Primary mediastinal large B cell lymphoma in a woman who is human immunodeficiency virus positive presenting with superior vena cava syndrome: a case report
© The Author(s). 2017
Received: 18 August 2016
Accepted: 5 January 2017
Published: 11 February 2017
The risk of non-Hodgkin lymphoma is increased 200-fold in individuals seropositive for human immunodeficiency virus compared to those free from human immunodeficiency virus. Human immunodeficiency virus-associated non-Hodgkin lymphoma is known for its atypical presentation, aggressive ability, widespread involvement, poor response to chemotherapy, and high relapse potential which makes both the diagnosis and management a difficult undertaking especially in resource-poor settings.
We report a case of primary mediastinal large B cell lymphoma in a 46-year-old woman of African descent who is human immunodeficiency virus positive who presented with symptoms of superior vena cava syndrome. Her past medical history was remarkable for a 23-year history of systemic hypertension and a 10-year history of human immunodeficiency virus infection. A physical examination revealed an underweight woman with right-sided facial, neck, upper limb, and trunk swelling together with distended veins on her chest and abdomen draining downwards. A respiratory examination revealed a reduced chest expansion, stony dull percussion note, and absent breath sounds on her entire right side with a left-sided tracheal deviation. She had a CD4 count of 146 cells/μL. A chest X-ray revealed a homogenous opacification on her right side with a left-sided tracheal deviation while a computed tomography scan of her chest revealed a solid mass on her right side. An echocardiogram showed a huge well-circumscribed mass (4.6×3.3 cm) with spontaneous echocardiographic contrast compressing her heart inferiorly. She had severe pulmonary hypertension (right ventricular systolic pressure 58 mmHg) but preserved left ventricular systolic function, no thrombus was seen, and her pericardium was normal. A computed tomography angiography of her aorta ruled out an aortic aneurysm. Finally, she underwent mediastinoscopy and a direct biopsy of the mass was taken for histopathology. Hematoxylin and eosin staining demonstrated a dense lymphoid infiltrate of large malignant cells with pleomorphic nuclei in clusters, compartmentalized by fine bands of fibrosis, and frequent mitoses were present. A diagnosis of mediastinal large B cell lymphoma was reached.
The presence of a mediastinal widening coupled with a history of unintentional yet significant weight loss in an individual who is human immunodeficiency virus seropositive should raise an index of suspicion for lymphomas and warrant aggressive investigations and timely management.
KeywordsMediastinal large B cell lymphoma Non-Hodgkin lymphoma HIV infection AIDS Superior vena cava syndrome Case report
The lung and mediastinum are often targets of human immunodeficiency virus (HIV) infection and severe thoracic complications are not uncommon. Differentiating the respiratory clinical manifestations of acquired immunodeficiency syndrome (AIDS) as infectious or neoplastic may pose a diagnostic challenge especially in resource-limited settings. Non-Hodgkin lymphoma (NHL) is among the AIDS-defining illnesses and in approximately 5 % of cases it is the initial manifestation of AIDS . The risk of NHL is increased 200-fold in individuals infected with HIV compared to those free from it and up to 14 % of patients with AIDS with comorbid NHL present with a pulmonary involvement [2, 3].
Diffuse large B cell lymphomas (DLBCLs) are the commonest lymphomas in an HIV setting. Mediastinal large B cell lymphoma (MLBCL) is a subtype of DLBCL arising in the mediastinum. MLBCL is fast growing and aggressive usually presenting with symptoms of superior vena cava syndrome and a localized mediastinal mass with characteristic morphologic and genotypic features . The introduction of highly active antiretroviral therapy (HAART) has resulted in longevity of life and a decreased incidence of lymphomas; however, the overall survival of patients who are HIV seropositive with NHL remains inferior to the survival of their general population counterparts . We report a case of primary MLBCL in a 46-year-old woman of African descent who is HIV positive who presented with symptoms of superior vena cava syndrome.
