.jpg "David Goldenberg, DO photo")
David Goldenberg, DO
Advent Health
Orlando, Florida, United States
Disclosure information not submitted.
Huzaifa Wasanwala, n/a
student
University of Central Florida College of Medicine Orlando,FL, United States
Disclosure information not submitted.
Michael Stokes, M.S.
Medical Student
University of Central Florida College of Medicine, Orlando, Fl, United States
Disclosure information not submitted.
Avni Agrawal, n/a
student
Lake Erie College of Osteopathic Medicine, Bradenton, FL, United States
Disclosure information not submitted.
Stephanie Erickson, n/a
student
University of Central Florida College of Medicine Orlando,FL, United States
Disclosure information not submitted.
Samantha Snyder, M.D.
Medical Doctor
Department of Critical Care Medicine. AdventHealth Orlando, Orlando, FL., United States
Disclosure information not submitted.
Christian Hernandez, M.D.
Medical Doctor
Department of Critical Care Medicine. AdventHealth Orlando, Orlando, FL., United States
Disclosure information not submitted.
Arnaldo Lopez Ruiz, MD
Critical Care Intensivist
Advent Health
Orlando, Florida, United States
Disclosure information not submitted.
Title: A Case of Severe Vaso-Occlusive Crisis Complicated by Thrombotic Thrombocytopenic Purpura
Case Report Body:
Introduction: We report a case of a critically ill adult patient with sickle cell disease complicated by cerebral venous sinus thrombosis and severe multi-organ failure due to vaso-occlusive crisis and thrombotic microangiopathy. She was treated with exchange transfusion, plasma exchange and corticosteroids with improvement in mental status, liver function and thrombocytopenia.
Description: A 35-year old female with a history of sickle cell disease (SCD) initially presented to an outside hospital with bilateral lower extremity and back pain. She was started on intravenous fluids and pain management. Her Hgb was 5.2 g/dl and she was transfused 2 units of packed red blood cells. On day three, the patient developed an acute change in mental status, fever, and hypoxemia. Computed tomography of the head revealed extensive dural venous sinus thrombosis and she was started on heparin drip. The patient was transferred to the medical intensive care unit. On arrival, she was found to have acute renal and liver failure. Her mental status continued to worsen and she was intubated for airway protection. Labs showed WBC 14.9 × 109/L, Hgb 7.1 g/dl , platelet count of 104 × 109/L, HgbS 38.6 g/dl, ferritin 100,000 ng/mL ug/L, elevated LDH and low haptoglobin. Peripheral blood smear (PBS) revealed sickle and target cells. Exchange transfusion was initiated. On day six, a more than 50% drop in platelet count was noted. A repeat of PBS showed schistocytes (1+). Heparin-induced thrombocytopenia (HIT) and disseminated intravascular coagulation (DIC) workup were negative. An initial PLASMIC score of 4. However, ADAMTS13 enzyme activity was significantly reduced (5%). Plasmapheresis was started on day seven with subsequent improvement in mental status, liver function, and thrombocytopenia.
Discussion: Her presentation was challenging due to a broad differential including hemophagocytic lymphohistiocytosis , DIC , and HIT. Her PLASMIC score was low on admission and her initial PBS did not show schistocytes not suggestive of TTP. However, a continuing decrease in platelet prompted a repeat in workup which established her diagnosis. The diagnosis of TTP can be challenging in a patient with a severe vaso-occlusive crisis and a high index of suspicion and timely intervention is essential in reversing the progression of multi-organ failure.