PGY3 George Samaan presented a very interesting case of a 22 year old M who presented with 2 months of polyarthralgias and a full body rash. Physical exam was significant for multiple violaceous plaques and nodules over the face, chest and back, with tenderness to palpation over multiple joints but no evidence of erythema, swelling or synovitis.
The differential for polyarthralgias is broad and includes infectious, reactive arthritis, rheumatoid arthritis, systemic rheumatic disorders (lupus, vasculitides, Still’s disease, sarcoidosis).
Work up for infections and rheumatologic disorders were negative, including ANA, RF/anti-CCP, anti-streptolysin, ANCA, HIV, Hepatitis B/C. The patient eventually received a skin biopsy which showed Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN), and patient’s diffuse joint pain was thought to be due to paraneoplastic arthritis. BPDCN is a rare hematologic malignancy that commonly manifests as cutaneous lesions. Malignant cells can be detected in peripheral blood in ~60% of case, however the number of circulating cells is variable and may not be detected. Similarly although bone marrow involvement occurs in ~80% of cases, it may be difficult to identify the malignant cells. Diagnosis should be suspected in those presenting with brown/violaceous bruise-like lesions, and skin biopsy is necessary.
Thank you George for presenting this interesting and rare case, and for reminding us that unusual skin lesions without clear etiology may be the presenting sign of an occult malignancy!
Today’s morning report case was a 62 year old M with no known PMH (15 pack year smoking history) presenting with chronic cough, shortness of breath and weight loss. Labs were significant for mild anemia/thrombocytopenia and significant lymphopenia and CXR showed a large right pleural effusion. The patient was subsequently found to be HIV+ (first diagnosis, CD4 count 52).
Differential for pleural effusions in HIV+ patients:
– Infectious etiologies: Must consider the common bacteria that cause pneumonia (Strep pneumo, Staph aureus), which accounts for up to 30% of pleural effusions in HIV patients. Mycobacteria (M Tb, MAC), PCP, cryptococcus and disseminated histoplasmosis should also be considered, especially when the CD4 is <150.
– Malignant etiologies: Kaposi Sarcoma (large bilateral pleural effusions, majority present with skin lesions), Non-hodgkin lymphoma (presents with pulmonary nodules/masses, lymphadenopathy, hepatosplenomegaly), Primary effusion lymphoma, primary lung cancer and metastases.
– Other causes of pleural effusions such as heart failure, hypoalbuminemia, nephrotic syndrome/CKD should also be considered.
Our patient received a thoracentesis demonstrating an exudative effusion with 46% “other” cells, which were identified as large lymphoma cells. Further cytology studies revealed CD19/CD20 negative, CD45 positive, HHV8 positive neoplastic cells, and the patient was diagnosed with Primary Effusion Lymphoma.
Primary Effusion Lymphoma (PEL)
– PEL is a rare HIV associated non-Hodgkin’s Lymphoma (accounts for 4% of HIV associated NHL)
– Arises in body cavities such as pleural space, pericardium and peritoneum and generally demonstrates evidence of HHV-8 infection (>90% of HIV associated PEL also are EBV+). There are usually no extracavitary tumor masses present and patients typically present with dyspnea (from pleural/pericardial disease) or abdominal distension (from peritoneal disease). Imaging typically does not show any solid masses, mediastinal enlargement or parenchymal abnormalities.
– The prognosis for PEL is poor with median survival time of ~6 months.
– The diagnosis is made by analyzing effusions (will almost always be positive for malignant cells due to the liquid-phase growth of tumor). The malignant cells are usually CD45+ but negative for most B cell markers (CD19, 20, 79a) and T cell antigens.
– There is limited data on the treatment of PEL and generally involves initiation of antiretroviral therapy, cytotoxic chemotherapy, and local radiation therapy.
Thank you Brian Yu for presenting such an interesting and rare case!
PGY2 Stefan Nguyen presented an great case of a 65 year old Vietnamese man presenting with 2 weeks of worsening shortness of breath, lower extremity edema and weight loss. He was found to have a large pericardial effusion without clinical or echocardiographic evidence of tamponade, had a pericardiocentesis done and pericardial fluid cultures were positive for Mycobacterium Tuberculosis!
– Common infectious causes of pericardial effusions include Staph/Strep, Coxsackievirus, echovirus and don’t forget HIV!
– Malignancy should also be considered, in particular lung/breast cancer and Hodgkin lymphoma, though any malignancy can metastasize to the pericardium.
– Other etiologies include post-MI pericarditis, drug induced, rheumatologic (lupus/RA), uremia and idiopathic
Tuberculous Pericarditis: In the case today, Tuberculosis was suspected early on given that the patient was from Vietnam and presented with leukopenia and an elevated Alkaline phosphatase.
– When analyzing pericardial fluid, the most important studies include cell count (should be lymphocyte predominant in Tb), gram stain, culture and cytology. The yield of AFB smear is low from pericardial fluid and will generally be negative.
– There are no clear parameters for distinguishing transudative v exudative pericardial effusions.
– The utility of ADA in pericardial effusions is controversial, and generally the diagnosis of Tb depends upon clinical suspicion, lymphocyte predominant fluid and positive AFB culture. The Official American Thoracic Society and Infectious Diseases Society of America do recommend checking an ADA level, however this is a conditional recommendation with low-quality evidence.
**Remember that ADA is falsely elevated in PMN predominant pericardial or pleural fluid, and should only be in lymphocyte predominant fluid analysis.
– The use of steroids in the treatment of Tb Pericarditis was studied in a large trial in NEJM in 2014. The results showed that steroids did not decrease mortality, however did reduce the incidence of constrictive pericarditis. In patients with HIV, steroid use was associated with HIV-related cancer, specifically Kaposi’s Sarcoma. (https://www.nejm.org/doi/10.1056/NEJMoa1407380)