Norzagaray OF, Sosa QLS, Rubio JB, Alonso GD, Ramírez AJM, Sánchez SA, Coronado RAA, García MG, Anaya CJ, Arguelles EMF, Martínez VMI, Maldonado GJD, Balmaceda MA, Bernal GMA, Martínez LI, Trejo CDA, Soto GJI, Osuna DJA, Rojas ABL

Language: Spanish
References: 6
Page: 169-173
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ABSTRACT

Background: Aggressive NK cell leukemia is a lymphoproliferative disorder with high involvement of Epstein-Barr virus infection in its pathogenesis, with a higher incidence in the Asian population and young adults. It has an acute and aggressive clinical presentation, characterized by B symptoms, hepatosplenomegaly, lymphadenopathy, and disseminated intravascular coagulation. Diagnosis is based on cellular characteristics, clinical course, and poor prognosis, with a median survival of 2 months. Intermediate to large cells with basophilic cytoplasm, vacuoles, and nuclei are found in the bone marrow, irregular, open chromatin and prominent nucleoli. Immunophenotype: CD2+, CD16+, CD56+, CD3-, CD4-, CD7-, CD8-. Treatment is based on small clinical trials, with better results with regimens such as SMILE (dexamethasone, methotrexate, ifosfamide, etoposide, and L-asparaginase). Allogeneic hematopoietic cell transplanta- tion is the best treatment, showing a 2-year survival after the transplant of 38% vs 0% in those who did not receive a transplant.
Clinical case: A 23-year-old male patient with fever and diaphoresis of nocturnal onset, weight loss of 16 kg in 4 months, abdominal pain predominantly in the left upper quadrant and weakness of the extremities that made walking difficult, in whom NK cell leukemia was diagnosed.
Conclusions: NK cell leukemia is a rare disease in our environment. The clinicopathological characteristics are variable. Diagnosis is complicated by the lack of specific immunolabeling and karyotype.

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