Diagnostic Challenges in a Case of Immune-Mediated Thrombotic Thrombocytopenic Purpura With Severe ADAMTS13 Deficiency

Thrombotic Thrombocytopenic Purpura (TTP) is rare and potentially life-threatening thrombotic microangiopathy (TMA) caused by acquired immune-mediated or congenital deficiency of the von Willebrand factor regulatory enzyme, a Disintegrin And Metalloproteinase with a Thrombospondin Type 1 motif, memb...

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Published in:Curēus (Palo Alto, CA) CA), 2024-08, Vol.16 (8), p.e67138
Main Authors: Escoto-Pineda, Karel, Alas-Pineda, César, Pavón-Varela, Dennis Javier, Cortés, David
Format: Article
Language:English
Subjects:
Anemia
Apheresis
Blood
Blood platelets
Cancer
Case reports
Creatinine
Cytomegalovirus
Dehydrogenases
Enzymes
Hematology
Hematuria
Hemoglobin
Internal Medicine
Leukocytes
Medical diagnosis
Purpura
Therapeutics
Thrombocytopenia
Tomography
Transplants & implants
Ultrasonic imaging
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Summary:Thrombotic Thrombocytopenic Purpura (TTP) is rare and potentially life-threatening thrombotic microangiopathy (TMA) caused by acquired immune-mediated or congenital deficiency of the von Willebrand factor regulatory enzyme, a Disintegrin And Metalloproteinase with a Thrombospondin Type 1 motif, member 13 (ADAMTS13) which cause microthrombi to form and occlude the microvasculature. The occurrence of acute kidney injury (AKI) in TTP is rare and often underestimated due to confusion with hemolytic uremic syndrome (HUS). A 23-year-old Mestizo male patient presented with altered mental status, hemolytic anemia, thrombocytopenia, intermittent fever, laboratory tests suggestive of thrombotic microangiopathy, and clinical findings consistent with acute kidney injury. Predictive values of the platelet count, lactate dehydrogenase, absent active cancer, schistocytes, mean corpuscular volume, international normalized ratio, creatinine (PLASMIC) score, were used to assess the likelihood of ADAMTS13 deficiency, were employed, and enzymatic activity testing confirmed severe protein deficiency. Honduras' lack of advanced diagnostic capabilities is underscored, emphasizing the urgent need to invest in precision medical technology. ADAMTS13 testing allows for a more precise diagnosis of TTP, which is crucial for early diagnosis and timely treatment.
ISSN:2168-8184
2168-8184
DOI:10.7759/cureus.67138