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!
Thank you to PGY2 Shawn Shah for presenting an interesting case of a 66 year old homeless male who presented with fevers, productive cough, and chronic intermittent chest pain. The patient had a transthoracic echocardiogram done which showed an EF of 35%, as well as a 1.6×1.4cm aortic valve vegetation. The patient had repeatedly negative blood cultures and culture-negative endocarditis was suspected.
What is culture negative endocarditis? -3 negative blood cultures (after 5 days of incubation) **Remember that administration of antibiotics before obtaining blood cultures decreases the recovery rate of bacteria by 35-40%!!! -HACEK organisms previously thought to be the most common causes, however can be easily isolated when cultured fro more than 5 days -Certain exposures/conditions can point to a specific diagnosis such as: farm animals (Brucella, Coxiella), homeless shelters (bartonella quintana), cats (Bartonella henselae), unpasteurized milk (Brucella/Coxiella) -Immunocompromised and HIV+ patients are at risk for fungal and Coxiella endocarditis
Remember to consider causes of noninfective endocarditis as well such as anti-phospholipid antibody syndrome (sterile valvular vegetations, most commonly affecting the mitral valve), atrial myxoma, marantic endocarditis
Empiric Therapy -ID consult is recommended for the empiric therapy of culture negative endocarditis. -For acute presentations of native valve endocarditis (NVE), coverage for Staph aureus, beta-hemolytic strep and aerobic gram negative bacilli should be initiated (vancomycin + cephalosporin) -For subacute presentations of NVE, coverage for Staph aureus, viridans group strep, HACEK and enterococci should be initiated (vancomycin + ampicillin-sulbactam). **These regimens do not cover organisms for all organisms that cause culture negative endocarditis. If other organisms suspected, need additional coverage such as Doxycycline.
The patient was subsequently found to have elevated Bartonella titers and had an aortic valve replacement surgery performed, with the native valve staining positive for Warthin-Starry stain (supportive of Bartonella). Treatment for confirmed Bartonella endocarditis: Doxycycline + Gentamicin (Rifampin if cannot use gentamicin) for 14 days. If valve surgery is performed, doxycycline alone is continued for 6 weeks. If surgery is not performed and infected tissue is still present, doxycycline is continued for 3 months.
On a semi-related note, check out the POET trial (https://www.nejm.org/doi/full/10.1056/NEJMoa1808312) which discusses the use of partial oral antibiotics in the treatment of left sided endocarditis. We will be discussing this in journal club on December 20th!
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!