First-line R-CVP versus R-CHOP induction immunochemotherapy for indolent lymphoma with rituximab maintenance. A multicentre, phase III randomized study by the Polish Lymphoma Research Group PLRG4.

R-CVP (cyclophosphamide, vincristine, prednisone) and R-CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone + rituximab) are immunochemotherapy regimens frequently used for remission induction of indolent non-Hodgkin lymphomas (iNHLs). Rituximab maintenance (RM) significantly improves progression-free survival (PFS) in patients with complete/partial remission (CR/PR). Here we report the final results of a randomized study comparing R-CVP to R-CHOP both followed by RM. Untreated patients in need of systemic therapy with symptomatic and progressive iNHLs including follicular (FL) and marginal zone lymphoma (MZL), mucosa-associated lymphoid tissue (MALT), small lymphocytic (SLL), and lymphoplasmacytic (LPL) lymphoma were eligible. Patients were randomized to receive R-CVP or R-CHOP for eight cycles or until complete response (CR). All patients with CR/PR (partial response) received RM 375 mg/m2 q 2 months for 12 cycles. Primary endpoint was event-free survival (EFS). Two-hundred and fifty patients [FL 42%, MZL/MALT 38%, LPL/ Waldenström Macroglobulinaemia (WM) 11%, SLL 9%] were enrolled and randomized (R-CHOP: 127, R-CVP: 123). Median age was 56 years (21-85), 44% were male, 90% were in stage III-IV, 43% of FL patients had a Follicular Lymphoma International Prognostic Index (FLIPI) score ≥3, and 33·4% of all patients had an IPI score ≥3. At the end of induction treatment, the CR/PR rate was 43·6/50·9% and 36·3/60·8% in the R-CHOP and R-CVP groups (P = 0·218) respectively. After a median follow-up of 67, 66, and 70 months, five-year EFS was 61% vs. 56% (not significant), progression-free survival (PFS) was 71% vs. 69% (not significant) and overall survival (OS) was 84% vs. 89% in the R-CHOP vs. the R-CVP arm respectively. Grade III/IV adverse events (65 vs. 22) occurred in 40 (33·1%) and 18 (15·3%) patients, P = 0·001; neutropenia in 16 (11·6%) and 4 (3·4%) patients, P = 0·017; infection in 14 (10·7%) and 3 (2·5%) patients,; P = 0·011; and a second neoplasm in three versus seven patients., in the R-CHOP and the R-CVP groups respectively. This multicentre randomized study with >five-year follow-up shows similar outcome in patients with indolent lymphoma in need of systemic therapy treated with R-CVP or R-CHOP immunochemotherapy and rituximab maintenance in both arms. The minor toxicity of the R-CVP regimen makes it a reasonable choice for induction treatment, leaving other active agents like doxorubicin or bendamustin for second-line therapy.

[Massive infiltration of the right vetricular wall caused by large B-cell lymphoma]. / Masywny naciek wolnej sciany prawej komoryprzez chloniaka z duzych komórek B.

We presented a case of symptomatic secondary cardiac B-cell lymphoma localised in the free wall of the right ventricle (RV). It was detected during transthoracic echocardiography and confirmed by nuclear magnetic resonance imaging. The RV free wall motion abnormalities, decreased dimensions of RV and small pericardial effusion were found. The tumour dimensions declined after the first cycle of chemiotherapy with antracyclins.

[Asymptomatic myxoma of the tricuspid valve septal leaflet]. / Bezobjawowy sluzak platka przegrodowegozastawki trójdzielnej.

We presented a case of asymptomatic myxoma of the tricuspid valve septal leaflet. The tumour was diagnosed accidentally during rutine transthoracic echocardiography and confirmed by transesophageal echocardiography. It was resected and the septal leaflet repaired during surgery.

[A large asymptomatic pericardial cyst in the apical region]. / Duza bezobjawowa torbiel osierdzia w okolicy koniuszka serca.

We presented a case of the large asymptomatic pericardial cyst localised near cardiac apex, filled with fluid of water- -equivalent density. The cyst was detected accidentally during transthoracic echocardiography and confirmed by 64-slice multi-detector computed tomography. Repeated transthoracic echocardiography was recommened.

[Fungal endocarditis of mitral and aortic biological prosthetic valves]. / Grzybicze zapalenie wsierdzia na protezie biologicznej w ujsciu mitralnym i aortalnym.

We present a rare case of fungal (Candida albicans) endocarditis on the two (mitral and aortic) biological prosthetic valves. Vegetations were detected by transthoracic echocardiography and confirmed by transesophageal echocardiography.

