Thrombotic thrombocytopenic purpura (TTP) is characterized by disseminated thrombotic occlusions in the microcirculation and a syndrome of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, fever, renal and neurologic abnormalities. Several factors such as viral and bacterial pathogens, pancreatitis, drugs, collagen-vascular diseases, cancers, and pregnancy have been reported to cause TTP, Brucellosis is an exceptional cause of this disorder. We present a case of a 33 year old male who was found to have Brucella antigen (IgG) positivity who responded well to antibiotic therapy directed to Brucella infection. He subsequently reported back with B/L diminution of vision, fever and was found to have severe thrombocytopenia. Ophthalmology opinion revealed retinal hemorrhages. In view of severe thrombocytopenia with a normal coagulogram, raised LDH, renal azotemia and peripheral blood smear showing fragmented RBCs he was diagnosed to have Thrombotic Thrombocytopenic Purpura (TTP) secondary to Brucellosis. He was immediately treated with Plasma exchange; however, he relapsed after initial cycles. He underwent further plasma exchanges with unsatisfactory response, thus was eventually started on Rituximab to which he responded well.
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Am J Hematol
December 2024
Clinical Haematology, Austin Hospital, Melbourne, Victoria, Australia.
J Manag Care Spec Pharm
December 2024
Health Economics and Value Assessment, Sanofi, Boston, MA.
Background: Immune thrombotic thrombocytopenic purpura (iTTP) is a rare, life-threatening thrombotic microangiopathy. Caplacizumab is the only treatment approved by the European Medicines Agency and the US Food and Drug Administration for iTTP, to be given in combination with plasma exchange therapy (PEX) and immunosuppression (IS). The National Institute for Health and Care Excellence's independent appraisal committee assessed the cost-effectiveness of caplacizumab and concluded that the addition of caplacizumab to PEX+IS is cost-effective under a patient access scheme in the United Kingdom.
View Article and Find Full Text PDFPediatr Rheumatol Online J
December 2024
Department of Pediatrics, Kanazawa University Hospital, Takara-machi 13-1, Kanazawa, 920-8640, Ishikawa, Japan.
Int J Lab Hematol
December 2024
LabPLUS, Auckland City Hospital, Auckland, New Zealand.
Introduction: The TECHNOSCREEN ADAMTS-13 assay (ADSC) is a new lateral flow test which is simple and quick to perform, with a high negative predictive value (NPV); it may improve the diagnostic workflow for TTP. LabPlus in Auckland, New Zealand, performs all ADAMTS13 tests in the Auckland and Northland regions. The ADSC was used at LabPlus between 2022 and 2023 as part of a protocol where results of 0 IU/mL and 0.
View Article and Find Full Text PDFGlob Pediatr Health
December 2024
Universidad de Antioquia, Medellín, Colombia.
. Primary thrombotic microangiopathy includes hemolytic uremic syndrome caused by Shiga toxin-producing , atypical hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. .
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