Skip to main content
Top
Published in: Journal of Cardiothoracic Surgery 1/2022

Open Access 01-12-2022 | Venous Thrombosis | Case report

Complete recovery of deep venous thrombosis from Coombs (+) thrombotic thrombocytopenic purpura: case report

Authors: Mi Zhou, Jie Yin

Published in: Journal of Cardiothoracic Surgery | Issue 1/2022

Login to get access

Abstract

Background

Acute thrombotic thrombocytopenic purpura (TTP) is an aggressive thrombotic microangiopathy that if not treated, can have a 90% mortality rate. Timely, extensive plasma exchange (PEX) has been indicated to reduce the mortality rate to < 10%, but its side effects are not well-known. We present here a case of a patient presented with Comb (+) TTP and developed catheter-associated deep vein thrombosis (DVT).

Case presentation

A 27-year-young man presented with persistent thrombocytopenia and Coombs positive anemia was firstly diagnosed with Evans syndrome. However, he was refractory to a methylprednisolone pulse therapy with a combination of platelet transfusion and eventually developed microangiopathy of central nerve system. Several pathological manifestations of the disease were prevented by PEX. The immediate start of PEX (1500 mL/d) induced a complete remission of acquired TTP and disappearance of neurological signs and symptoms. However, external iliac and femoro-popliteal venous thrombosis was diagnosed subsequently, inferior vena cava filter (IVC) filter was immediately implanted accompanied with anticoagulation therapy. Meanwhile, PEX session was sustained as well as oral anticoagulant (rivaroxaban). 14 days later, the patient got full recovery.

