An extranodal NK/T cell lymphoma, nasal type, with specific immunophenotypic and genotypic features.

Extranodal NK/T cell lymphoma, 'nasal type,' is a rare clinicopathological entity in Europe. The main clinical features are nasal congestion, sore throat, dysphagia and epistaxis, due to a destructive mass involving the midline facial tissues. Pathologically, lymphoma cells exhibit angioinvasion, angiodestruction and coagulative necrosis. We report the case of a patient who presented with fever, dyspnea, nasal congestion, headache, distention of right nasal turbinates and exophytic lower leg ulcerating lesions. A CT scan of visceral scull demonstrated a filling mass of right frontal, ethmoidal and maxillary sinuses with erosion of the wall of right maxillary sinus and ventral portion of the diaphragm. A biopsy was performed in the skin lesion and showed an angioinvasive NK/T cell lymphoma CD56 negative with clonal rearrangement of the T-cell-receptor gamma gene. Up to our knowledge, this is a rare immunophenotype for NK/T-cell, 'nasal type,' lymphomas. However, the lymphoma may be classified as extranodal NK/T cell lymphoma, 'nasal type,' due to typical clinical presentation, radiologic findings and pathological characteristics of polymorphism, angioinvasion, angiodestruction and coagulative necrosis.

Dilated cardiomyopathy during the course of hemolytic uremic syndrome.

A 47-year-old woman presented with severe hemolytic uremic syndrome (HUS) followed by heart failure. An echocardiogram showed an ejection fraction of 20%, and a cardiac catheterization followed by a myocardial histologic evaluation demonstrated dilated cardiomyopathy. Plasma exchange and hemodialysis were performed regularly. The later outcomes of renal function and cardiomyopathy were favorable. A review of the literature confirmed the rare and severe nature of cardiac lesions occurring in the course of HUS. This case indicates the importance of cardiac monitoring in HUS and the need for prolonged support.

First case of Penicillium marneffei fungemia in Greece and strain susceptibility to five licensed systemic antifungal agents and posaconazole.

We report the first case of Penicillium marneffei infection in a Greek bisexual man who is a frequent traveler to China. Penicilliosis and AIDS were diagnosed and antifungal treatment plus highly active antiretroviral therapy were administered successfully. In nonendemic areas travel history, clinical suspicion and laboratory alertness are critical for infection management.

Isolated native tricuspid valve Candida endocarditis in a non-drug-addicted patient: case report and review of the literature.

A case is reported of isolated native tricuspid calve Candida parapsilosis endocarditis (INTVCE) in a male patient with no history of drug abuse or heart disease. The patient had received hyperalimentation and antibiotics for four months via a central venous catheter after abdominal surgery. He underwent successful treatment with tricuspid valve debridement, liposomal amphotericin (AmBisome) and fluconazole, and remained without relapse during an eight-year follow up. A literature review of 12 similar cases (including the present patient) without history of drug abuse or heart disease, dating from 1970, is included.

Development of non-Hodgkin's lymphoma in a patient with primary biliary cirrhosis: A case report and review of the literature.

Patients with autoimmune disorders seem to have an elevated risk of lymphoma, especially non-Hodgkin's lymphoma (NHL). The increased risk has been attributed to the disturbance of immune function found in these patients or to the immunosuppressive therapy used to treat the autoimmune disorders. However, little information exists about the estimated baseline risk for lymphoma in patients with primary biliary cirrhosis (PBC). In this case report, we describe a female patient who developed nodal diffuse large B-cell lymphoma ten years following PBC diagnosis. Twenty five additional case reports (19 NHL and 4 Hodgkin's disease (HD), 2 without data about NHL or Hodgkin's disease) predominantly females were identified in the English literature. B-cell lymphoma was the most common NHL type reported but beyond that no clear predisposition for any specific lymphoma subtype was documented. PBC usually preceded lymphoma diagnosis. Fifteen cases had extranodal localization and the most common site was the liver.

Sarcoid-like granulomatosis in patients treated with anti-TNFα factors. A case report and review of the literature.

This report describes a 56-year-old woman who developed granulomatous lesions consistent with sarcoidosis during adalimumab therapy for rheumatoid arthritis. Cervical and axillary lymphadenopathy developed approximately 21 months after adalimumab administration. Non-caseating epithelioid cell granulomas consistent with sarcoidosis were detected both in an axillary lymph node specimen and in the bone marrow. Diseases showing similar histologic changes, especially tuberculosis, were excluded, and a diagnosis of sarcoidosis was made. Adalimumab was discontinued, and recovery was observed. The current case is, to our knowledge, the first to describe adalimumab-induced non-caseating granulomas in lymph nodes and bone marrow without pulmonary involvement in a patient treated for rheumatoid arthritis.

Concomitant diagnosis of myeloproliferative neoplasm and non-Hodgkin's lymphoma in a patient with portal vein thrombosis.

We describe here the rare coexistence, at the time of diagnosis, of a myeloproliferative neoplasm (MPN) and non-Hodgkin's lymphoma (NHL) in a 74-year-old patient who presented with thrombocytosis and signs of portal hypertension on physical examination. Abdominal computed tomography scan demonstrated extensive portal vein system thrombosis. Secondary causes of thrombocytosis were excluded. JAK2 V617F mutation was present in the peripheral blood, while bone marrow biopsy revealed marginal zone B-cell lymphoma. Molecular analysis failed to detect BCR-ABL rearrangement in peripheral blood cells. Simultaneous occurrence of MPN and NHL was diagnosed. This case may be of interest not only due to the rare coexistence of PMN and NHL, but also because of the undetermined clinical significance of JAK2 mutation in this subset of patients.

Angioimmunoblastic T-cell lymphoma-associated arthritis: case report and literature review.

Angioimmunoblastic T-cell lymphoma (AITL) is a rare type of non-Hodgkin lymphoma with systemic manifestations, including fever, lymphadenopathy, rash, and rarely arthritis. We report the case of a patient who presented with symmetric inflammatory polyarthritis and skin nodules resembling rheumatoid arthritis (RA). The patient responded initially to low-dose prednisolone, but 12 months later, he developed typical features of AITL. The characteristics of AITL-associated arthritis from 16 additional cases from the English literature are also reviewed. AITL-associated arthritis is an uncommon manifestation of angioimmunoblastic lymphoma that can mimic RA, especially when the typical systemic features of lymphoma are absent. This type of arthritis should be included in the differential diagnosis of patients presenting with an inflammatory polyarthritis.