title: Thrombotic thrombocytopenic purpura or immune thrombocytopenia in a sickle cell/β+-thalassemia patient: a rare and challenging condition.
abstract: The diagnosis of thrombotic thrombocytopenic purpura is one of the possible diagnosis when a patient is admitted with unexpected micro-angiopathic hemolytic anemia and thrombocytopenia. The combination of sickle cell/β(+)-thalassemia and thrombotic thrombocytopenic purpura is rare and triggering. This article describes the poor outcome of a patient with sickle cell/β(+)-thalassemia presenting with gingival bleeding, severe thrombocytopenia and anemia. The patient had normal renal function, no neurological deficit and he was initially treated as immune thrombocytopenic purpura. He eventually died due to multi-organ failure and brain hemorrhage even though he had started plasma exchange sessions. The co-existence of thrombotic thrombocytopenic purpura and sickle cell anemia is making the diagnosis of the former difficult. Early and rapid intervention is critical to the outcome.
authors.affiliation: Thalassemia Unit, 2nd Department of Internal Medicine, Aristotle University, Hippokrateon Hospital, Thessaloniki, Greece. Electronic address: efivlachaki@yahoo.gr.
authors.forename: Efthymia
authors.initials: E
authors.lastname: Vlachaki
authors.affiliation: Thalassemia Unit, 2nd Department of Internal Medicine, Aristotle University, Hippokrateon Hospital, Thessaloniki, Greece.
authors.forename: Aleka
authors.initials: A
authors.lastname: Agapidou
authors.affiliation: Thalassemia Unit, 2nd Department of Internal Medicine, Aristotle University, Hippokrateon Hospital, Thessaloniki, Greece.
authors.forename: Nikolaos
authors.initials: N
authors.lastname: Neokleous
authors.affiliation: Thalassemia Unit, 2nd Department of Internal Medicine, Aristotle University, Hippokrateon Hospital, Thessaloniki, Greece.
authors.forename: Despoina
authors.initials: D
authors.lastname: Adamidou
authors.affiliation: Thalassemia Unit, 2nd Department of Internal Medicine, Aristotle University, Hippokrateon Hospital, Thessaloniki, Greece.
authors.forename: Evaggelia
authors.initials: E
authors.lastname: Vetsiou
authors.affiliation: Thalassemia Unit, 2nd Department of Internal Medicine, Aristotle University, Hippokrateon Hospital, Thessaloniki, Greece.
authors.forename: Panagiota
authors.initials: P
authors.lastname: Boura
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D002543:Cerebral Hemorrhage / Q000209:etiology / Q000628:therapy; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D009102:Multiple Organ Failure / Q000209:etiology / Q000628:therapy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D017086:beta-Thalassemia / Q000150:complications / Q000175:diagnosis / Q000628:therapy
keywords: ADAMTS-13; Plasma exchange; Sickle cell; Thrombocytopenia; Thrombotic thrombocytopenic purpura; β-thalassemia