Conclusions

Catheter-related DVT under the setting of TTP should be cautious. It is necessary to start anticoagulation and antiplatelet therapy for thrombosis early, especially in such cases when PLT count > 50 × 109/L.
Literature
1.
go back to reference George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371:654–66.CrossRef George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371:654–66.CrossRef
2.
go back to reference Chapman K, Seldon M, Richards R. Thrombotic microangiopathies, thrombotic thrombocytopenic purpura, and ADAMTS-13. Semin Thromb Hemost. 2012;38:47–54.CrossRef Chapman K, Seldon M, Richards R. Thrombotic microangiopathies, thrombotic thrombocytopenic purpura, and ADAMTS-13. Semin Thromb Hemost. 2012;38:47–54.CrossRef
3.
go back to reference George JN, Sandler SA, Stankiewicz J. Management of thrombotic thrombocytopenic purpura without plasma exchange: the Jehovah’s Witness experience. Blood Adv. 2017;1:2161–5.CrossRef George JN, Sandler SA, Stankiewicz J. Management of thrombotic thrombocytopenic purpura without plasma exchange: the Jehovah’s Witness experience. Blood Adv. 2017;1:2161–5.CrossRef
4.
go back to reference Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, Peyvandi F, Cheung B, Machin SJ. British Committee for Standards in H: guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012;158:323–35.CrossRef Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, Peyvandi F, Cheung B, Machin SJ. British Committee for Standards in H: guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012;158:323–35.CrossRef
5.
go back to reference Froissart A, Buffet M, Veyradier A, Poullin P, Provot F, Malot S, Schwarzinger M, Galicier L, Vanhille P, Vernant JP, et al. Efficacy and safety of first-line rituximab in severe, acquired thrombotic thrombocytopenic purpura with a suboptimal response to plasma exchange. Experience of the French Thrombotic Microangiopathies Reference Center. Crit Care Med. 2012;40:104–11.CrossRef Froissart A, Buffet M, Veyradier A, Poullin P, Provot F, Malot S, Schwarzinger M, Galicier L, Vanhille P, Vernant JP, et al. Efficacy and safety of first-line rituximab in severe, acquired thrombotic thrombocytopenic purpura with a suboptimal response to plasma exchange. Experience of the French Thrombotic Microangiopathies Reference Center. Crit Care Med. 2012;40:104–11.CrossRef
6.
go back to reference Dimopoulou D, Dimosiari A, Mandala E, Dimitroulas T, Garyfallos A. Autoimmune thrombotic thrombocytopenic purpura: two rare cases associated with juvenile idiopathic arthritis and multiple sclerosis. Front Med (Lausanne). 2017;4:89.CrossRef Dimopoulou D, Dimosiari A, Mandala E, Dimitroulas T, Garyfallos A. Autoimmune thrombotic thrombocytopenic purpura: two rare cases associated with juvenile idiopathic arthritis and multiple sclerosis. Front Med (Lausanne). 2017;4:89.CrossRef
7.
go back to reference Zhang C, Chen XH, Zhang X. Quick development and sudden death: evans syndrome followed by thrombotic thrombocytopenic purpura. Am J Emerg Med. 2014;32(1156):e1153–4. Zhang C, Chen XH, Zhang X. Quick development and sudden death: evans syndrome followed by thrombotic thrombocytopenic purpura. Am J Emerg Med. 2014;32(1156):e1153–4.
8.
go back to reference Page EE, Kremer Hovinga JA, Terrell DR, Vesely SK, George JN. Thrombotic thrombocytopenic purpura: diagnostic criteria, clinical features, and long-term outcomes from 1995 through 2015. Blood Adv. 2017;1:590–600.CrossRef Page EE, Kremer Hovinga JA, Terrell DR, Vesely SK, George JN. Thrombotic thrombocytopenic purpura: diagnostic criteria, clinical features, and long-term outcomes from 1995 through 2015. Blood Adv. 2017;1:590–600.CrossRef
9.
go back to reference 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. Transfusion. 2006;46:154–6.CrossRef 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. Transfusion. 2006;46:154–6.CrossRef
10.
go back to reference Oshima T, Ikutomi M, Shinohara H, Ishiwata J, Fukino K, Amaki T, Nakamura F. Acute myocardial infarction caused by thrombotic microangiopathy complicated with myelodysplastic syndrome. Int Heart J. 2016;57:634–6.CrossRef Oshima T, Ikutomi M, Shinohara H, Ishiwata J, Fukino K, Amaki T, Nakamura F. Acute myocardial infarction caused by thrombotic microangiopathy complicated with myelodysplastic syndrome. Int Heart J. 2016;57:634–6.CrossRef
11.
go back to reference McGee DC, Gould MK. Preventing complications of central venous catheterization. N Engl J Med. 2003;348:1123–33.CrossRef McGee DC, Gould MK. Preventing complications of central venous catheterization. N Engl J Med. 2003;348:1123–33.CrossRef
12.
go back to reference Rottenstreich A, Kalish Y, Tvito A, Hauschner H, Arad A. Acquired thrombotic thrombocytopenic purpura in pregnancy: the role of placental and breast-milk mediated transfer of ADAMTS13-autoantibodies. Thromb Res. 2017;156:80–1.CrossRef Rottenstreich A, Kalish Y, Tvito A, Hauschner H, Arad A. Acquired thrombotic thrombocytopenic purpura in pregnancy: the role of placental and breast-milk mediated transfer of ADAMTS13-autoantibodies. Thromb Res. 2017;156:80–1.CrossRef
13.
go back to reference Nakao H, Ishiguro A, Ikoma N, Nishi K, Su C, Nakadate H, Kubota M, Hayakawa M, Matsumoto M. Acquired idiopathic thrombotic thrombocytopenic purpura successfully treated with intravenous immunoglobulin and glucocorticoid: a case report. Medicine (Baltimore). 2017;96:e6547.CrossRef Nakao H, Ishiguro A, Ikoma N, Nishi K, Su C, Nakadate H, Kubota M, Hayakawa M, Matsumoto M. Acquired idiopathic thrombotic thrombocytopenic purpura successfully treated with intravenous immunoglobulin and glucocorticoid: a case report. Medicine (Baltimore). 2017;96:e6547.CrossRef
14.
go back to reference Jestin M, Benhamou Y, Schelpe A-S, Roose E, Galicier L,Girault S, et al. Rituximab prevents long-term relapses in TTP. Blood. 2018;132:2210.CrossRef Jestin M, Benhamou Y, Schelpe A-S, Roose E, Galicier L,Girault S, et al. Rituximab prevents long-term relapses in TTP. Blood. 2018;132:2210.CrossRef
15.
go back to reference Thumma S, Idrees S, Phuyal P, Manchala V, Mattana J. When the standard treatment fails: rituximab therapy for refractory TTP. Am J Ther. 2019;26:e552–3.CrossRef Thumma S, Idrees S, Phuyal P, Manchala V, Mattana J. When the standard treatment fails: rituximab therapy for refractory TTP. Am J Ther. 2019;26:e552–3.CrossRef
16.
go back to reference Peyvandi F, Scully M, Kremer Hovinga JA, Cataland S, Knobl P, Wu H, Artoni A, Westwood JP, Mansouri Taleghani M, Jilma B, et al. Caplacizumab for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2016;374:511–22.CrossRef Peyvandi F, Scully M, Kremer Hovinga JA, Cataland S, Knobl P, Wu H, Artoni A, Westwood JP, Mansouri Taleghani M, Jilma B, et al. Caplacizumab for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2016;374:511–22.CrossRef
Metadata
Title
Complete recovery of deep venous thrombosis from Coombs (+) thrombotic thrombocytopenic purpura: case report
Authors
Mi Zhou
Jie Yin
Publication date
01-12-2022
Publisher
BioMed Central
Published in
Journal of Cardiothoracic Surgery / Issue 1/2022
Electronic ISSN: 1749-8090
DOI
https://doi.org/10.1186/s13019-022-01789-8

Other articles of this Issue 1/2022

Journal of Cardiothoracic Surgery 1/2022 Go to the issue