A 70-year-old newly diagnosed HIV-positive Ugandan African woman presented with a three-month history of cough, fever, weight loss and drenching night sweats. Three weeks prior to admission she developed right sided chest pain and difficulty in breathing. There was no history of haemoptysis or bleeding from any site. She had occasional palpitations. There was no history of leg swelling, orthopnea or paroxysmal nocturnal dyspnoea. She had been vomiting for two days prior to admission, but had no oral sores or alteration in bowel habits. A review of the rest of her systems was unremarkable.
This was her first admission to hospital; she reported having been unwell for three months and had received treatment for cough from a nearby clinic. As she was newly diagnosed with HIV, she had not yet received cotrimoxazole or highly active antiretroviral therapy (HAART).
She was nulliparous, had never married but had had several sexual partners. She had been smoking a pipe (with tobacco); however, she denied alcohol consumption.
On examination, she was elderly, sick looking, with severe pallor, dehydration and wasting. She had no lymphadenopathy, jaundice, or edema. Her liver, spleen and kidneys were not palpable. She had no Kaposi sarcoma lesions on her skin or mucus membranes. She had tachycardia (92 beats per minute) with apparently normal heart sounds but she had a functional systolic murmur. Her blood pressure was 125/90 mmHg. She had tachypnoea (35 breaths per minute), reduced air entry and fine crackles on the right side of her chest.
Results of laboratory investigations are shown in table . A chest x-ray showed bilateral pleural effusions and subcutaneous emphysema. Echocardiography showed a mild pericardial effusion.
Results of laboratory investigations
On ultrasonography, her liver, spleen, kidney and pancreas were normal in size, shape and echo pattern. Her urinary bladder wall was normal. There was no ascites or lymphadenopathy. Bilateral large pleural effusions were confirmed. No solid tumor masses were present.
A sample of about 150 ml of pleural fluid was taken from her right hemithorax and sent to the Department of Pathology, Makerere University, College of Health Sciences, Kampala, Uganda, where it was cytocentrifuged. The sediment was made into a cell block and haematoxylin and eosin stained slides revealed a neoplastic proliferation of large lymphoid cells with round to irregular nuclei, prominent nucleoli, and varying amounts of vacuolated cytoplasm. There were immunoblastic, plasmablastic and anaplastic variants with bizarre, pleomorphic nuclei (Figure ). They included multinucleated and Reed-Sternberg-like cells. From these findings a preliminary diagnosis of PEL was made.
Figure 1 At light microscopy, the sample consisted of a frankly neoplastic population provided with plasmablastic and/or anaplastic morphology (Figure 1A), which turned out CD3-, CD20- (Figure 1B), CD79a-, CD45+ (Figure 1C), CD38+, CD30+, IRF4+, LANA-1+ (Figure (more ...)
Since immunohistochemistry is not routinely available in Uganda, it was carried out at the Institute of Haematology and Oncology "L and A Seragnoli", Bologna University School of Medicine, Bologna, Italy. The alkaline phosphatase anti-alkaline phosphatase method and the primary antibodies listed in table were used. In situ hybridization was also used for detection of EBV. The tumor cells were CD45+ (Figure ), CD30+, CD38+, HHV-8 LANA-1+ (Figure ); but were negative for CD3-, CD20- (Figure ), CD19-, and CD79a- and EBV RNA+ (Figure inset) on in situ hybridization. CD138 and Ki-67 were not evaluable. These findings confirmed the diagnosis of PEL. The patient tested HIV-positive and her CD4 cell count was 127/μL.
Primary antibodies used for diagnosis
Treatment: She was initially rehydrated with normal saline and 5% dextrose, and later received a blood transfusion. She also received allopurinol, ceftriaxone and metronidazole. After she had stabilized, she was given the first course of chemotherapy with a CHOP protocol (cyclophosphamide 750 mg/m2, Adriamycin (doxorubicin) 50 mg/m2 and Oncovin (vincristine) 14 mg/m2 on day one and prednisolone 100 mg on days one to five, repeated every 21 days). In addition, she received omeprazole, metoclopramide and dexamethasone. She registered some improvement after the first course of chemotherapy and was allowed home. She was discharged through the Infectious Disease Institute clinic to be initiated on HAART. She was due to return for subsequent anti-cancer treatment but died two days after discharge from our hospital.