Table 1.

Clinical characteristics of the 9 patients with VITT-like prothrombotic disease without proximate exposure to heparin or vaccination

Patient 1 Patient 2 Patient 3Patient 4Patient 5Patient 6Patient 7Patient 8 Patient 9
Sex, age (y) Female,
70-80 y 
Female,
30-40 y 
Female,
70-80 y 
Male,
60-70 y 
Female,
20-30 y 
Female,
30-40 y 
Male,
60-70 y 
Male,
20-30 y 
Male,
5-10 y 
Symptoms Variable Severe
Headache 
Dyspnea Neurological symptoms Common cold, severe headache with hemianopia N/A Dyspnea Severe
headache 
Severe
headache,
vomiting, nausea 
Diagnosis Recurrent DVT and PE, stroke over 3 y CVST with secondary bleeding PE Stroke with secondary
intracranial bleeding 
CVST with secondary bleeding, PE, portal vein thrombosis Recurrent venous and arterial thrombotic events over 13 years, including stroke Bilateral DVT with PE CVST with secondary bleeding CVST 
Preceding infection (interval to admission) — Common cold (7 d) — — Respiratory syncytial virus (unknown, active at admission) — Urinary tract infection (11 d) Common cold (14 d) Adenovirus (10 d) 
Other underlying disease Monoclonal gammopathy — — — — — Crohn‘s disease — — 
Therapy Multiple anticoagulants and platelet aggregation inhibitors Argatroban, IVIG Unfractioned heparin, fondaparinux, apixaban Plasma exchange, fondaparinux, apixaban Unfractionated heparin, followed by VKA Multiple anticoagulants and platelet aggregation inhibitors, over 13 y, IVIG, plasmapheresis Patient refused further therapy Unfractioned heparin; followed by fondaparinux; prednisolone LMWH, rivaroxaban, dexamethasone, acetazolamide 
Outcome PLT count stabilized in lower reference range, lately no further thromboembolism Fatal Recovered Recovered with persistent sequelae from stroke Recovered Recurrent
relapses 
Fatal Recovered with persistent sequelae from CVST Recovered 
Patient 1 Patient 2 Patient 3Patient 4Patient 5Patient 6Patient 7Patient 8 Patient 9
Sex, age (y) Female,
70-80 y 
Female,
30-40 y 
Female,
70-80 y 
Male,
60-70 y 
Female,
20-30 y 
Female,
30-40 y 
Male,
60-70 y 
Male,
20-30 y 
Male,
5-10 y 
Symptoms Variable Severe
Headache 
Dyspnea Neurological symptoms Common cold, severe headache with hemianopia N/A Dyspnea Severe
headache 
Severe
headache,
vomiting, nausea 
Diagnosis Recurrent DVT and PE, stroke over 3 y CVST with secondary bleeding PE Stroke with secondary
intracranial bleeding 
CVST with secondary bleeding, PE, portal vein thrombosis Recurrent venous and arterial thrombotic events over 13 years, including stroke Bilateral DVT with PE CVST with secondary bleeding CVST 
Preceding infection (interval to admission) — Common cold (7 d) — — Respiratory syncytial virus (unknown, active at admission) — Urinary tract infection (11 d) Common cold (14 d) Adenovirus (10 d) 
Other underlying disease Monoclonal gammopathy — — — — — Crohn‘s disease — — 
Therapy Multiple anticoagulants and platelet aggregation inhibitors Argatroban, IVIG Unfractioned heparin, fondaparinux, apixaban Plasma exchange, fondaparinux, apixaban Unfractionated heparin, followed by VKA Multiple anticoagulants and platelet aggregation inhibitors, over 13 y, IVIG, plasmapheresis Patient refused further therapy Unfractioned heparin; followed by fondaparinux; prednisolone LMWH, rivaroxaban, dexamethasone, acetazolamide 
Outcome PLT count stabilized in lower reference range, lately no further thromboembolism Fatal Recovered Recovered with persistent sequelae from stroke Recovered Recurrent
relapses 
Fatal Recovered with persistent sequelae from CVST Recovered 

DVT, deep vein thrombosis; PE, pulmonary embolism; VKA, vitamin K antagonists.

Detailed course described in Greinacher et al.20 

Detailed course described in Greinacher et al.21 

Detailed course described in Warkentin et al.22 

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