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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 15  |  Issue : 3  |  Page : 492-494

Acute chest syndrome, a distinctive manifestation in sickle cell disease – A case study


Department of Respiratory Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences (Deemed to be University), Wardha, Maharashtra, India

Date of Submission29-Jul-2020
Date of Decision05-Aug-2020
Date of Acceptance20-Sep-2020
Date of Web Publication1-Feb-2021

Correspondence Address:
Dr. Reshma Babu
Department of Respiratory Medicine, Jawaharlal Nehru Medical College, Sawangi, Wardha - 442 001, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jdmimsu.jdmimsu_166_20

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  Abstract 


Acute chest syndrome is the most common cause of death and second most common cause for hospitalization of the adult population suffering from sickle cell disease. Therefore, it is important to make the diagnosis and begin treatment quickly and for this clinician must have a high degree of suspicion. Here, we report the case of a 27-year-old male with homozygous sickle cell disease (SS pattern) since childhood who presented with complaints of high-grade fever with chills, dry cough, chest pain, joint pain, and breathlessness? MMRC Grade I. The patient was stable on admission, but he had recurrent fever spikes. Chest X-ray posteroanterior view was suggestive of right lower lobe consolidation. Contrast-enhanced computed tomography of the thorax revealed consolidation in the superior and posterior basal segments of the right lower lobe. He developed sudden onset of breathlessness and severe chest pain and abdominal pain and saturation drop on the 4th day of admission, and he was put on oxygen support. There was a fall in his hemoglobin. After appropriate management with intravenous (IV) antibiotics, IV analgesics, hydroxyurea, IV fluids resuscitation and blood transfusion, the patient was clinically better and was discharged home safely. Significant improvement was noted on subsequent follow-up.

Keywords: Acute chest syndrome, sickle cell disease, transfusion


How to cite this article:
Jadhav U, Babu R, Ghewade B, Lanjewar A, Gandhasiri D. Acute chest syndrome, a distinctive manifestation in sickle cell disease – A case study. J Datta Meghe Inst Med Sci Univ 2020;15:492-4

How to cite this URL:
Jadhav U, Babu R, Ghewade B, Lanjewar A, Gandhasiri D. Acute chest syndrome, a distinctive manifestation in sickle cell disease – A case study. J Datta Meghe Inst Med Sci Univ [serial online] 2020 [cited 2021 Mar 7];15:492-4. Available from: http://www.journaldmims.com/text.asp?2020/15/3/492/308551




  Introduction Top


Sickle cell disease is one of the most prevalent genetic disorders. The incidence of organ failure has increased in sickle cell disease patients as the survival to adulthood has become more common.[1] Lung is one of the major organs involved and may manifest in two forms: Acute chest syndrome (ACS) and sickle cell chronic lung disease (SCCLD). ACS is a distinctive manifestation of sickle cell disease, more common in homozygous (HbSS) and rare in heterogeneous sickle cell disease and is characterized by cough, fever, hypoxia, chest pain and shortness of breath, and pulmonary infiltrates on chest radiography.[2] We report the case of a 27 year old male with homozygous sickle cell disease who presented with ACS.


  Case Report Top


A 27-year-old male with homozygous sickle cell disease (SS pattern) since childhood presented with complaints of high-grade fever with chills, dry cough, and breathlessness MMRC grade I for 8 days. The patient also complained of generalized, dull aching chest pain for 3 days along with joint pain. He gave a history of multiple blood transfusions in the past, last transfusion being 2 years back. On examination, the patient was moderately built and nourished, afebrile to touch with a pulse rate of 128 beats/min, respiratory rate was 20/min, abdominothoracic, blood pressure 110/70 mm of hg, and oxygen saturation was 95% in room air. Pallor was present. There was no cyanosis, icterus, clubbing, lymphadenopathy, and pedal edema.

Upper respiratory tract was normal. Lower respiratory tract examination revealed crepitation's on auscultation in right infrascapular area. Chest X-ray PA view revealed opacity on the right lower lobe with hilar congestion [Figure 1].
Figure 1: Chest X-ray PA view suggestive of opacity on the right lower lobe with hilar congestion

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All routine investigations were done which revealed hemoglobin 8.6 gm%, total white blood cell (WBC) count 12,200, and platelet 5.68 lakh. LFT, KFT, and urine routine were normal. Sputum acid-fast bacillus was negative. The patient was maintaining saturation in room air, but he had recurrent fever spikes. His repeat hemoglobin was 8.6 g%, total WBC count 14,800, and platelet 3.69 lakh. Blood culture was done which revealed no growth?. Electrocardiogram was done which was normal and did not show any evidence of myocardial ischemia or pulmonary embolism. High-resolution CT (HRCT) of the thorax revealed consolidation in the superior and posterior basal segments of the right lower lobe [Figure 2].
Figure 2: Revealed HRCT thorax revealed consolidation in the superior and posterior basal segments of the right lower lobe

