Case presentation
A 73 year old caucasian woman with a past medical history of mammectomy
for in situ breast cancer, appendectomy and spinal angioma,
presented to her hematologist consultation in February 2022 with acute
carotidynia, left otalgia, and fever. She had been diagnosed with
intermediate-2 risk MDS and she had therefore started treatment with
hypomethylating agents (chemical analog of cytidine - AZACYTIDINE) with
complete remission obtained 6 months thereafter. Progression of MDS with
8% of blasts in the bone marrow in January 2022 led to the addition of
inhibitor of BCL-2 protein (VENETOCLAX), in association with AZACYTIDINE
that was still ongoing. She was diagnosed with SARS-COV2 infection on
February 04, 2022, with cough and rhinitis without any fever. Since this
was an immunocompromised patient, we treated this pauci-symptomatic
SARS-COV2 infection (without the need for oxygen therapy) with an
intravenous infusion of sotrovimab 500mg on February 05, 2022. She
already received oral amoxicillin 1g twice per day prescribed by her
general practitioner on February 04, 2022, and then the treatment was
stopped the following day. On February 08, 2022, the patient displayed
deep neutropenia (290/mm3) without fever and was treated with
granulocyte colony-stimulating factor (NIVASTIM) for four days.
Before those events, last injection of AZACYTIDINE was performed on
January 18, 2022 and last dose of VENETOCLAX was taken on January 26,
2022. COVID-19 PCR test remained positive
Odynophagia started February 10th, followed by left carotidynia on the
12th with swelling of the neck. Probabilistic antibiotherapy with
macrolids was started on the 16th in the hypothesis of
an ear, throat and nose (ENT) infection, with no improvement. At her
hematology consultation on February 18th: patient displayed fever at
38,5°C and elevation of the C-reactive protein at 328 mg/L(FIGURE A). A
computed tomography (CT) scan performed the same day revealed tissue
infiltration thickening surrounding the left internal carotid artery,
the carotid bifurcation, the common carotid artery; as well
circumferential thickening of the aortic arch (FIGURE B). Cervical
ultrasound confirmed perivascular infiltration, maximum next to the
internal carotid bifurcation/external as well as a harmonious left
peri-carotid circumferential thickening. In the more anterior fat /
region of cervical group II A, there was a well differentiated node, not
suspicious. After disinfection, cytopunctures (2 passages) in the inter
carotid region was performed. Cytology analysis found a largely hematic
material consisting sheets of red blood cells associated with some
figured elements of the blood, polynuclear and lymphocyte. No giant cell
was visualized. No clearly identifiable tissue fragment was observed. No
element of suspicious character was observed within the limits of these
documents. The patient was transferred the same day to our internal
medicine and vascular department.
Hypothesis of an infectious disease led to probabilistic broad-spectrum
beta-lactam antibiotics. Examination revealed painful swelling of the
neck with tenderness over his left carotid but had no vascular bruit.
Other hypothesis was a large vessel vasculitis associated to MDS,
Temporal arteries were normal, she had no headaches,arthralgias and
blood pressure was symmetrical.
Oral cavity examination was normal. There were no thyroid nodules.
Pulmonary and heart auscultation were normal. There were no skin rashes,
Ultrasound of the left internal carotid artery found isoechoic,
circumferential wall thickening extending to the origins of the internal
and external carotid arteries (figure C). There were no inflammatory
halos of the temporal arteries. 18-FDG-TEP scanner showed
hypermetabolism of the left carotid, circumferential hypermetabolism of
the aortic arch, moderate hypermetabolism of the anterior wall of the
aorta of its transdiaphragmatic passage.
Blood cultures and mycobacterial blood cultures were sterile. EBV
specific serology showed past infection. HIV, HVC, HVB and syphilis
serologies were negative. Increased alpha-1 and alpha-2 were detected on
protein electrophoresis. Determination of immunoglobulins and subclasses
were normal. Serum complement C3 and C4 normal.
Inflammatory syndrome decreased as well as neck tenderness. It was
decided to not introduce steroids due to spontaneous evolution. Broad
spectrum antibiotics were stopped after five days. Ultrasound control 7
days later showed diminished thickening infiltration. 1 month and half
later (45 days later) in April 2022 : CT scanner (FIGURE D) and
ultrasound showed clear regression of the periaortic infiltration at the
level of the aortic arch and the isthmus but persistent periaortic
thickening of the descending aorta. C reactive protein returned to
normal.
Hematologists collegially decided a therapeutic pause due to recent
events with no reintroduction of azacytidine nor venetoclax and
filgrastim. After 6 months of follow-up, she remains free from disease
progression with persistent asymptomatic moderate bicytopenia
(neutrophils 1200/mm3, platelets 40 000/mm3).