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A patient presenting with an acute shortness of breath

  • Author Footnotes
    1 Affiliated to Faculty of Medicine, Technion — Israel Institute of Technology, Haifa, Israel.
    Abdel-Rauf Zeina
    Footnotes
    1 Affiliated to Faculty of Medicine, Technion — Israel Institute of Technology, Haifa, Israel.
    Affiliations
    Department of Radiology, Hillel Yaffe Medical Center, Hadera, Israel
    Search for articles by this author
  • Author Footnotes
    1 Affiliated to Faculty of Medicine, Technion — Israel Institute of Technology, Haifa, Israel.
    Mika Shapira-Rootman
    Correspondence
    Corresponding author at: Department of Radiology, Hillel Yaffe Medical Center, P.O.B. 169, Hadera 38100, Israel.
    Footnotes
    1 Affiliated to Faculty of Medicine, Technion — Israel Institute of Technology, Haifa, Israel.
    Affiliations
    Department of Radiology, Hillel Yaffe Medical Center, Hadera, Israel
    Search for articles by this author
  • Author Footnotes
    1 Affiliated to Faculty of Medicine, Technion — Israel Institute of Technology, Haifa, Israel.
Published:September 06, 2014DOI:https://doi.org/10.1016/j.ejim.2014.08.004

      1. Introduction

      A 37-year-old woman presented with shortness of breath, cough, and fever that began a few days earlier. Her pulse rate was high (110 bpm) with a body temperature of 38.6 °C. Pulmonary auscultation revealed decreased breath sounds over the lower lung fields. Laboratory study showed pronounced leukocytosis (white cell count of 27,500 per cm3) with 89.4% neutrophils and anemia with hemoglobin levels of 9 g per dl. Chest X-ray and computed tomography (CT) scan of the chest revealed bilateral pulmonary opacities over the lower lung fields (Fig. 1 panels A and B). Additionally, the spleen appeared small and heavily calcified (panel C, arrow). Sagittal reconstruction revealed central depression of both superior and inferior end plates of several vertebrae (panel D).
      Figure thumbnail gr1
      Fig. 1Imaging findings revealed by chest x-ray and computed tomography: bilateral pulmonary opacities (Panels A and B), small and calcified spleen (Panel C) and vertebral end-plate depressions at various heights.

      What is the diagnosis?

      Answer: The patient suffers from sickle cell anemia complicated by acute chest syndrome.

      2. Diagnosis

      Sickle cell anemia is an autosomal recessive disease with abnormally shaped red blood cells. Disease manifestations are numerous and attributed mostly to anemia, vaso-occlusive events, or superimposed infection [
      • Lonergan G.J.
      • Cline D.B.
      • Abbondanzo S.L.
      Sickle cell anemia.
      ]. The patient presented with a few of the classical radiological findings associated with sickle cell disease.
      The H-shaped deformity (or Lincoln log vertebrae) is considered essentially pathognomonic for sickle cell anemia and results from ischemic changes involving the microvasculature at the end plates [
      • Lonergan G.J.
      • Cline D.B.
      • Abbondanzo S.L.
      Sickle cell anemia.
      ]. An H-shaped vertebra deformity appears in approximately 10% of patients [
      • Lonergan G.J.
      • Cline D.B.
      • Abbondanzo S.L.
      Sickle cell anemia.
      ].
      Hyposplenism is a known feature of sickle cell anemia. In the majority of patients, recurrent vaso-occlusive events ultimately lead to splenic atrophy [
      • Brousse V.
      • Buffet P.
      • Rees D.
      The spleen and sickle cell disease: the sick(led) spleen.
      ]. With time, the spleen becomes small and calcified [
      • Lonergan G.J.
      • Cline D.B.
      • Abbondanzo S.L.
      Sickle cell anemia.
      ], as is shown in this case.
      Acute chest syndrome is a leading cause of death among sickle cell patients [
      • Vichinsky E.P.
      • Neumayr L.D.
      • Earles A.N.
      • Williams R.
      • Lennette E.T.
      • Dean D.
      • et al.
      Causes and outcomes of the acute chest syndrome in sickle cell disease.
      ]. The syndrome is defined as new pulmonary opacities associated with any combination of fever, chest pain, dyspnea, and tachypnea [
      • Lonergan G.J.
      • Cline D.B.
      • Abbondanzo S.L.
      Sickle cell anemia.
      ]. The leading etiologies include infection, pulmonary fat embolism, and infarction [
      • Vichinsky E.P.
      • Neumayr L.D.
      • Earles A.N.
      • Williams R.
      • Lennette E.T.
      • Dean D.
      • et al.
      Causes and outcomes of the acute chest syndrome in sickle cell disease.
      ]. Radiologic features include airspace disease that affects mainly the middle and lower lobes [
      • Lonergan G.J.
      • Cline D.B.
      • Abbondanzo S.L.
      Sickle cell anemia.
      ], as is shown in this case.

      Conflict of interest

      The authors state that they have no conflicts of interest.

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        Causes and outcomes of the acute chest syndrome in sickle cell disease.
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