Two-to-One AV Block Associated with the Congenital Long QT Syndrome

2:1 AV Block in LQTS. Introduction Conduction abnormalities associated with long QT syndrome (LQTS) have been reported as "pseudo 2:1 AV block" due to sinus intervals shorter than ventricular refractoriness.
Method and Results: We report the electrophysiologic characteristics of a patient suffering from congenital LQTS with episodes of true 2:1 AV block. Induction of 2:1 infra-Hisian blocks and return to 1:1 conduction were observed using single atrial and ventricular extrastimuli. The block was located in the Purkinje network but not in the myocardium.
Conclusion: The His-Purkinje system of our LQTS patient displayed dynamic properties with a strong increase in refractoriness for short-long sequences and a decrease for long-short sequences that triggered intermittent 2:1 AV blocks.     

Trisomy 3 in marginal zone B-cell lymphoma: a study based on cytogenetic analysis and fluorescence in situ hybridization

Trisomy 3 represents the most frequent and consistent chromosomal abnormality characterizing the recently defined entity marginal zone B-cell lymphoma (MZBCL). By cytogenetic analysis and/or fluorescence in situ hybridization (FISH) on interphase nuclei we found an increased copy number of chromosome 3 in 22/36 (61%) successfully analysed cases, including 8/12 cases with extranodal MZBCL, 8/13 cases with nodal MZBCL, and 6/11 patients with splenic MZBCL. Sensitivity of interphase cytogenetics was somewhat higher than that of conventional cytogenetic investigation. Structural chromosomal changes involving at least one chromosome 3 were seen in 11/20 cases with an increased copy number of chromosome 3: +del(3)(p13) was demonstrated in three cases, and was the sole chromosomal abnormality in one of them; +i(3)(q10) was seen in two other patients; and rearrangements involving various breakpoints on the long arm of chromosome 3 were found in the remaining cases. FISH on metaphase spreads confirmed these structural abnormalities and additionally showed two unexpected translocations involving chromosome 3. We conclude that: (1) trisomy 3 occurs in a high proportion of extranodal, nodal and splenic MZBCL; (2) FISH on interphase nuclei is an additional and sensitive tool in detecting an increased copy number of chromosome 3 in MZBCL; (3) additional structural abnormalities involving the long arm of chromosome 3 are frequent but non-recurrent and are perhaps secondary changes; and (4) abnormalities such as +del(3)(p13) and +i(3)(q10) suggest that genes located on the long arm of chromosome 3 are of particular importance in the pathogenesis of MZBCL.