An uncommon case of lymphoma was reported when a 53-year-old man came to the emergency room with chief complaints of fever, shortness of breath, and rapidly progressive cutaneous lesion, all of which were present for about one month at the time of presentation.
On physical examination of the limbs, trunk, and back, painful, tender, indurated nodules were found. The nodules’ surroundings had purplish discolouration. (Panel A).
Laboratory investigations were carried out which gave the following results:
White blood cell count (WBC) of 3000 per mm3
Low platelet count of 36,000 per mm3
An increased ferritin level of > 40,000 ng per mm3
Bone marrow aspiration and skin biopsy were conducted.
The former showed phagocytosis of the blood cells by the macrophages/histiocytes, called hemophagocytosis and the latter revealed natural killer T-cells. Positron-emission tomography (PET) scan also pointed in the same direction.
The patient’s Epstein–Barr viral load was 18,486 U per millilitre.
A diagnosis of a rare neoplasm, called extranodal natural killer (NK) T-cell lymphoma, was made.
Extranodal means growing outside the lymphatic system and Natural killer cells are a type of T-cells that kill viruses and cancer cells, so this lymphoma is the unregulated excess growth of the NK cells outside the lymphatic system, most commonly in the nose. It is a subtype of non-Hodgkin lymphoma and is found to be associated with Epstein–Barr virus.
Although rare (UK gets around 20 patients of it per year), it is a rapidly growing aggressive lymphoma.
It usually affects men in their 50s and 60s, starting off as a lump in their nose and sinuses giving symptoms of nasal congestion, difficulty breathing, mouth breathing, and nose bleeds. Eventually, it may spread to adjacent structures, clinically evident as swollen face, sore throat, voice changes, facial and cheek involvement.
Due to its early and rapid destructive power, prompt diagnosis and treatment are the crucial steps!
The pathological analysis is indispensable in confirming the diagnosis of NK/T-cell lymphoma. Concurrently, PCR is used to detect EBV. For managing NK/T-cell lymphoma, chemotherapy is the fundamental strategy. For localised disease, radiation can be coupled with chemotherapy.
Radiation therapy alone was found to be associated with higher recurrence rates.
Since the disease is quite rare, clinical trials for its treatment are of a rare occurrence. Nevertheless, certain chemotherapeutic agents have been tested, theses regimens include
- AspaMetDex: L-asparaginase, methotrexate and dexamethasone.
Allogenic stem cells transplantation may eliminate the diseased cells completely, that is called remission in medical jargon.
The patient in the discussion here underwent systemic chemotherapy for six months with gemcitabine, cisplatin, dexamethasone, and asparaginase.
At the 8-month follow-up, the patient had substantially recovered clinically with a spectacular improvement in his skin manifestation (Panel B). investigations revealed undetectable Epstein–Barr virus levels and the PET scan showed resolved lesions.
Kuan-Yu Chi, M. a.-N. (2020, February 06). Extranodal Natural Killer T-Cell Lymphoma. Retrieved from The New England Journal of Medicine: https://www.nejm.org/doi/full/10.1056/NEJMicm1910000
Reviewed by Prof Tim Illidge, P. o. (2019, August). Extranodal NK/T-cell lymphoma, nasal type. Retrieved from Lymphoma Action: https://lymphoma-action.org.uk/types-lymphoma-non-hodgkin-lymphoma-t-cell-lymphomas/extranodal-nkt-cell-lymphoma-nasal-type
Metgud, R. S., Doshi, J. J., Gaurkhede, S., Dongre, R., & Karle, R. (2011). Extranodal NK/T-cell lymphoma, nasal type (angiocentric T-cell lymphoma): A review about the terminology. Journal of oral and maxillofacial pathology: JOMFP, 15(1), 96–100. https://doi.org/10.4103/0973-029X.80016