutive patients, 2002-2005. Transfusion 46: 154-156, 2006. [Full Text]

Nguyen L, Swisher KK, Terrell DR, Duvall D, Vesely SK, George JN: Complications of plasma exchange in patients treated for clinically suspected thrombotic thrombocytopenic purpura-hemolytic uremic syndrome IV. An additional study of 43 consecutive patients, 2005-2008. Transfusion 49: 392-394, 2009. [Full Text]

6. A complication of plasma exchange treatment for TTP-HUS that is often unrecognized is platelet loss caused by removal of platelets by the apheresis instrument.

Perdue JJ, Chandler LK, Vesely SK, Duvall DS, Gilcher RO, Smith JW, George JN: Unintentional platelet removal by plasmapheresis. J Clin Apheresis 16: 55-60, 2001. [Full Text]

7. Pregnancy is a risk factor for provoking acute episodes of TTP-HUS. Most patients with TTP-HUS are women, and in many women TTP-HUS occurs during pregnancy. The period of greater risk appears to be near delivery or soon after delivery. Although there is a risk that pregnancy may provoke a recurrence of TTP-HUS, most pregnancies following recovery from TTP-HUS are uncomplicated. TTP-HUS is difficult to distinguish from the more common complications of pregnancy, such as preeclampsia.

George JN: The association of pregnancy with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Current Opinion Hematol 10: 339-344, 2003. [Full Text]

Vesely SK, Li X, McMinn JR, Terrell DR, George JN: Pregnancy outcomes after recovery from thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Transfusion 44: 1149-1158, 2004. [Full Text]

McMinn JR, George JN: Evaluation of women with clinically suspected thrombotic thrombocytopenic purpura-hemolytic uremic syndrome during pregnancy. J Clnical Apheresis 16: 202-209, 2001. [Full Text]

8. Although there are descriptions suggesting that TTP-HUS may be caused by bone marrow transplantation, most patients who have been diagnosed with TTP-HUS may not actually have had TTP-HUS. Serious infections may cause all of the clinical features of TTP-HUS.  A specific entity of transplantation-associated thrombotic microangiopathy (TA-TMA) may exist, distinct from TTP-HUS.

Roy V, Rizvi MA, Vesely SK, George JN: Thrombotic thrombocytopenic purpura-like syndromes following bone marrow transplantation: an analysis of associated conditions and clinical outcomes. Bone Marrow Transplantation 27: 641-646, 2001. [Full Text]

George JN, Li X, McMinn JR, Terrell DR, Vesely SK, Selby GB: Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome following allogeneic hematopoietic stem cell transplantation: a diagnostic dilemma. Transfusion 44: 294-304, 2004. [Full Text]

Kojouri K, George JN: Thrombotic microangiopathy following allogeneic hematopoietic stem cell transplantation. Current Opinion in Oncology 19: 148-154, 2007. [Full Text]

Siami K, Kojouri K, Swisher KK, Selby GB, George JN, Laszik ZG:  Thrombotic microangiopathy after allogeneic hematopietic stem cell transplantation.  An autopsy study.  Transplantation 85: 22-28, 2008. [Text unavailable]

George JN:  Hematopoietic stem cell transplantation-associated thrombotic microangiopathy: defining a disorder.  Bone Marrow Transplantation 41: 917-918, 2008. [Full Text]

9. Widespread cancer can mimic all of the signs of TTP-HUS.

Francis KK, Kojouri K, George JN: Occult systemic carcinoma masquerading as thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Community Oncology 2: 339-343, 2005.

Francis KK, Kalyanam N, Terrell DR, Vesely SK, George JN. Disseminated malignancy misdiagnosed as thrombotic thrombocytopenic purpura: A report of 10 patients and a systematic review of published cases. The Oncologist 12: 11-19, 2007 [Full Text]

10. Quinine is the most common cause of drug-induced TTP-HUS. Quinine can also cause many other problems, such as liver toxicity, that can make the diagnosis difficult. The severity of quinine-induced TTP-HUS and the difficult diagnosis are emphasized her in “Melissa’s Story”

Kojouri K, Vesely SK, George JN: Quinine-associated thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: frequency, clinical features, and long-term outcomes. Ann Int Med 135: 1047-1051. [Full Text]

Howard MA, Hibbard AB, Terrell DR, Medina PJ, Vesely SK, George JN: Quinine allergy causing acute severe systemic illness: report of four patients manifesting hematologic, renal and hepatic abnormalities. Baylor University Medical Center Proceedings 16: 21-26, 2003.

11. Although the deficiency of a plasma enzyme, termed ADAMTS13, can contribute to the cause of TTP-HUS, many patients do not have a severe deficiency of this enzyme.

Vesely SK, George JN, Lammle B, Studt J-D, Alberio L, El-Harake MA, Raskob GE: ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood 102: 60-68, 2003. [Full Text]

12. Because drugs that suppress the immune system, such as steroids and rituximab, may be effective treatment for TTP, in addition to plasma exchange, we are participating in a national study of rituximab.

