Bone Lymphoma Caused by NK/T-cells Leading to Hemophagocytic Lymphohistiocytosis: A Real-World Study Case
In the realm of paediatric oncology, a particular concern arises when NK/T-cell lymphoma (NKTCL), an aggressive subtype of non-Hodgkin lymphoma (NHL), is complicated by hemophagocytic lymphohistiocytosis (HLH). This combination presents a challenging clinical course, with a significantly worse prognosis compared to other childhood lymphomas.
NKTCL, known for its poor response to standard treatments and rapid progression, is among the most aggressive forms of NHL. Even when the lymphoma achieves remission, recurrence rates are high, and the disease is often refractory to standard chemotherapy regimens. In the broader category of peripheral T-cell lymphomas, the five-year survival rate is only 30–40%. However, NKTCL, especially the nasal type, is associated with even poorer outcomes, particularly in advanced or refractory cases [1][4].
The presence of HLH further exacerbates the situation. HLH is a syndrome of excessive immune activation that can occur as a complication of malignancies, including lymphomas. It is associated with high morbidity and mortality. The presence of HLH in the setting of NKTCL significantly worsens prognosis. HLH causes multi-organ dysfunction, cytokine storm, and accelerated clinical deterioration, often leading to rapid death if not aggressively managed [2].
Treating both the underlying lymphoma and the HLH is extremely difficult. The intense inflammation of HLH can complicate chemotherapy administration and increase the risk of treatment-related toxicity.
A case study illustrates the severe impact of this combination. A child was initially admitted to the hospital with a diagnosis of HLH. Over the following months, the child was repeatedly hospitalized for fever and continued the previous chemotherapy regimen. After 17 days, laboratory results showed signs of a possible hematological malignancy, and a CT scan revealed bone destruction in the middle and lower humerus accompanied by a soft tissue mass, consistent with lymphoma. Pathological examination confirmed the presence of NK/T-cell lymphoma, and the child was clinically diagnosed with hemophagocytic syndrome and treated with intrathecal chemotherapy [3].
In light of these challenges, early recognition and aggressive therapy are critical. Prompt diagnosis and immediate initiation of both lymphoma-directed and HLH-directed therapies are essential, though outcomes remain dismal [1]. Given the high mortality, early involvement of palliative care teams is important to optimize quality of life and support for families.
In conclusion, children diagnosed with NKTCL complicated by HLH face a very poor prognosis, with survival rates significantly lower than those for other childhood lymphomas. The combination of an aggressive, chemotherapy-resistant malignancy and a life-threatening hyperinflammatory syndrome leads to high mortality, despite intensive treatment efforts [1][3][4]. Early, multidisciplinary intervention is essential, but curative outcomes are rare.
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