Philippine E-Journals

A Case of a Young Female with Fever, Rashes, and a Previous Diagnosis of Thrombotic Thrombocytopenic Purpura

Juzy Haldos

 

Abstract:

Systemic lupus erythematosus (SLE or Lupus) is an autoimmune disorder that involves multisystem microvascular inflammation with the generation of autoantibodies, which can damage every organ system of the body. Worldwide, a conservative estimate states that over 5 million people have lupus, roughly 15–50 per 100,000 people per year. The constellation of several physical findings reflecting multisystem involvement suggests the diagnosis of SLE. Thrombotic thrombocytopenic purpura (TTP or Moschcowitz disease) is a rare thrombotic microangiopathic disorder, causing extensive microscopic blood clots to form in the small blood vessels throughout the body. The incidence of TTP is about 4-6 per 1,000,000 people per year. The classic “pentad” of microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever, and renal dysfunction is indicative of TTP. Thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) are distinct entities that share many overlapping features. The two diseases rarely coexist. This is a report describing a case of a young female with SLE preceeded by TTP by an interval of five years. In 2002, K.L., 18F presented with pallor, fever, easy bruisability and seizures, Hb:5.8, APC:23, LDH:383. She was diagnosed with TTP and treated with blood transfusions, Corticosteroids, and Anti-convulsant. In 2008, K.L., now 23y/o, presented with fever and rashes. She had Malar rash, Palatal ulcers, photosensitivity, ANA positive, and Lymphopenia. She was diagnosed with SLE and received steroids.

SLE is an autoimmune disorder and TTP is a coagulation disorder. They are distinct diseases and they vary in their therapeutic approach. Therapy mainly consists of plasma exchange and blood transfusions in TTP and corticosteroids and immunosuppressants in SLE. However, the diagnosis between them is challenging because TTP and SLE share many similar characteristics. TTP and SLE have been known to occur simultaneously. The onset of SLE may precede TTP and in some cases, TTP may appear first. SLE is caused by gene-environment interactions while TTP is caused by a vWF protease (ADAMTS-13) deficiency. Acquired TTP occurs with the production of autoantibodies inhibiting ADAMTS-13 activity. This may be the link between SLE-an autoimmune disease, and TTP-an ADAMTS-13 deficiency disorder, and may explain the occurrence of both entities in our patient. In conclusion, a high clinical suspicion for TTP and SLE is warranted in patients with anemia, low platelets, fever, and neurologic as well as renal deficits. TTP and SLE may precede each other or occur simultaneously. Appropriate management rests on accurate diagnosis.