A 17-year-old male patient, with no relevant morbid history, who, two months before admission, presented with a high respiratory condition characterized by dysphonia, odynophagia, fever, night sweats and fever.
She received several antibiotics on an outpatient basis, without clinical improvement.
Computed axial tomography (CAT) of the paranasal sinuses showed pansinusitis and nasofibroscopy that reported "coastal laryngitis", indicating antibiotics and oral and inhaled corticosteroids.
Febrile fever was present at a temperature of 39°C and laryngeal stridor, and the patient was referred to the emergency department for hospitalization.
The patient was evaluated by an otorhinolaryngologist and a new endoscopy showed a crusted glottic lesion with decreased caliber of the laryngeal lumen.
Blood count at admission showed hematocyte 40%, Hb 13.5, leukocytes 4,700
ANC 2,700, normal platelets, sedimentation rate of 20 mm.
Antibiotic treatment, corticosteroids and nebulizations were started, with improvement of respiratory symptoms and remission of fever.
Seven days later a new endoscopic control was performed, which showed a decrease in crusted lesions with appearance of new granulomatous lesions.
Given these findings, it was decided to perform biopsy and culture in the operating room under direct laryngoscopy.
Intraoperatively, the patient developed profuse bleeding and fever.
Examinations taken in the operating room showed 37% hematocrit, 900 leukocytes and 74,000 platelets.
She was admitted to the surgical ICU and developed respiratory failure rapidly, requiring mechanical ventilation.
At this time, due to the appearance of pancit, the patient was evaluated by endoscopy, performing a bone marrow biopsy, aspirated for myelogram and flow cytometry.
In the following 48 h, the patient developed rapidly progressive multiple organ failure with respiratory and renal impairment and coagulopathy, associated with refractory shock to vasoactive drugs and antibiotic and antifungal coverage of broad-spectrum ventilation.
Bone marrow cytology showed 68% of medium to large blasts with thick granules in cytoplasm, while flow cytometry revealed 22% of NK cells with moderate pattern of intense CD56 and abnormally high CD56 membrane light scattering.
B and T lymphocytes were immunophenoidal normal.
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Computed tomography scans of the chest, abdomen and pelvis showed nodal involvement or in the most caudal midline.
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Biopsy of the glottic lesion performed in the operating room under direct laryngoscopy showed only necrotic tissue.
The pathological report of the bone marrow showed diffuse large NK cells (CD56 intensely positive by immunohistochemistry).
Given the nature of these findings and the extreme severity of the condition, pulses of methylprednisolone were immediately initiated at a dose of 1 g/day and prophylaxis of tumor lysis, with no response.
Two hours later, the patient died due to a completely refractory multiple organ failure.
