Abstract
Extranodal natural killer/T-cell lymphoma of the nasal cavity is a rare malignancy with poor overall prognosis. We report the case of a rapidly fatal natural killer/T-cell lymphoma of the nasal cavity complicated by the concominant development of hemophagocytic lymphohistiocytosis. This disorder is marked by clinical findings such as fever and splenomegaly, a multitude of abnormal laboratory findings, and a profound proliferation of circulating macrophages. Left untreated, multi-organ failure and death are common. Prompt diagnosis is essential to the successful management of this disorder and for subsequent recovery.
1
Introduction
Natural killer (NK)/T-cell lymphoma is a rare lymphoproliferative disorder with an aggressive nature and poor overall prognosis. Nonetheless, NK/T-cell lymphoma is the most common form of sinonasal lymphoma and should be included in the differential diagnosis for any destructive process involving the nasal cavity or midline facial anatomy. A strong association between NK/T-cell lymphoma and Epstein-Barr virus (EBV) status has been confirmed in several studies. These lesions comprise up to 8% of all non-Hodgkin lymphomas and are more common in individuals from Central and South America and particularly Asia . In the United States and other Western societies, NK/T-cell lymphoma is quite rare, likely representing less than 2% of all non-Hodgkin lymphomas .
Hemophagocytic lymphohistiocytosis (HLH), commonly known as hemophagocytic syndrome, is a rare clinical entity characterized by fever, pancytopenia, splenomegaly, and hemophagocytosis in the bone marrow, liver, or lymph nodes. Hemophagocytic lymphohistiocytosis is associated with a variety of viral, bacterial, and fungal infections and is seen in some malignancies and autoimmune disorders. Although uncommon, HLH is a serious illness and is almost uniformly fatal if left untreated.
We describe a case of NK/T-cell lymphoma that presented as a destructive mass involving the paranasal sinuses and nasal septum. The patient presented with a history of refractory sinusitis and findings consistent with HLH. Standard treatment was ultimately initiated after confirmation of the diagnosis by operative biopsy.
2
Case report
A 21-year-old white man presented with a 6-week history of fevers, progressive nasal obstruction, and mucopurulent nasal drainage. He was found to be profoundly pancytopenic with a white blood cell count of 1570 and absolute neutrophil count of 923. Platelet count on presentation was 35, and hemoglobin level was 10.6. He was also noted to have elevated liver function tests (bilirubin, 8.3; aspartate aminotransferase, 104; alanine aminotransferase, 187). Lactate dehydrogenase was 1252. A maxillofacial computed tomography (CT) showed complete opacification of the left maxillary, frontal, and sphenoid sinus and near complete opacification of the ethmoid air cells bilaterally ( Fig. 1 ). Also noted was osteolysis of the nasal septum, left nasal bones, and the nasal process of the left maxillary bone. Nasal endoscopy revealed extensive soft tissue necrosis, insensate mucosa, and moderate purulence with few appreciable anatomical landmarks. Tissue biopsy of the nasal septum and lateral nasal sidewall was taken at bedside and showed extensive inflammation and necrosis with no fungal elements. The patient was taken to the operating room where biopsies showed angiocentric inflammation without fungal elements, consistent with extranodal NK/T-cell lymphoma. Further histopathologic examination revealed atypical lymphoid cells positive for CD56, CD8, and CD3 and negative for CD4 and CD20 ( Fig. 2 ).
Postoperatively, the patient was transferred to the hematology/oncology service for further management. Subsequent viral screens were negative for hepatitis and HIV but markedly positive for EBV. Positron emission tomography–CT showed moderate-to-intense uptake in the left medial maxillary wall, medial canthus, ethmoid sinus, nasal septum, and nasopharynx. Mild-to-moderate uptake was noted throughout the enlarged spleen. Bone marrow biopsy showed marrow involvement by his NK/T-cell lymphoma as well as evidence of HLH ( Fig. 3 ). Because of the patient’s marginal liver function, standard CHOP (doxorubicin, cyclophosphamide, viscristine, prednisone) therapy was not initiated. He was instead treated with cyclophosphamide, rituximab, and prednisone. The patient’s renal and hepatic function continued to decline, and he progressed to respiratory failure over subsequent days. Shortly thereafter, the family decided to discontinue further measures; and the patient died 6 weeks after initial presentation.
2
Case report
A 21-year-old white man presented with a 6-week history of fevers, progressive nasal obstruction, and mucopurulent nasal drainage. He was found to be profoundly pancytopenic with a white blood cell count of 1570 and absolute neutrophil count of 923. Platelet count on presentation was 35, and hemoglobin level was 10.6. He was also noted to have elevated liver function tests (bilirubin, 8.3; aspartate aminotransferase, 104; alanine aminotransferase, 187). Lactate dehydrogenase was 1252. A maxillofacial computed tomography (CT) showed complete opacification of the left maxillary, frontal, and sphenoid sinus and near complete opacification of the ethmoid air cells bilaterally ( Fig. 1 ). Also noted was osteolysis of the nasal septum, left nasal bones, and the nasal process of the left maxillary bone. Nasal endoscopy revealed extensive soft tissue necrosis, insensate mucosa, and moderate purulence with few appreciable anatomical landmarks. Tissue biopsy of the nasal septum and lateral nasal sidewall was taken at bedside and showed extensive inflammation and necrosis with no fungal elements. The patient was taken to the operating room where biopsies showed angiocentric inflammation without fungal elements, consistent with extranodal NK/T-cell lymphoma. Further histopathologic examination revealed atypical lymphoid cells positive for CD56, CD8, and CD3 and negative for CD4 and CD20 ( Fig. 2 ).
