Sultan Qaboos University Med J, May 2015, Vol. 15, Iss. 2, pp. e297–298, Epub.
Submitted 10 Sep 14
Accepted 6 Nov 14

Departments of 1Haematology and 2Medicine, Sultan Qaboos University Hospital, Muscat, Oman
*Corresponding Author e-mail: arwa.alriyami@gmail.com

عرض نادر ملريض عماين مصاب بلمفوما اخلاليا اللمفاويه الطبعيه القاتله
اروى الرياميه، خليل الفار�سي، �ساهيمه املعمريه، احمد الفرقاين، مرت�سى اخلابوري

Rare Presentation of Aggressive T/Natural Killer 
Lymphoproliferative Disorder in a Leukaemic 

Phase in an Omani Patient
*Arwa Z. Al-Riyami,1 Khalil Al-Farsi,1 Sahimah Al-Mamari,1 Ahmed Al-Farqani,2 Murtadha Al-Khabori1

Natural killer (NK) cells are cytotoxic cells that target tumour, bacteria- and virus-infected cells.1 
They produce cytokines such as interferon-γ and 
tumour necrosis factor-α. Morphologically, NK 
cells appear as large granular lymphocytes with 
abundant pale cytoplasm with azurophilic granules. 
They are negative for surface CD3; positive for 
CD16, CD56 and CD57, and variably express some 
T cell antigens like CD2, CD7 and CD8.2 NK and T/NK 
lymphomas are very rare, and are mainly described in 
areas of East Asia, where they account for 7–10% of all 
lymphomas.3 They are commonly associated with the 
presence of Epstein-Barr virus in the neoplastic cells. 
To the best of the authors’ knowledge, no cases from 
the Gulf region have been reported in the medical 
literature. Herein, is a description of a presentation of 
this rare disease in an Omani patient. 

A 46-year-old Omani female with schizophrenia 
presented to the Department of Haematology at the 
Sultan Qaboos University Hospital in Muscat, Oman, 
in January 2013. She was referred for evaluation of 
bilateral adrenal masses, abdominal lymphadenopathy, 
hepatosplenomegaly, transaminitis and elevated 
lactate dehydrogenase levels at 540 u/L (normal range: 
135–225 u/L). She had a two-week history of vomiting, 
abdominal pain and jaundice. A complete blood count 
revealed a total white cell count of 17.0 x 109/L (normal 
range: 2.4–9.5 x 109/L), lymphocytosis of 11.4 x 109/L 
(1.2–3.8 x 109/L), haemoglobin of 6.6 g/dL (11.0–14.5 
g/dL) and a platelet count of 48 x 109/L (150–450 x 
109/L). The patient’s blood film showed medium to 
large lymphoid cells with prominent nucleoli and deep 
basophilic cytoplasm with occasional vacuolation and 
cytoplasmic granulation [Figure 1].

online interesting medical image 

 
Figure 1: Blood film showing large lymphoid cells with prominent nucleoli and deep basophilic cytoplasm.



Rare Presentation of Aggressive T/Natural Killer Lymphoproliferative Disorder in a Leukaemic Phase in an Omani Patient

e298 | SQU Medical Journal, May 2015, Volume 15, Issue 2

negative NK lymphomas are very rare, with only 
three cases described in the medical literature from 
East Asia of extranodal NK/T cell lymphoma-nasal 
type, with nasal cavity involvement.3 The current case 
represents the first described case of CD45-negative 
T/NK lymphoproliferative disorder in the leukaemic 
phase.

References
1. Spits H, Lanier LL, Phillips JH. Development of human T and 

natural killer cells. Blood 1995; 85:2654–70. 

2. Kwong YL. Natural killer-cell malignancies: Diagnosis 
and treatment. Leukemia 2005; 19:2186–94. doi: 10.1038/
sj.leu.2403955.

3. Au WY, Ma SY, Chim CS, Choy C, Loong F, Lie AK, et al. 
Clinicopathologic features and treatment outcome of mature 
T-cell and natural killer-cell lymphomas diagnosed according to 
the World Health Organization classification scheme: A single 
center experience of 10 years. Ann Oncol 2005; 16:206–14. doi: 
10.1093/annonc/mdi037.

Peripheral blood flow cytometry showed that T/
NK cells made up 25–30% of the patient’s blood count. 
The T/NK cells were uniformly positive for CD56, 
CD16, CD2, CD7, CD8 (dim) and α/β. However, 
they were negative for surface CD3, CD4, CD5 and 
γ/δ. Interestingly, the cells were also CD45-negative. 
The diagnosis of T/NK lymphoproliferative disorder 
in a leukaemic phase was made and a bone marrow 
aspiration and biopsy procedure were planned. 
However, the patient deteriorated rapidly with 
increasing oxygen requirements. She eventually went 
into cardiac arrest and died.

Comment

This case illustrates the leukaemic phase of a 
disseminated T/NK lymphoproliferative disorder. 
The presentation and the rapid deterioration of the 
patient were consistent with aggressive disease. CD45-