Chronic lymphoproliferative disorder of natural killer cells C91.7

CLPD-Nk is an extremely rare hematologic disorder. It is characterized by a persistent increase (≥2 ×¹⁰⁹ /L, over > 6 months) in large granular lymphocytes of the NK cell type in peripheral blood. Only a few case series have been published to date. The disease is characterized by an indolent clinical course (Kurt H et al. 2018).

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The average age is from years (25-89 years) (Kurt H et al. 2018).

Cytopenias are conspicuous in 90% of cases. Bone marrow involvement by CLPD-NK ranged from 5-15%. Lung involvement is possible (Zhang J et al. 2021)

Mutations in the JAK/STAT pathway are detected in 80% of patients and affect STAT3 and JAK3.

The presence of mutations tended to correlate with the occurrence of other cytopenias (anemia/thrombocytopenia) and the need for treatment.

1. Jerez A et al. (2012) STAT3 mutations unify the pathogenesis of chronic lymphoproliferative disorders of NK cells and T-cell large granular lymphocyte leukemia. Blood 120:3048-3057.
2. Kurt H et al. (2018) Chronic lymphoproliferative disorder of NK-cells: A single-institution review with emphasis on relative utility of multimodality diagnostic tools. Eur J Haematol 100:444-454.
3. Kawakami Tet al. (2019) STAT3 mutations in natural killer cells are associated with cytopenia in patients with chronic lymphoproliferative disorder of natural killer cells. Int J Hematol109: 563-571.
4. Zhang J et al. (2021) Pulmonary infiltration as the initial manifestation of chronic lymphoproliferative disorder of natural killer cells: a case report and literature review. BMC Pulm Med 21:94.