The patient was an 18-year-old black woman with a history of weakness, weight loss, productive cough, and night fever for 3 months who was admitted to the Hospital Central de Maputo. She also described abdominal distension and left flank pain. On admission, the patient presented with these symptoms and was found to have moderate anemia, thrombocytopenia, hypoalbuminemia, peripheral generalized lymphadenopathy, and splenomegaly. Testing for Mycobacterium tuberculosis in sputum was negative. She was treated with broad-spectrum antibiotics. Ultrasonography confirmed grade IV homogenous splenomegaly and showed para-aortic lymphadenopathy and liver features consistent with hepatic steatosis.
The HIV test by was consistently negative for HIV-1 and -2 (serologic test with Determine HIV1/2, Uni-Gold HIV-1/2 and SD BioLine HIV 1/2; molecular test by PCR for HIV-1 was also performed and was negative, and viral load for HIV-1 was undetectable). CD4+ count performed during admission was 579/ ml blood. A clinical diagnosis of lymphoma was suspected and the case was subjected to FNAC.
During admission, the patient progressed to have hallucinations, headache, and nuchal rigidity. Her disease progressed to multiorgan failure and she died. No autopsy was performed.
Two FNAC were performed in the cervical lymph node. Aspirations were obtained using a 0.6-mm-diameter (23-gauge) needle attached to a 10-cc syringe held in a standard metal syringe holder. The material obtained was placed on standard glass slides, thinly smeared, and air-dried for May Grunwald Giemsa staining.
The cytological examination showed bloody smear disclosing a mixed population of isolated large and small lymphocytes. The large lymphocytes presented non-cleaved nuclei with prominent nucleoli and a rim of basophilic cytoplasm. Isolated and groups of overlapping spindle cells with spindle nuclei were also found scattered throughout the smears, sometimes in a cohesive cluster with radial arrangements and nuclear crush artifacts. Because of the presence of 2 distinct patterns, FNAC was repeated. This showed the same results, confirming the cytological picture. On the basis of these cytological features, a diagnosis of lymphoproliferative process compatible with lymphoma with possible simultaneous fusocellular tumor was made and excisional biopsy was done.
A tissue sample obtained by excisional biopsy from the same cervical lymph node as previously aspirated. It was fixed in 10% formalin, embedded in paraffin, cut into 4 mum sections and hematoxylin and eosin stained.
The histological examination showed complete distortion of the lymph node architecture (Figure 1A), mostly by a lymphoproliferative lesion composed of a diffuse population of large lymphoid cells displaying vesicular nuclei with prominent nucleoli and high mitotic index, intermingled with abundant small lymphocytes and histiocytes (Figure 1B and 1D); the remaining lymph node was occupied by a vascular lesion composed of a discrete proliferation of slit-like vessels, filled with red blood cells, surrounded by slightly atypical spindle cells with 12 mitoses/10 high-power fields (Figure 1C and 1D), focal hyaline globules, and hemosiderin deposits.
Immunohistochemical analyses were performed by the avidin-biotin-peroxidase method with the Ventana Benchmark autostaining system on formalin-fixed, paraffin-embedded sections from the same sample. Stains were performed with the following antibodies: CD31, CD34, CD20, CD3, CD15, and CD30.
The immunohistochemical study of the vascular lesion showed expression of CD31 and CD34 in the atypical spindle cells (Figure 1E and 1F). In the lymphoproliferative lesion, the large lymphoid cells had clear-cut membrane CD20 positivity (Figure 1G), whereas the small lymphocytes expressed membrane CD3 positivity (Figure 1H). CD15 and CD30 were not expressed by any of the lymphoid cell types.
The morphological and immunophenotypical features of the aforementioned lesions are consistent with the co-existence of a T cell-rich LBCL and a KS lesion in the same lymph node.
We tested for DNA from KSHV/HHV-8 and EBV by PCR amplification of DNA obtained from areas of distinct types selected in the paraffin block. These yielded KSHV/HHV-8 and EBV DNA in the sampled lymph node in the areas of KS and LBCL, respectively.