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| IMAGE IN HEMATOLOGY |
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| Year : 2017 | Volume
: 8
| Issue : 2 | Page : 82-83 |
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A “Killer Disease” Living up to its name
Fahad Alenazi1, Nasir A Bakshi MD, FCAP, FASCP 2
1 Department of Medical Oncology, KFSH&RC, Riyadh, KSA
2 Department of Pathology and Laboratory Medicine, KFSH&RC, Riyadh, KSA
| Date of Web Publication | 17-Jul-2017 |
Correspondence Address:
Nasir A Bakshi
Hematopathology Section, Department of Pathology and Laboratory Medicine, KFSH&RC, PO Box 3354, Riyadh - 11211
KSA
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/joah.joah_35_17
A 33-year old female presenting with hepatosplenomegaly and pancytopenia showed atypical lymphoid cells in peripheral blood and bone marrow. Immunophenotyping confirmed the diagnosis of Aggressive NK-cell leukemia. Despite treatment by chemotherapy and targeted therapy she deteriorated and died only 7 months after diagnosis.
Keywords: NK-cell leukemia, Aggressive, bone marrow
How to cite this article:
Alenazi F, Bakshi NA. A “Killer Disease” Living up to its name. J Appl Hematol 2017;8:82-3 |
| Case Report | |  |
A 33-year-old female was admitted with hepatosplenomegaly and pancytopenia with abnormal circulating cells in peripheral blood [[Figure 1]a]. She had no lymphadenopathy or any previous history of hematologic abnormality. Bone marrow test was performed, which confirmed abnormal cellular lymphoid infiltrate comprising medium-to-large cells with moderate cytoplasm, remarkable for the presence of large azurophilic granules, resembling those seen in peripheral blood. | Figure 1: (a) Peripheral blood smear, Wright-Giemsa stain X 1000; (b) Bone marrow trephine biopsy, EBER-1 in-situ hybridization X 500
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Bone marrow trephine biopsy showed the same neoplastic cells in diffuse and interstitial pattern. Flow immunophenotyping showed that these cells were positive for CD2, CD7, CD8, CD16, CD57, and CD94 [NK (Natural Killer)-cell marker] along with dim partial CD56. Surface CD3, CD5, and T-cell receptor alpha-beta/gamma-delta were negative. Epstein–Barr virus in situ hybridization on trephine biopsy, [EBER-1 Epstein-Barr virus Encoded RNA], showed diffuse and strong positivity in the tumor cells [[Figure 1]b]. A diagnosis of aggressive NK-cell leukemia was made. She received methotrexate-based chemotherapy (SMILE, dexamethasone, methotrexate, ifosfomide, L-asparginase, etoposide protocol) but had persistent disease. She then received platinum-based therapy with persistent disease, following which she was given a third-line therapy with Daratumumab (anti-CD38) but her general condition continued to deteriorate, and she died only 7 months after the diagnosis of her disease.
[Figure 1]
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