A physical examination revealed an underweight woman with a body mass index (BMI) of 16.8 kg/m2, with right-sided facial, neck, upper limb, and trunk swelling together with distended veins on her chest and abdomen draining downwards. Her blood pressure was 138/90 mmHg, respiratory rate 22 breaths/minute, oxygen saturation 97 % on room air, and she had a temperature of 37.1 °C. A respiratory examination revealed reduced chest expansion, stony dull percussion note, and absent breath sounds on her entire right side with a left-sided tracheal deviation. An apex beat was felt at the ninth intercostal space along the mid clavicular line and auscultation revealed a murmur of tricuspid regurgitation. A local breast examination revealed an edematous right breast that was otherwise normal. A musculoskeletal assessment revealed reduced muscle bulkiness in all four limbs but normal tone, power, and reflexes. The lymph nodes in her cervical, axillary, and femoral regions were not palpable.
Although the diagnosis was finally reached, she died of respiratory failure a few days before the biopsy results were available.
Lymphomas are the second commonest malignancies after Kaposi’s sarcoma in the HIV-infected subpopulation. It is estimated that one out of five patients who are HIV seropositive will develop NHL in their lifetime . HIV-associated NHL and in particular DLBLC are known for their atypical presentation, aggressive ability, widespread involvement, poor response to chemotherapy, and high relapse rates which complicates both the diagnosis and management . The mediastinum is a common extranodal but a rare primary site for AIDS-related NHL and the occurrence of such malignancy is almost always an indicator of advanced HIV infection. Distinctively, HIV-related MLBCL displays a lower frequency of mediastinal adenopathy compared to its HIV-free counterparts. Although fatal if not treated, MLBCL is often curable with intensive chemotherapy combinations including Cytoxan (cyclophosphamide), Adriamycin (doxorubicin), vincristine, and prednisone (CHOP). The extent of disease, and extranodal and bone marrow involvement are key prognostic indicators in AIDS-related NHL and the reported median survival is 8 months .
In the case presented, she was known to be seropositive for HIV for approximately a decade with a self-reported good adherence to HAART. It is to our understanding that she started developing respiratory symptoms at least 5 months before she was referred to us. Moreover, she had experienced unintentional but significant weight loss for a couple of months’ duration. Disappointingly, despite evidence of a widened mediastinum not seen in the previous chest film taken 14 weeks prior, the attending clinicians did not investigate the cause of her mediastinal widening. It took a further 21 weeks before a lymphoma diagnosis was suspected and 3 additional weeks for the biopsy results to become available, at such time she had already died of respiratory failure. It is evident that several conditions manifest atypically in individuals who are HIV positive but this should sensitize clinicians to be even more aggressive in searching for the definitive diagnosis. Arguably, if the diagnosis of lymphoma was made during the initial presentation 24 weeks earlier this patient could have potentially benefited from chemotherapy with an ultimate increased survival.
In conclusion, the presence of a mediastinal widening coupled with a history of unintentional yet significant weight loss in an individual who is HIV seropositive should raise an index of suspicion for lymphomas and warrant aggressive investigations and timely management.
Acquired immunodeficiency syndrome
Antineutrophil cytoplasmic antibody
Body mass index
Cytoxan (cyclophosphamide), Adriamycin (doxorubicin), vincristine, and prednisone
Diffuse large B cell lymphoma
Highly active antiretroviral therapy
Mean corpuscular hemoglobin
Mean corpuscular volume
Mediastinal large B cell lymphoma
Random distribution of red cell width
Right ventricular systolic pressure
Venereal Disease Research Laboratory
The authors are grateful to the staff of the Jakaya Kikwete Cardiac Institute and the Muhimbili National Hospital.
Availability of data and materials
PP and PN took the history and performed the physical examination. FL interpreted the CT angiograms. PP wrote the initial draft of the manuscript. All authors reviewed and contributed to the final version of this case report. All authors read and approved the final manuscript.
The authors declare that they have no competing interests.
Consent for publication
Written informed consent was obtained from the patient’s next-of-kin for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Ethics approval and consent to participate
Ethical clearance was sought from the Directorate of Research of the Jakaya Kikwete Cardiac Institute.
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