DOI: 10.1097/QAD.0b013e32830fbd91
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PMID: 18753872
Issn Print: 0269-9370
Publication Date: 2008/09/12
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Excerpt
In the present review, we report three additional cases of HIV-associated ALCL, all presenting in African–Americans with advanced extranodal disease. The clinical features, immunophenotype and molecular profile of our patients are summarized in Table 1, together with previously published cases from the literature. Interestingly, our cases were difficult to diagnose and often required extensive work up, including multiple biopsies. The CD4 cell count and viral load were <44 cells/μl and 600 000 copies/ml, respectively. The clinical course was complicated by opportunistic infections and lethality within 1 year despite our best efforts. The duration of HIV infection preceding the diagnosis of ALCL was 6, <1 and 8 years, respectively, for cases one, two and three (Table 1).
Case one presented with left thigh pain, underwent a muscle biopsy revealing atrophic muscle, and was diagnosed with pyomyositis and treated with ambulatory antibiotics. Readmission for persistence of pain and further work up, including computed tomography (CT) scans and bone marrow biopsy, showed pleural and pericardial effusions with no lymphadenopathy. A repeat open muscle biopsy established ALCL. Septicemia with vancomycin-resistant Enterococcus faecalis and Mycoplasma avium was successfully treated with antimicrobials. However, rapid clinical deterioration made the patient intolerant to chemotherapy or radiation therapy, and the patient expired 1.5 months after the diagnosis.
Case two presented with sudden onset of sharp bilateral lower back pain, 15-pound weight loss, night sweats and generalized weakness for the past 2 months. A CT scan revealed multiple lytic lesions in the ribs, thoracic lumbar spine and pelvis. An infectious cause was ruled out. Ultrasound-guided core biopsies of the liver, rib and bone marrow were nondiagnostic. Finally, a laparoscopic liver wedge biopsy showed ALCL. Radiation therapy was initiated, with a plan for subsequent cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) chemotherapy. However, rapid clinical deterioration, including progressive nosocomial pneumonia and ileus, precluded chemotherapy, and the patient expired 1 month after the diagnosis.
Case three presented with persistent fatigue, fevers, chills and sweats. Full work up revealed ALCL involving bone marrow and lymph nodes (mediastinal and retroperitoneal). Satisfactory response to CHOP chemotherapy showed a marked decrease in lymphadenopathy and disappearance of marrow involvement. Because of adriamycin-related cardiomyopathy, doxorubicin was replaced with etoposide for the sixth cycle. Readmission for cytomegalovirus retinitis was necessary 1 month after discharge. Progressive dyspnea developed, and a CT scan demonstrated bibasilar air space disease with pleural effusions, pericardial effusion and no lymphadenopathy. Despite extensive supportive therapy, he expired in the hospital 1 year after the diagnosis.
Morphologically, all three cases showed atypical large lymphocytes with abundant cytoplasm and pleomorphic nuclei with prominent nucleolus, consistent with the common variant of ALCL. They were CD30 positive, anaplastic lymphoma kinase (ALK) negative, Epstein–Barr virus (EBV) negative and some T cell markers positive. In addition, these cases were negative for the t(2;5) translocation by reverse transcriptase PCR, in agreement with the ALK negativity by immunohistochemistry.
The association of ALCL with HIV has been reported previously [2–15]. However, some reported cases were CD30 positive B-cell lymphomas associated with EBV showing downregulation of B-cell antigens [9–12,16].
