Critical Care |

Thrombotic Thrombocytopenic Purpura (TTP) Presenting as Acute Respiratory Distress Syndrome (ARDS) FREE TO VIEW

Ma. Theresa Bautista, MD; Jay Steingrub, MD; Ramakant Sharma, MD
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Baystate Medical Center, Springfield, MA

Chest. 2014;145(3_MeetingAbstracts):167A. doi:10.1378/chest.1835176
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SESSION TITLE: Critical Care Case Report Posters

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM

INTRODUCTION: TTP was once recognized for its classic pentad features of fever, thrombocytopenia, microangiopathic hemolytic anemia, neurologic dysfunction and renal impairment. However TTP may present with atypical features and the diagnosis may be unrecognized.

CASE PRESENTATION: A 55-year-old gentleman with hypertension and eczema, reported cough, colds and fevers for a week. He presented to an urgent care center with worsening dyspnea and hypoxia, then transferred to our ER. He was mentally intact, normotensive, tachycardic, tachypneic with O2 saturations of 95% on 100% oxygen, and diffuse crackles on chest auscultation. CXR showed interstitial opacities bilaterally. Work-up showed WBC 9,400/mm3 (88% neutrophils), hemoglobin 12g/dl and low platelets (48,000/mm3). Metabolic panel was within normal limits. He received ceftriaxone and azithromycin while non-invasive positive pressure ventilation was initiated. He deteriorated and was intubated, transferred to ICU. Repeat CXR showed worsening ARDS. Bronchoscopy with lavage revealed thick secretions but samples for stain and cultures did not yield any organism. He developed persistent high fevers and worsening thrombocytopenia down to 10,000/mm3. Repeat haptoglobin was severely low <10mg/dl (323mg/dl on admission) and LDH was high (1,600units/L). His creatinine peaked at 1.3mg/dl (from 0.8mg/dl). A peripheral smear was reviewed and revealed schistocytes. Plasmapheresis was initiated for the diagnosis of TTP. Platelet counts improved and normalized with therapy. He clinically improved and was extubated. His extensive fever work-up was negative for any infectious etiology. ADAMTS13 activity results came back severely low <5% (normal >67%), and ADAMTS 13 antibody elevated at >90 (normal <18), consistent with idiopathic TTP. He was transferred to the floors after a week in ICU and subsequently discharged to rehab.

DISCUSSION: Idiopathic TTP with severe ADAMTS13 deficiency may have atypical presentation including cough, dyspnea and even ARDS. The classic pentad may not be reliable as they may not occur in majority of the patients.

CONCLUSIONS: ARDS may be a presenting feature of TTP. Clinical suspicion should be raised when patients with ARDS present with unexplained thrombocytopenia. Prompt recognition and timely treatment are key to survival.

Reference #1: George JN. How I treat patients with thrombotic thrombocytopenic purpura: 2010. BLOOD. 2010; 116 (20).

Reference #2: Chang JC et al. Acute Respiratory Distress Syndrome as a Major Clinical Manifestation of Thrombotic Thrombocytopenic Purpura. The American Journal of the Medical Sciences. 2001; 321(2).

DISCLOSURE: The following authors have nothing to disclose: Ma. Theresa Bautista, Jay Steingrub, Ramakant sharma

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