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He developed sudden onset of breathlessness MMRC Grade III and severe chest pain and abdominal pain, and there was saturation drop on the 4th day of admission, and he was put on oxygen through nasal mask. His repeat hemoglobin was 7.3 g%. He was treated with IV antibiotics, intravenous (IV) analgesics, hydroxyurea 500 mg BD, folic acid 1 mg daily, and IV fluids. Two units of packed red blood cells were transfused immediately. Posttransfusion hemoglobin was 10.6 gm%, and he improved symptomatically. Final diagnosis of ACS in homozygous sickle cell disease in crisis (SS pattern) with right lower lobe consolidation was made. He improved clinically and was discharged home safely after 10 days. Repeat chest X-ray on follow-up after 1 month revealed significant resolution [Figure 3].
Figure 3: Revealed repeat chest X-ray on follow-up revealed significant resolution

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  Discussion Top


Sickle cell anemia (SCA) is an autosomal recessive disorder characterized by a point mutation in codon 6 of the betaglobin chain, where glutamic acid is replaced by valine, which results in the formation of HbS.[3] This abnormal Hb is insoluble and polymerizes when exposed to low O2 tension, leading to the major clinical manifestations of SCA, including recurrent vaso-occlusive crisis. Vaso-occlusive crises are more common in homozygous sickle cell anemia.[4]

Nowadays, survival to the adulthood has increased in patients with sickle cell disease mainly due to the improvement of the supportive care. The average age of death is 42 years old for men and 48 years old for women.[1] The increase in survival rate is associated with an increase in the frequency of complications that results in chronic failure of different organs. Lung failure is one of the most common complications. The pulmonary complications of the SCD are either due to a chronic condition (SCCLD) or to an acute condition (ACS).

Up to 78% of the ACS incidents suffer a vaso-occlusive crisis, which usually occurs within the first 72 h after hospital admission. In our patient, this crisis occurred on the 4th day after the admission. While the treatment of ACS episodes is similar in adults and children suffering SCD, the ACS in adults should treated with rapid red blood cell transfusion therapy. Our patient was treated with RBC transfusions on the 4th day of admission. Vascular occlusion is either the cause triggered by some conditions (e.g., infection, asthma, and hypoventilation) or the result (e.g., fat or marrow emboli).[5] Oxygen desaturation was presented in our patient during the 4th day of hospital stay. Oxygen desaturation (hypoxia) in sickle cell disease is an ominous sign because it promotes sickling on massive scale, leads to crisis and needs to be corrected urgently.

ACS in patients with SCD is characterized by a new radiodensity on chest radiograph accompanied by fever and/or respiratory symptoms.[6] ACS patients with sickle cell disease are at increased risk of infections including pneumonia.[7] The pulmonary infiltration and the fever in an adult with SCD may be classified as either an ACS or as pneumonia[8] so empiric antibiotic therapy for community acquired pneumonia, including coverage for atypical bacteria, should be recommended for ACS treatment. In our patient, empirical therapy was initiated with ceftriaxone and moxifloxacin.

The supportive management of an episode of ACS requires a number of active interventions, including adequate pain control and fluid management, use of bronchodilators when wheezing is present, incentive spirometry to prevent the development of pulmonary atelectasis and use of supplemental oxygen. Transfusions are often recommended for the treatment of ACS, and in a large American study,[5] transfusions were given in 75% of episodes; however, no controlled studies have been performed to evaluate the effect of transfusion on lung function and mortality. Our patient had significant improvement following transfusion, oxygen therapy, broad-spectrum antibiotic therapy, hydroxyurea, adequate pain and fluid management, which correlates well with this American study.

