Burkholderia cepacia infection associated with sickle cell disease: An uncommon entity

Introduction

Burkholderia cepacia is an emerging pathogen mostly associated with a compromised immune system. Infection with Burkholderia closely mimics tuberculosis infection.¹ Cystic fibrosis and chronic granulomatous disease patients are more likely to be affected by this uncommon pathogen.² Identification of this unusual pathogen needs a high degree of suspicion.³ Hereby, we report a case of perisplenic intra-abdominal abscess caused by B. cepacia in a patient with sickle cell disease (SCD) which was been successfully treated with meropenem and levofloxacin.

Case report

A 62-year old male presented to our Pulmonary Medicine outpatient department with complaints of intermittent fever, loss of weight and left chest pain for six months. He had nausea and vomiting for the last month. Empirical anti-tubercular therapy had been given for the past six weeks on the basis of a left pleural effusion with no relief. He was known to have SCD.

On general examination, he was febrile at 39.5°C. He was thin built and pale. Breath sounds were diminished and percussion was dull in the left infrascapular and infra-axillary areas. The spleen was palpably tender just below the left coastal margin.

Laboratory analysis showed an anaemia (Hb 80g/l), with haematocrit of 22.9% and mean corpuscular volume 84.5 femtolit, leucocytosis (3.2 × 109/l), with a differential of neutrophil 50%, lymphocyte 40%, monocytes 9%, eosinophils 1% and normal platelet count (161 × 109/l). High-performance liquid chromatography revealed HbA1 8.2%, HbF 33.9%, HbA2 0.8% and HbS 55.5% serum bilirubin 1.39 mgdl, and raised serum glutamic oxaloacetic transaminase (SGOT) (105 U/l) and serum glutamic pyruvic transaminase (SGPT) (146 U/l). Raised serum C-reactive protein and procalcitonin were found at 85mg/l (normal <5 mg/l) and 1.16 ng/ml (normal <0.5 ng/ml), respectively. Investigations for dengue, malaria and typhoid were negative. Blood glucose and creatinine were normal, as was routine urine microscopic examination. The chest radiograph showed a blunted left-side costophenic angle. Abdominal ultrasonography revealed an enlarged spleen 16 cm in size with calcification. A perisplenic collection 8.9 cm × 5.5 cm × 3.1 cm in size was adjacent (Figure 1), from which aspirated pus grew B. cepacia, sensitive to meropenem which was commenced at 1 g tds intravenously with oral levofloxacin 750 mg od for 14 days. A prompt resolution of symptoms resulted, with normalisation of blood parameters within two weeks, though Hb and haematocrit levels remained low on account of sickle cell haemoglobinopathy. Further treatment with oral trimethoprim at 160mg and sulfamethoxazole 800mg bd was continued for a further 6 months.

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Figure 1.

USG whole abdomen and CT scan abdomen suggestive of Perisplenic collection (Pretreatment).

Discussion

Common organisms causing infection in individuals with SCD are Streptococcus pneumoniae, Haemophilus influenzae, Neiserria meningitidis and Salmonella. Other infections include Escherchia coli, urinary tract infection, Mycoplasma pneumonia or Chlamydia pneumoniae respiratory infection and cholecystitis caused by anaerobes.⁴ Functional hyposplenism or asplenia occurs with SCD, despite splenomegaly.⁴ The spleen acts as a phagocytic filter and removes damaged cells, blood-borne microbes and produces antibodies.⁵ Some bacteria are directly recognised by macrophages but many require opsonisation.⁶ With repeated insults, the spleen loses its capacity of filtering encapsulated microbes.⁴ B. cepacia is rarely found. A synergy of two broad-spectrum antibiotics is recommended with long term ‘mopping up’ by co-trimoxazole.