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!