Tatsuro Goto

Successful treatment of a patient with cardiac lymphoma who presented with a complete atrioventricular block

A patient with primary cardiac lymphoma, which is very rare, generally is regarded to have a poor prognosis. We herein report a patient with cardiac lymphoma who was treated successfully by systemic chemotherapy and radiotherapy using a pacemaker to control the complete atrioventricular (A‐V) block. A 70‐year‐old man had a syncope caused by a complete A‐V block. An echocardiogram, a computed tomographic scan, and magnetic resonance imaging of his chest showed a cardiac tumor. At this time, a biopsy of the cardiac tumor disclosed malignant lymphoma (diffuse large cell type, B cell type). The patient was thus treated with systemic chemotherapy and radiotherapy and, as a result, achieved a complete remission with a disappearance of the A‐V block. Recently, several successful outcomes involving primary cardiac lymphoma have been reported because of the progress in diagnostic techniques including echocardiography, computed tomographic scanning, and magnetic resonance imaging, as well as improvement in the therapy of malignant lymphoma. Our clinical experience indicated that an early and accurate diagnosis combined with the appropriate therapy can thus help in obtaining a long survival in patients with primary cardiac lymphoma. Am. J. Hematol. 59:171–174, 1998.

Soluble fas in the serum of patients with non-Hodgkin's lymphoma : Higher concentrations in angioimmunoblastic T-cell lymphoma

The soluble form of Fas (sFas) can block apoptosis induced by the Fas ligand in vitro. A recent report demonstrated that mice injected with sFas displayed autoimmune features. Therefore, an elevated serum concentration of sFas may be associated with lymphoproliferation and autoimmune diseases. We measured the serum concentrations of sFas in 77 patients with non‐Hodgkins lymphoma (NHL) [8 angioimmunoblastic T‐cell lymphoma (AIL), 12 T‐cell NHL, 53 B‐cell NHL, and 4 natural killer‐cell NHL]. Elevated concentrations of sFas were detected only in AIL, which is frequently accompanied by autoimmune diseases (P < 0.005 compared with age‐matched controls). A possible association of sFas and autoimmune features in AIL is discussed. Am. J. Hematol. 58:334–336, 1998.

IMMUNOGLOBULIN CLASS SWITCH FROM IgA1 TO IgG2 AND SIMULTANEOUS ASSOCIATION WITH BENCE JONES PROTEINURIA IN THE ESCAPE PHASE IN A MYELOMA PATIENT TREATED WITH INTERFERON ALPHA

Immunoglobulin (Ig) class switch from α1 to γ2 associated with κ‐type Bence Jones proteinuria was evident in the escape phase of an IgA1 myeloma patient treated with interferon α (IFNα). The additional M‐protein, IgG2‐κ, level rapidly increased and was associated with Bence Jones proteinuria, whereas monoclonal IgA1‐κ progressively declined. The N‐terminal 21 amino acid sequences of the κ‐chains of monoclonal IgA1, IgG2 and the Bence Jones protein were the same. The N‐terminal 15 amino acid sequence of the γ2‐chain was identical to that of the α1‐chain. Based on these findings, the IgA1 myeloma cells underwent a class switch in CH gene expression from α1 to γ2 with cell differentiation in vivo. The mechanism of the Ig class switching is discussed from three points of view: (1) Increase in immature and plasmablastic myeloma cells in the escape phase is susceptible to Ig class switching by the T‐cell‐ derived cytokines. (2) We presumed that administered IFNα increased the amounts of secreted IFNγ from the Th1 cells. (3) Due to a large quantity of IFNγ, an inducer of Cγ2 germ‐line transcript, Ig class switching occurred stepwise from the α1 constant region gene to the next 3′CH γ2 gene.

Recurrent appearance of 14q+ chromosome associated with lymphoid crisis of Ph-positive chronic myelogenous leukemia.

The 14q+ chromosomal anomaly commonly found in cases of lymphoid neoplasm recurrently occurred during the lymphoid crisis of a patient with Philadelphia chromosome (Ph) positive chronic myelogenous leukemia (CML). At presentation lymphoblasts, with pre-B phenotype increased, and both the Ph and 14q+ were found in the same metaphases. After treatment with vincristine and prednisolone, the patient entered into the chronic phase, and only a Ph was detected in 100% of the cells examined. The 14q+ reappeared at the recurrence of the lymphoid crisis, and then disappeared in the second chronic phase. The BCR/ABL mRNA, which is specific for CML, was detected in the blastic cells by a method using reverse transcriptase and polymerase chain reaction. The rearrangement of the immunoglobulin heavy chain gene (JH gene) was also detected in the blastic cells. These results suggest that the 14q+ was closely associated with the lymphoid crisis of the CML patient.