Thrombotic thrombocytopenic purpura and cardiac papillary fibroelastoma: a 'unique coexistence'.
Thrombotic thrombocytopenic purpura (TTP), a complex thrombotic microangiopathy, remains an evolving enigma. A 49-year-old African-American woman presented with acute left hemiplegia, an ischemic cerebrovascular accident involving the right middle cerebral artery. Sequential appearance of thrombocytopenia and evidence of microangiopathic haemolysis led to the diagnosis of acquired idiopathic autoimmune TTP. This was managed with plasma exchange (PEX) and steroids. Early haematologic relapse within a month was managed with the addition of rituximab attaining sustained remission. The patient presented 3 years later with acute confusion and expressive aphasia due to multiple infarcts involving the left parieto-occipital cortex. Transoesophageal echocardiography demonstrated a pedunculated 6 mm mitral valvular mass consistent with a papillary fibroelastoma. Anticoagulation was instituted and the patient was continued on therapeutic oral warfarin. A haematologic relapse of TTP eventually emerged and was managed with PEX, steroids and rituximab. This vignette demonstrates several dilemmas in the clinical presentation, diagnosis and management of TTP in current day practice. Rituximab has adjuvant benefits to PEX and is being investigated as potential first-line therapy. Monitoring ADAMTS13 activity and inhibitor titre, as in our case, prove to have prognostic significance. Cardiac fibroelastomas are rare benign cardiac tumours usually arising from valvular endocardium with thromboembolic potential. One of the proposed mechanisms of origin of these masses is organizing thrombi in the setting of endocardial injury and inflammation questioning a possible link to thrombotic microangiopathy. To the best of our knowledge, this is the first report of this unique coexistence.
aDepartment of Internal Medicine bDivision of Cardiology cDivision of Hematology-Oncology, Albert Einstein Medical Center, Philadelphia, Pennsylvania, USA.
Praveen
P
Ramakrishnan Geethakumari
Alexander
A
Rubin
Gabor
G
Varadi
D051722:ADAM Proteins / Q000097:blood; D000071120:ADAMTS13 Protein; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000925:Anticoagulants / Q000627:therapeutic use; D005260:Female; D005350:Fibroma / Q000150:complications / Q000473:pathology* / Q000628:therapy; D006338:Heart Neoplasms / Q000150:complications / Q000473:pathology* / Q000628:therapy; D006429:Hemiplegia / Q000150:complications / Q000473:pathology* / Q000628:therapy; D006801:Humans; D008875:Middle Aged; D008943:Mitral Valve / Q000187:drug effects / Q000473:pathology*; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications / Q000473:pathology* / Q000628:therapy; D000069283:Rituximab; D013256:Steroids / Q000627:therapeutic use; D014859:Warfarin / Q000627:therapeutic use
D058846:Antibodies, Monoclonal, Murine-Derived; D000925:Anticoagulants; D013256:Steroids; D000069283:Rituximab; D014859:Warfarin; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human