We present the clinical case of a 42 year old male, with no drug allergies, with a personal history of infectious mononucleosis in childhood, fracture of the left arm 5 years ago, morbid obesity with hepatic steatosis treated by bariatric surgery.
He came to the Otorrolaryngology department for a few weeks of left nasal obstruction, with repeated episodes of purulent rhinorrhoea, with a sensation of left nasal packing, accompanied by an intranasal mass and a left submandibular tumour. Simultaneously, the patient was referred to the haematology department for repeated asymptomatic thrombocytosis in the blood tests. He was seen in the haematology department and, with the suspicion of essential thrombocythemia, a bone marrow aspirate/biopsy was performed, showing a platelet count of 487,000/ul in the haemogram.
The patient underwent a biopsy of the intranasal mass, which was completed with an incisional biopsy of the left submandibular tumour. The cervico-facial CT scan showed a mass of soft tissue in the left submaxillary cell, with areas of necrosis or abscessation, 3 cm in greatest diameter; accompanied by an adjacent locoregional adenopathic component of probably inflammatory nature. There was also a second lesion in discontinuity with the previous one, corresponding to a left paralateronasal cyst.
Biopsy of the intranasal lesion consisted of an infiltrate of lymphoid neoplastic cells with a diffuse growth pattern, no 'starry sky' images, and a moderate mitosis rate. The neoplastic cells are of intermediate size, with discretely irregular nuclei and moderate pleomorphism. The immunophenotype of the neoplastic cells determined by immunohistochemistry was: CD45+/cCD3+/Granzime B+/CD56+, CD20-/CD4-/CD8-/LMP1(EBV)-/Perforin-. Biopsy of the submandibular tumour showed massive infiltration by intermediate to large lymphoid neoplastic cells with nuclear irregularities and coarse, irregular and sparse azurophilic granulation. The immunophenotype is identical to that of the intranasal lesion. The EBER(EBV) determination was positive, there was polyclonal re-enhancer of T-lymphocyte receptor gamma chain (TCR) genes and immunoglobulin heavy chain (IgH) genes. This was compatible with a diagnosis of extranodal nasal type NK lymphoma.
The patient was immediately sent to the medical oncology department for evaluation. The patient was in very good general condition with a performance status of 0, with no B symptoms and asymptomatic, except for the symptoms of left nasal obstruction. The extension study was completed with thoraco-abdominal computed tomography, which showed no evidence of distant dissemination, bone marrow biopsy already requested due to suspicion of essential thrombocythemia, biochemistry with renal and hepatic profile, proteinogram, LDH and serum beta-2-microglobulin.
Bone marrow examination showed normocellular parenchyma (3/5), with moderate megakaryocytic hyperplasia with elements in all maturational stages, some of them with hypertrophic appearance, with the remaining series preserved. There was no evidence of myelofibrosis, parasites or granulomas. There was also no lymphomatous infiltration. The bone marrow study does not support (nor does it absolutely rule out) the diagnosis of essential thrombocythemia.
LDH and beta-2-microglobulin, as well as the rest of the biochemical parameters were in the normal range. Serology for HIV, HBV and HCV were negative. Haemoglobin and leukocyte counts were normal, with an unaltered leukocyte formula, with only a thrombocytosis of 487,000/ul standing out.
With the diagnosis of extranodal NK lymphoma nasal type in localised stage with reactive thrombocytosis, the patient immediately started treatment with polychemotherapy followed by sequential radiotherapy, deciding to carry out an autologous bone marrow transplant as the first line of treatment.