A Review on Differential Diagnosis and Diagnostic Criteria of Complement-Mediated Thrombotic Microangiopathy with a PLASMIC Score Below Six and Coexisting Hepatitis B Positivity in a Male Patient

Abstract

Microangiopathic Hemolytic Anemia (MAHA); Congenital Thrombotic thrombocytopenic purpura (TTP), Acquired (Immune) TTP, Shiga toxin associated Endemic hemolytic uremic syndrome (HUS) and Complement-Mediated TMA (CM-TMA), which may present with different clinical findings, Thrombocytopenia is a severe condition that affects multiple organ systems with anemia. In the congenital form, ADAMTS13 (von Willebrand Factor-Cleaving Protease or a metalloprotease that belongs to the “α disintegrin and metalloprotease with a thrombospondin type I motif) is diagnosed by the deficiency of the enzyme and the absence of antibodies. While in autoimmune TTP, the enzyme deficiency is associated with antibodies, endemic HUS associated with Shiga toxin is characterized by decreased ADAMTS13 levels due to endothelial damage. CM-TMA is associated with complement factor H (CFH) inhibitory dysfunction and increased complement levels due to a genetic mutation. On March 27, 2023, the patient with complaints of shortness of breath, headache, dizziness, weakness, and numbness in the hands and arms was admitted to the internal medicine clinic. The patient, presenting with Thrombocytopenia, reduced haptoglobin levels, elevated reticulocyte count, increased LDH, indirect hyperbilirubinemia, and a PLASMIC score of 6 in peripheral blood smear, was hospitalized and treated with the prediagnosis of TTP. Later, ADAMTS13 level was found to be 73% (normal range: 40– 130), and the diagnosis of CM-TMA was considered. In addition, we discussed the clinical distinction and treatment of TTP by reviewing the literature.

Keywords

• microangiopathic hemolytic anemia
• thrombotic thrombocytopenic purpura
• complement-related thrombotic thrombocytopenic purpura
• ADAMTS13 protein
• schistocyte
• plasmic score

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