Case
A 33-year-old male, with a history of admission to the intensive care
unit for influenza A in 2016, consulted for skin lesions at primary care
and was given treatment with amoxicillin/clavulanic acid and ibuprofen
in October 2020. Three days later, he consulted the emergency department
of our hospital due to worsening of clinical symptoms with thermometric
tympanic fever of 40.5ยบ, blood pressure 123/67 mmHg and heart rate 98
beats per minute. He had not taken any other medication or drugs
previously. Examination revealed a 2cm branching ulcer on the jugal
mucosa and four indurated skin lesions with an erythematous halo and
necrotic centre, suggestive of gangrenous ecthyma (Fig1) , the
largest on the left hand measuring 3cm in diameter.
Laboratory tests on admission showed normal renal, liver and thyroid
function, C-reactive protein 3104.7 nmol/L (Normal Range 0-1100),
Procalcitonin 1400 ng/L (NR 0-100), Haemoglobin 139 g/L (NR 130-160),
Platelets 161 x 109 /L (NR 150-450), Leukocytes 0.5 x
109 /L (NR 4.5-10) (Revised formula: 100%
lymphocytes). IgG 4.79 g/L (NR 6-17), IgA 0.57 g/L (NR 0.7-4) IgM 0.69
g/L (NR 0.4-2.30), C3 and C4 normal. Anti-nuclear, anti-neutrophil
(ANCA), anti-Musk and anti-acetylcholine antibodies were negative.
Serology for HBV, HCV and HIV were negative; he tested IgG+ against EBV
and CMV. Peripheral blood flow cytometry analysis showed
CD4+/CD8+ ratio inversion 0.56, low
B lymphocytes, but no data suggestive of B/T clonality. Blood cultures,
nasal swab for S. aureus as well as bacterial culture and PCR of
skin lesions were negative. Bone marrow aspirate showed normal erythroid
and megakaryocytic series. Granulocytic series represented 3.6% of the
total cellularity, promyelocyte maturational arrest was observed. There
was no evidence of dysplasia or increased blast cellularity, karyotype
46, XY. CT scan revealed an anterior mediastinal mass measuring 47 x 71
x 60 mm with no evidence of locoregional infiltration (Fig2) . A
biopsy of the mass was performed with an anatomopathological result of
mixed type AB thymoma.
On admission, empirical antibiotic therapy was started with
Piperacillin/tazobactam and daptomycin with improvement of symptoms and
resolution of fever in the following days. With the initial diagnosis of
agranulocytosis, G-CSF 480mcr/24h was added to the treatment for 13 days
with no increase in the neutrophil count, so it was discontinued. Once
the diagnosis of thymoma was confirmed and with the suspicion of related
PWCA, single dose of intravenous Inmunoglobuline G (IVIG) 1g/Kg and
ciclosporine (CyA) with target levels 200-300 ng/dl were initiated. On
day +10 there were signs of granulocytic recovery: neutrophils 0.17 x
109 /L in peripheral blood, so G-CSF was associated;
on day +14 from the start of CyA the patient reached neutrophils 17 x
109 /L. Thymectomy was performed on day +21, without
remarkable incidents.
After thymectomy, CyA tappering was started. Six months later, CyA was
discontinued, and neutrophil count remains still stable.
Inmunnoglobuline levels, CD4+/CD8+ratio and B lymphocytes returned to normal values. The patient has not
presented any infectious, CyA-related or post-surgical complications
during follow-up.