Journal du sang et de la lymphe

Primary cardiac lymphoma (PCL) is an extremely rare malignancy and an uncommon presentation of Non-Hodgkin’s Lymphoma. We report the first case of Precursor B-cell lymphoblastic PCL. A 44 year old Caucasian woman presented to our institution with one week history of worsening dyspnea, leg swelling and a weight gain of 12 pounds. Urgent echocardiography revealed pericardial effusion with tamponade physiology. Subsequent imaging showed a mass in the right atrio-ventricular groove. Pericardiocentesis was performed and cytopathology showed malignant appearing immature lymphocytes. Immunophenotypic analysis revealed 85% of the cells to be CD19 and CD10 positive. The cells were negative for CD20, surface light chains, CD3 and CD5 consistent with immature B-cells. Terminal deoxynucleotidyl Transferase (TDT) was strongly positive confirming Precursor B-cell Lymphoblastic Lymphoma. Clinical presentation of PCL can be varied and can mimic non-neoplastic primary cardiac problems. When PCL is suspected and a pericardial effusion is present, pericardial fluid analysis can be a reasonable first step. If diagnostic, it can avoid further invasive procedures. Chemotherapy remains the standard treatment and should be initiated early given the aggressive nature of these high grade lymphomas. Surgery is needed only in selected cases with obstructive features.

Abnormalities of chromosome (ch)1 have been shown to be significant adverse prognostic factors in multiple myeloma (MM) but they have not yet been systematically studied in patients undergoing autologous hematopoietic cell transplantation (auto-HCT). The aim of this study was to determine whether patients with high-risk MM and ch1 abnormalities (1q gain, 1p deletion, translocations of ch1) constitute a highest risk group compared to a contemporaneous cohort of high-risk MM patients without ch1 abnormalities. 232 patients (169 induction, 63 salvage) with MM and at least one recognized high-risk feature met criteria for inclusion. The presence of a ch1 abnormality (n=15) was highly significant in patients undergoing salvage autologous HCT (n=6) for predicting shorter PFS (p<0.001; HR= 22.93; 95% CI: 4.94- 106.48), and OS (p = 0.0002; HR= 21.22; 95% CI: 1.18-14.98). Median PFS and OS for those with a ch1 abnormality and del 13q (n=7) were 4.76 and 9.43 months, with ch1 abnormality and no del 13q (n=8) were 16.79 and 35.22 months respectively, and for those Without cytogenetic abnormalities, 24.44 and 57.03 months respectively. Based upon the impact of ch1 abnormalities on auto-HCT outcomes in this study, further investigation in larger series is warranted.