Thrombotic thrombocytopenia purpura (TTP, rare blood disorder)

Description A rare and life thr...

Description

A rare and life threatening blood disorder. Blood clots develop in the small blood vessels of many of the organs of the body. These clots cause damage to the kidneys, brain and lungs. These blood clots also consume most of the platelets in the body resulting in a low platelet count (thrombocytopenia). TTP is associated with pregnancy, certain cancers, HIV infection, autoimmune diseases, medications, and serious infections (sepsis).

Symptoms

Pinpoint red spots under the skin (petechiae). Purplish bruises on the skin, mouth, or vaginal area (purpura). Confusion, seizures, weakness, numbness, fever, blood in urine (hematuria), anemia, breathing problems, yellow skin color (jaundice).

Tests

Common tests used for diagnosis and treatment

Workup:
A history and physical exam will be done. Blood test and imaging will be done to confirm the diagnosis, rule out other disorders and identify damage done by the many small blood clots.

Tests:
Complete blood count (CBC), Comprehensive metabolic panel (CMP), CT Scan, Magnetic resonance imaging (MRI), Urinalysis (UA)

Other Specific Tests: LDH, blood smear

Specialists:
Blood and Cancer Care (hematology and oncology), Pediatric Hematology and Oncology

Treatment

Plasma exchange has greatly decreased the mortality of TTP. First, some of the patient's blood is removed. Then the plasma from the blood is removed and replaced with plasma from healthy people. The patient's own blood cells along with the new plasma is given back to the patient. This treatment is performed daily until the number of platelets increases, and the symptoms improve. Some patients require surgery to remove the spleen (splenectomy). Others may need medications such as immunosuppressive medications and/or steroids.