European Journal of Internal Medicine 26 (2015) e13–e14
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European Journal of Internal Medicine journal homepage: www.elsevier.com/locate/ejim
Internal Medicine Flashcards
A patient presenting with an acute shortness of breath☆,☆☆ Abdel-Rauf Zeina 1, Mika Shapira-Rootman ⁎,1 Department of Radiology, Hillel Yaffe Medical Center, Hadera, Israel
a r t i c l e
i n f o
Article history: Received 14 July 2014 Accepted 16 August 2014 Available online 16 September 2014
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).
A
B
What is the diagnosis? Answer: The patient suffers from sickle cell anemia complicated by acute chest syndrome.
C
☆ Authorship: all authors had access to the data and a role in writing the manuscript. ☆☆ Funding: none. ⁎ Corresponding author at: Department of Radiology, Hillel Yaffe Medical Center, P.O.B. 169, Hadera 38100, Israel. E-mail address: [email protected] (M. Shapira-Rootman). 1 Affiliated to Faculty of Medicine, Technion — Israel Institute of Technology, Haifa, Israel.
D
Fig. 1. Imaging findings revealed by chest x-ray and computed tomography: bilateral pulmonary opacities (Panels A and B), small and calcified spleen (Panel C) and vertebral endplate depressions at various heights.
http://dx.doi.org/10.1016/j.ejim.2014.08.004 0953-6205/© 2014 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
e14
A.-R. Zeina, M. Shapira-Rootman / European Journal of Internal Medicine 26 (2015) e13–e14
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 [1]. 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 [1]. An H-shaped vertebra deformity appears in approximately 10% of patients [1]. Hyposplenism is a known feature of sickle cell anemia. In the majority of patients, recurrent vaso-occlusive events ultimately lead to splenic atrophy [2]. With time, the spleen becomes small and calcified [1], as is shown in this case. Acute chest syndrome is a leading cause of death among sickle cell patients [3]. The syndrome is defined as new pulmonary opacities
associated with any combination of fever, chest pain, dyspnea, and tachypnea [1]. The leading etiologies include infection, pulmonary fat embolism, and infarction [3]. Radiologic features include airspace disease that affects mainly the middle and lower lobes [1], as is shown in this case. Conflict of interest The authors state that they have no conflicts of interest. References [1] Lonergan GJ, Cline DB, Abbondanzo SL. Sickle cell anemia. Radiographics 2001 Jul– Aug;21(4):971–94. [2] Brousse V, Buffet P, Rees D. The spleen and sickle cell disease: the sick(led) spleen. Br J Haematol 2014 Jul;166(2):165–76. [3] Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. N Engl J Med 2000 Jun 22;342(25):1855–65.
Contents lists available at ScienceDirect
European Journal of Internal Medicine journal homepage: www.elsevier.com/locate/ejim
Internal Medicine Flashcards
A patient presenting with an acute shortness of breath☆,☆☆ Abdel-Rauf Zeina 1, Mika Shapira-Rootman ⁎,1 Department of Radiology, Hillel Yaffe Medical Center, Hadera, Israel
a r t i c l e
i n f o
Article history: Received 14 July 2014 Accepted 16 August 2014 Available online 16 September 2014
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).
A
B
What is the diagnosis? Answer: The patient suffers from sickle cell anemia complicated by acute chest syndrome.
C
☆ Authorship: all authors had access to the data and a role in writing the manuscript. ☆☆ Funding: none. ⁎ Corresponding author at: Department of Radiology, Hillel Yaffe Medical Center, P.O.B. 169, Hadera 38100, Israel. E-mail address: [email protected] (M. Shapira-Rootman). 1 Affiliated to Faculty of Medicine, Technion — Israel Institute of Technology, Haifa, Israel.
D
Fig. 1. Imaging findings revealed by chest x-ray and computed tomography: bilateral pulmonary opacities (Panels A and B), small and calcified spleen (Panel C) and vertebral endplate depressions at various heights.
http://dx.doi.org/10.1016/j.ejim.2014.08.004 0953-6205/© 2014 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
e14
A.-R. Zeina, M. Shapira-Rootman / European Journal of Internal Medicine 26 (2015) e13–e14
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 [1]. 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 [1]. An H-shaped vertebra deformity appears in approximately 10% of patients [1]. Hyposplenism is a known feature of sickle cell anemia. In the majority of patients, recurrent vaso-occlusive events ultimately lead to splenic atrophy [2]. With time, the spleen becomes small and calcified [1], as is shown in this case. Acute chest syndrome is a leading cause of death among sickle cell patients [3]. The syndrome is defined as new pulmonary opacities
associated with any combination of fever, chest pain, dyspnea, and tachypnea [1]. The leading etiologies include infection, pulmonary fat embolism, and infarction [3]. Radiologic features include airspace disease that affects mainly the middle and lower lobes [1], as is shown in this case. Conflict of interest The authors state that they have no conflicts of interest. References [1] Lonergan GJ, Cline DB, Abbondanzo SL. Sickle cell anemia. Radiographics 2001 Jul– Aug;21(4):971–94. [2] Brousse V, Buffet P, Rees D. The spleen and sickle cell disease: the sick(led) spleen. Br J Haematol 2014 Jul;166(2):165–76. [3] Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. N Engl J Med 2000 Jun 22;342(25):1855–65.