[Severe tricuspid regurgitation due to ventricular pacing lead - a case report]. / Ciezka niedomykalnosc zastawki trójdzielnej spowodowana zbyt krótka elektroda komorowa ukladu stymulujacego.

We present a case of severe symptomatic tricuspid valve regurgitation due to shifting of the septal leaflet of the valve toward the interventricular septum by a permanent ventricular pacemaker lead, making coaptation of the tricuspid leflats in systole impossible.

[Quadricuspid aortic valve]. / Czteroplatkowa zastawka aortalna.

We reported a case of a 45 year-old woman who had a quadricuspid aortic valve associated with moderate aortic regurgitation. The valve abnormality was detected by transthoracic echocardiography. Transesophageal echocardiography showed mild thickening of 4 symmetric aortic valve cusps, a small rectangular central regurgitant orifice, and moderate aortic insufficiency. In addition visualised this anomaly in 3D transesophageal echocardiography too.

[Mild type of the Ebstein anomaly]. / Lagodna postac anomalii Ebsteina.

Ebstein anomaly is a congenital malformation of the heart that is characterised by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialisation of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet. We present a case of a mild type Ebstein anomaly leading to moderate tricuspid valve regurgitation and some degree of right ventricular dysfunction.

[Circumflex coronary artery fistula during staphylococcal aortic valve endocarditis]. / Przetoka do tetnicy okalajacej w przebiegu gronkowcowego zapalenia wsierdzia zastawki aortalnej.

We presented a very rare case of the fistula to coronary artery during staphylococcal aortic valve endocarditis in a young man. The tranesophageal echocardiography detected vegetation on aortic valve leaflets and large regurgitatin. During transesophageal echocardiography the peri-anular multi-chamber abscess formation and fistulous communication to circumflex coronary artery was detected.

[Infective endocarditis of both atrioventricular valves in a young man with atrioventricular canal defect - a case report]. / Infekcyjne zapalenie wsierdzia obu zastawek przedsionkowo-komorowych u mlodego mezczyzny z ubytkiem przegrody miedzyprzedsionkowej typu I - opis przypadku.

We report a case of a 33-year-old man with infective endocarditis of both atrioventricular valves coexisting with a congenital heart defect: atrioventricular canal defect. Transthoracic and transesopageal echocardiography showed complete atrioventricular canal defect and vegetations affecting both the normal mitral and tricuspid valves. The patient received a combined antibiotic therapy and was qualified for cardiosurgical correction.

[Recurrent multi-focal cerebral stroke complicating Libman-Sacks endocarditis in a young woman with systemic lupus erythromatosus]. / Nawracajacy wieloogniskowy udar mózgu w przebiegu zapalenia wsierdzia Libmana-Sacksa u mlodej kobiety z toczniem rumieniowatym ukladowym.

Systemic lupus erythromatosus is often associated with an antiphospholipid syndrome (APS). A high prevalence of valvular heart disease in APS leads to increased risk of embolic events, particularly cerebrovascular. We present a patient with cerebral infarction, with positive lupus anticoagulant, anticardiolipin antibodies and factor V Leiden mutation. Echocardiographic examination revealed mitral valve anterior leaflet thickening and verrucous vegetations consistent with Libman-Sacks endocarditis, which is commonly associated with APS.

[Severe nonthrombotic pulmonary embolism due to primary cancer of the pancreas--a case report]. / Masywna nieskrzeplinowa zatorowosc plucna w przebiegu pierwotnego raka trzustki--opis przypadku.

We describe a patient with a severe, neoplastic pulmonary embolism due to primary cancer of the pancreas. The 40-year-old male was admitted to the haematology department with an initial diagnosis of lymphoma. Transthoracic echocardiography showed a mass in the right atrium. Transoesophageal echocardiography was performed and revealed two moving tumours: the first in the extend of the vena cava superior and the second one in the tricuspid valve annulus. After bronchoscopy the patient's haemodynamic condition deteriorated and was followed by cardiac arrest. Post-mortem examination showed extensive tumour emboli in the left pulmonary artery as well as pancreatic cancer at an advanced stage.

[Large, asymptomatic left atrial myxoma--a case report]. / Olbrzymi, bezobjawowy sluzak lewego przedsionka--opis przypadku.

We report a patient with a large, asymptomatic left atrial myxoma detected by transoesophageal echocardiography. The tumour filled the great part of the left atrium cave and led to mitral valve obstruction. Surgical management gave an excellent result.