Blood, Vol. 92 No. 9 (November 1), 1998:
pp. 3477-3478
CORRESPONDENCE
An Unusual Cause of Abdominal Pain in Sickle Cell Disease
 |
LETTER |
To the Editor:
A 21-year-old man with homozygous sickle cell disease presented with a
3-week history of epigastric pain, anorexia, and weight loss of 5 kg.
On examination he was in considerable discomfort and was febrile
(temperature 38.5°C) and tachycardic (110 beats per
minute [bpm]). Lung fields were clear, oxygen
saturation was 100% on air. The liver was enlarged to 6 cm below the
costal margin with tenderness over the left lobe. Blood count revealed
a hemoglobin level of 7.3 g/dL (2 g/dL below his steady-state value)
and marked neutrophilia (46.0 × 109/L) with left-shift.
Liver function tests showed moderate elevation in gamma-glutamyl
transferase (200 IU/L; normal range, 5 to 55 IU/L) but were otherwise
unremarkable. Differential diagnosis for such a presentation in sickle
cell disease includes hepatic sequestration, cholecystitis, and
mesenteric sickling crisis. The patient was treated with analgesics,
intravenous broad spectrum antibiotics, and exchange blood transfusion.
Ultrasound and computed tomography (CT) abdomen showed a subcapsular
fluid collection 10 cm in diameter related to the left lobe of the
liver, with no evident intrahepatic, biliary, or splenic pathology
(Fig 1). On percutaneous
aspiration 250 mL pus was obtained. Microscopy showed yeasts and
polymorphonuclear leukocytes, and Candida albicans was
isolated from pus and blood cultures. He was treated with intravenous
fluconazole and percutaneous drainage of abscess using an indwelling
pigtail catheter. A total of 1.5 L of pus was drained over a 4-week
period until no fluid remained. He has since remained well, with no
recurrence of the abscess.
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| Fig 1.
CT scan of abdomen showing a large subcapsular collection
of fluid compressing the left lobe of the liver. Liver and spleen
appear normal.
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The subject's past history includes several episodes of pneumonia
since early childhood and Salmonella osteomyelitis when he
was 16 years old. Of interest, 8 months before the current presentation he was noted to have pharyngitis, and a throat
swab yielded a heavy growth of Candida. He was treated with
nystatin suspension and symptoms swiftly resolved. Subsequent
occurrence of a perihepatic candida abscess remains difficult to
explain. There was no history of abdominal trauma and findings did not suggest perforation of bowel or biliary tree.
Infections are a major cause of morbidity and mortality in sickle cell
disease.1 Patients present with pulmonary, meningeal, bone,
or bloodstream infections usually caused by bacteria, although there is
increasing awareness of the role of mycoplasmas and viruses in sickle
lung disease. Fungal infections in sickle cell disease are very
rare.2,3 This case constitutes the first description of
fungal abscess in a sickle cell patient with no other identifiable immunocompromising factors. Serology for human immunodeficiency virus
(HIV)-1 and -2 was negative. Lymphocyte counts, T-cell subsets, serum
Ig, and complement levels were unremarkable. Neutrophil superoxide
production and integrins were normal and no defect in opsonization was
observed. These results notwithstanding, the occurrence of candida
infection, salmonella osteomyelitis, and recurrent pneumonias in a
single individual reflects profound and wide-ranging immune deficiency.
This may represent one extreme of a spectrum of immune dysfunction in
sickle cell disease. Functional asplenia was described in sickle cell
patients three decades ago,4 but abnormalities in humoral
and cell-mediated immunity5 remain less well-defined.
Further investigation is required, particularly in light of the use of
myelosuppressive agents such as hydroxyurea in sickle cell
patients, which can potentially heighten susceptibility to
infection.
Roopen Arya
James Wade
Adrian Stephens
Departments of Haematological Medicine and
Microbiology
King's College School of Medicine & Dentistry
London,
UK
| |
REFERENCES |
1.
Barrett-Connor E:
Bacterial infection and sickle cell anemia.
Medicine
50:97,
1971[Medline]
[Order article via Infotrieve]
2.
Hardy RE,
Cummings C,
Thomas F,
Harrison D:
Cryptococcal pneumonia in a patient with sickle cell disease.
Chest
89:892,
1986[Abstract/Free Full Text]
3.
Ho F,
Snape WJ Jr,
Venegas R,
Lechago J,
Klein S:
Choledochal fungal ball: An unusual cause of biliary obstruction.
Dig Dis Sci
33:1030,
1988[Medline]
[Order article via Infotrieve]
4.
Pearson HA,
Spencer RD,
Cornelius EA:
Functional asplenia in sickle cell anemia.
N Engl J Med
281:923,
1969
5.
Taylor SC,
Shacks SJ,
Mitchell RA:
In vitro lymphocyte blastogenic responses and cytokine production in sickle cell disease patients with acute pneumonia.
Pediatr Infect Dis J
15:340,
1996[Medline]
[Order article via Infotrieve]
