Navkiran Randhawa
Franciscan Health Olympia Fields
Olympia Fields, Illinois
Disclosure information not submitted.
Yhana Chavis, DO
Resident Physician, Internal Medicine
Francisan Health, United States
Disclosure information not submitted.
Title: The Bloody Dyscrasia: Severe Refractory TTP in a Young Asymptomatic Patient
Case Report Body:
Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare, fatal disease characterized by severe thrombocytopenia and microangiopathic hemolytic anemia with a mortality rate of 90% if left untreated and up to 20% with standard treatment. We present a 29 year old, asymptomatic patient with a new diagnosis of TTP who was a nonresponder to steroids and plasma exchange (PEX).
Description: A 29 year old African American male with no past medical history was informed that he had blood in his urine during a job screening. He had scleral icterus and petechiae in oral mucosa on exam. Labs revealed anemia and thrombocytopenia with a platelet count of 8,000/uL. Lactate dehydrogenase, reticulocyte count and indirect bilirubin were increased. Haptoglobin was decreased. Peripheral blood smear revealed schistocytes. PEX and solumedrol were initiated for the acquired TTP. ADAMTS13 (A Disintegrin And Metalloprotease with a ThromboSpondin type 1 motif, member 13) activity level was < 5%. He was transferred to the intensive care unit for altered mental status on day 7. His platelet counts dropped to 72,000/uL from 171,000/uL. Computed tomography (CT) head without contrast was negative. CT chest angiogram revealed acute pulmonary embolism. Prophylactic Enoxaprin was transitioned to therapeutic dosing. His mentation improved, however, platelet count decreased to 34,000/uL with daily PEX. He was given a dose of Rituximab and PEX was resumed a day after Rituximab infusion. He was transferred to a tertiary care center for refractory TTP.
Discussion: A complete response to treatment is considered when platelet count remains above 150,000/uL for 2 consecutive days with normal LDH and clinical improvement. Refractory disease should be considered if platelet counts do not respond to PEX after 4 to 7 days, or clinical deterioration noted with standard therapy. Our patient deteriorated and platelet counts decreased with daily PEX. Some studies recommend twice daily PEX for patients with refractory acquired TTP and new neurologic symptoms. Our patient’s neurological symptoms improved in a short period of time and daily PEX was resumed after a dose of Rituximab. This case illustrates the prompt diagnosis of refractory TTP to prevent delaying of management and decrease mortality associated with acquired TTP.