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Diffuse Alveolar Hemorrhage in a Patient With Hemoglobin SC Disease FREE TO VIEW

Ricardo Restrepo*, MD; Michael Baram, MD; Jay Herman, MD; Vipul Kumar, MD; Omar Ibrahim, MD
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Thomas Jefferson University, Philadelphia, PA

Chest. 2012;142(4_MeetingAbstracts):310A. doi:10.1378/chest.1388520
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SESSION TYPE: Critical Care Case Report Posters

PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION: Acute chest syndrome (ACS) is a complication of patients with sickling syndromes (SS). Diffuse Alveolar Hemorrhage (DAH) is not a common presentation of ACS.

CASE PRESENTATION: 19-year-old male with hemoglobin SC disease (SCD) without sickle sequelae presented with diffuse mild body pain associated to fevers, chills and mild nonproductive cough. There was neither pertinent social history nor sick contacts. A CT chest revealed bilateral lower lobe consolidations. The patient was found to have a CBC with 16300 WBC, Hb of 9.7 and 37000 platelets. His HgbS and C were 45% and 39% respectively. A nasopharyngeal swab was negative. Sputum culture was negative. Given his stable oxygen saturation and clinical condition, the patient received ceftriaxone and azithromycin for community-acquired pneumonia. Subsequently his respiratory status and clinical condition deteriorated requiring intubation. Given the persistent fever and increased oxygen requirement, a bronchoscopy was performed finding DAH. IV steroids were started and a red cell exchange transfusion was performed. His post-exchange Hgb S and C values decreased to 16% and 14% respectively. An autoimmune workup and cultures of bronchial specimens were negative. Following these interventions the patient improved and was successfully extubated one day after and weaned to room air.

DISCUSSION: ACS is defined as a new pulmonary infiltrate associated with chest pain, fever, tachypnea, or cough(1). The pathogenesis is complex, and the establishment of a cause is difficult. Multiple factors may be associated including: infection, fat embolus or hypoventilation(2). It’s often impossible to distinguish ACS from pneumonia/pneumonitis or pulmonary infarction on clinical grounds and a definite etiology is usually established only in 38% of cases after an extensive evaluation. SCD is a SS usually with lower anemia due to the compound heterozygosity of Hgb C providing some anti-sickling benefit that prolongs red cell survival and. However, individuals with SCD are at risk for the same complications including ACS but at a decreased frequency(3). To our knowledge, DAH is not a common complication in ACS in SS. Our patient’s development of DAH might have been from tissue ischemia from development of ACS, his underlying thrombocytopenia, or directly from the pneumonitis.

CONCLUSIONS: Recognizing the possibility of DAH in patients presenting with a clinical picture of ACS and initiating therapy is important given the high morbidity and mortality of this condition.

1) Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med. 2000;342(25):1855

2) Graham LM, Sickle cell disease: pulmonary management options. Pediatr Pulmonol Suppl. 2004;26:191

3) Ballas SK, Lewis CN, Noone AM, et al. Clinical, hematological, and biochemical features of Hb SC disease. Am J Hematol. 1982;13(1):37

DISCLOSURE: The following authors have nothing to disclose: Ricardo Restrepo, MIchael Baram, Jay Herman, Vipul Kumar, Omar Ibrahim

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Thomas Jefferson University, Philadelphia, PA




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