Despite being a common hematological disorder, the diagnosis of ACS in a patient with SCD is often delayed that may be life-threatening. With early diagnosis and appropriate management, there will be good prognosis.[9],[10],[11],[12],[13],[14],[15]


  Conclusion Top


ACS is severe complication of sickle cell disease and requires admission. Very high index of suspicion is required for the diagnosis of the ACS as timely medical intervention (transfusions for correction of hemoglobin, oxygen therapy to treat hypoxia, broad-spectrum antibiotics, hydroxyurea, adequate pain, and fluid management) results in good outcome. We should prioritize on preventing the sickle cell crisis developing into ACS.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Godeau B, Schaeffer A, Bachir D, Fleury-Feith J, Galacteros F, Verra F, et al. Bronchoalveolar lavage in adult sickle cell patients with acute chest syndrome: Value for diagnostic assessment of fat embolism. Am J Respir Crit Care Med 1996;153:1691-6.  Back to cited text no. 1
    
2.
Ballas SK, Lieff S, Benjamin LJ, Dampier CD, Heeney MM, Hoppe C, et al. Definitions of the phenotypic manifestations of sickle cell disease. Am J Hematol 2010;85:6-13.  Back to cited text no. 2
    
3.
Bunn HF. Pathogenesis and treatment of sickle cell disease. N Engl J Med 1997;337:762-9.  Back to cited text no. 3
    
4.
Athanasou NA, Hatton C, McGee JO, Weatherall DJ. Vascular occlusion and infarction in sickle cell crisis and the sickle chest syndrome. J Clin Pathol 1985;38:659-64.  Back to cited text no. 4
    
5.
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. National acute chest syndrome study group. N Engl J Med 2000;342:1855-65.  Back to cited text no. 5
    
6.
Davies SC, Luce PJ, Win AA, Riordan JF, Brozovic M. Acute chest syndrome in sickle-cell disease. Lancet 1984;1:36-8.  Back to cited text no. 6
    
7.
Young RC, Catro U, Baxter RP, Dunn R, Armstrong EM, Cook FJ, et al. Lung in sickle cell disease, a clinical overview of vascular infectious and other problems. J Natl Med Assoc 1981;73:19.  Back to cited text no. 7
    
8.
Ataga KI, Orringer EP. Bone marrow necrosis in sickle cell disease: A description of three cases and a review of the literature. Am J Med Sci 2000;320:342-7.  Back to cited text no. 8
    
9.
Chandak P, Vagga A, Chaudhary GA. Haematological Profile in Patient of Sickle Cell Anaemia in Vidarbha Region. Int J Pharm Res 2019;11:1161-4. Available from: https://doi.org/10.31838/ijpr/2019.11.01.205. [Last accessed on 2020 Jan 05].  Back to cited text no. 9
    
10.
Noman O, Bhake A, Bahadure S, Gupta N. Fetal Haemoglobin: A Novel Prognostic Determinant in Sickle Cell Anaemia. Eur J Mol Clin Med 2020;7:2003-8.  Back to cited text no. 10
    
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Wasnik RR, Akarte NR. Evaluation of Serum Zinc and Antioxidant Vitamins in Adolescent Homozygous Sickle Cell Patients in Wardha, District of Central India. J Clin Diagn Res 2017;11:BC01-3. Available from: https://doi.org/10.7860/JCDR/2017/30855.10320. [Last accessed on 2020 Jan 05].  Back to cited text no. 11
    
12.
Baliga S, Chaudhary M, Bhat S, Bhansali P, Agrawal A, Gundawar S. Estimation of Malondialdehyde Levels in Serum and Saliva of Children Affected with Sickle Cell Anemia. J Indian Soc Pedod Prev Dent 2018;36:43-7. Available from: https://doi.org/10.4103/JISPPD.JISPPD_87_17. [Last accessed on 2020 Jan 05].  Back to cited text no. 12
    
13.
Baliga S, Chaudhary M, Bhat S, Bhatiya P, Thosar N, Bhansali P. Determination of Total Antioxidant Capacity of Saliva in Sickle Cell Anemic Patients - A Cross-Sectional Study. J Indian Soc Pedod Prev Dent 2017;35:14-8. Available from: https://doi.org/10.4103/0970-4388.199219. [Last accessed on 2020 Jan 05].  Back to cited text no. 13
    
14.
Jagzape A, Jagzape T, Deshpande V. Simple Visual Reaction Time in Sickle Cell Disease Patients of Pediatric Age Group. Natl J Physiol Pharm Pharmacol 2017;7:1368-70. Available from: https://doi.org/10.5455/njppp.2017.7.0832731082017. [Last accessed on 2020 Jan 05].  Back to cited text no. 14
    
15.
Rakshale N, Sebastian T, Hatwar K, Bhoyar P, Bhute K, Bhusari P, et al. Knowledge Regarding Sickle Cell Anaemia among Newly Married Couples: A Qualitative Study. Int J Cur Res Rev 2020;12:128-33. Available from: https://doi.org/10.31782/IJCRR.2020.121915. [Last accessed on 2020 Jan 05].  Back to cited text no. 15
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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