George JN, Woodson RD, Kiss J, Kojouri K, Vesely SK: Rituximab therapy for thrombotic thrombocytopenic purpura: a proposed study of the Transfusion Medicine/Hemostasis Clinical Trials Network with a systematic review of rituximab therapy for immune-mediated disorders. J Clin Apheresis 21: 49-56, 2006. [Full Text]

13.  Although studies of patients who have died from TTP always demonstrate extensive involvement of the heart with thrombosis of small blood vessels, recognition of heart involvement in patients with TTP who survive is uncommonly reported.  But heart involvement may not be uncommon - it may be merely unrecognized.  We have begun systematic analysis of heart function and evidence for heart vessel thrombi in the patients of the Oklahoma Registry.

Hawkins B, Abu-Fadel M, Vesely SK, George JN: Clinical cardiac involvement in thrombotic thrombocytopenic purpura: a systematic review.  Transfusion 48: 382-392, 2008.

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14.  In patients who have critical problems with TTP and who do not respond to the usual once-daily plasma exchange treatments, twice-daily plasma exchange by be effective.

Nguyen L, Li X, Duvall D, Terrell DR, Vesely SK, George JN. Twice-daily plasma exchange for patients with refractory thrombotic thrombocytopenic purpura: the experience of the Oklahoma Registry, 1989 through 2006. Transfusion 48: 349-357, 2008. 

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15.  TTP associated with an acquired severe deficiency of ADAMTS13 activity may have similarities with other autoimmune disorders, notably systemic lupus erythematosus.

George JN, Vesely SK, James JA:  Overlapping features of thrombotic thrombocytopenic purpura and systemic lupus erythematosus. Southern Med J 100: 512-514, 2007. [Full Text]

16.  The TTP-HUS syndrome caused by E. coli O157:H7, typically from under cooked hamburger, is the typical HUS of young children that occurs following diarrhea.  This occurs much less commonly among adults, but when it occurs, it is much more severe.

Karpac CA, Li X, Terrell DR, Kremer Hovinga JA, Lammle B, Vesely SK, George JN:  Sporadic bloody diarrhoea-associated thrombotic thrombocytopenic purpura-haemolytic uraemic syndrome: an adult and paediatric comparison.  Brit J Haematol 141: 696-707, 2008.

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17.  Congenital TTP cause by a deficiency of ADAMTS13, an abnormality that must be inherited from both the mother and the father of the patient, is very rare.  We have not recognized a patient with congenital TTP in 19 years of the Oklahoma Registry.  But for these rare patients, careful observation and treatment with plasma infusions is effective treatment.

George JN: Congenital thrombotic thrombocytopenic purpura: Lessons for recognition and management of rare syndromes.  Pediatric Blood & Cancer 50: 947-948, 2008. [Full Text]

18. There are many publications about TTP-HUS and HIV infections. Some publications have suggested that HIV infection can cause TTP-HUS. But this is not our experience. In most patients, HIV-related complications merely mimic the clinical features of TTP-HUS. In some patients, it is merely an incidental additional disorder.

Benjamin M, Terrell DR, Vesely SK, Voskuhl G, Dezube BJ, Kremer Hovinga JA, Lämmle B, George JN: Frequency and significance of human immunodeficiency virus infection in patients diagnosed with thrombotic thrombocytopenic purpura. Clinical Infectious Diseases 48: 1129-1137, 2009. [Full Text]

19. For many years platelet transfusions have been thought to be harmful for patients with TTP. However a systematic review of all publications revealed that this dogma was based only on very few anecdotes. But these anecdotes are dramatic and persuasive, and they are consistent with the pathogenesis of platelet thrombi in patients with TTP. Our experience is that most patients with TTP receive platelet transfusions before the diagnosis of TTP is considered, and we could document no harm from platelet transfusions. Therefore we recommend that platelet transfusions are appropriate for patients with TTP who have appropriate indications, such as active hemorrhage or requirement for a surgical procedure.

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 49: 873-887, 2009. [Full Text]

20. As we continue to follow our patients, we have recognized that some patients are not the same after recovering from their acute episode of TTP-HUS. They have difficulties with memory, concentration, and endurance. These problems were first recognized from patient conversations at our support group meetings. We have documented abnormalities of health-related quality-of-life and cognitive ability in our patients that persist for years following recovery.

Lewis QF, Lanneau MS, Mathias SD, Terrell DR, Vesely SK, George JN: Long-term deficits in health-related quality-of-life following recovery from thrombotic thrombocytopenic purpura. Transfusion 49: 118-124, 2009. [Full Text]

Kennedy KA, Lewis QF, Scott J, Kremer Hovinga JA, Lämmle B, Terrell DR, Vesely SK, George JN: Neurocognitive deficits after recovery from thrombotic thrombocytopenic purpura. Transfusion 49: 1092-1101, 2009. [Full Text]

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