Nodal Marginal Zone Large B-Cell Lymphoma with Burkitt Translocation and Complex Chromosomal Changes Associated with Overexpression of BCL2, MYC, and BCL6.

We report the first case of a nodal marginal zone large B-cell lymphoma and the first with MYC rearrangement. This high proliferation rate lymphoma (40% of cells) occurred in the bilateral cervical, axillary, and para-aortic lymph nodes of an 82 year old woman. It involved extensively her bone marrow, and was lethal. Malignant B-cells were CD10 negative, harbored Burkitt translocation, and multiple chromosomal changes including trisomies of chromosomes 3 and 18, and three copies of 8q with an intact q24 cytoband (in addition to MYC rearrangement), associated with overexpression of BCL6, BCL2, and MYC respectively. We suggest that in aggressive nodular marginal zone lymphomas (clinical picture or high proliferation rate of lymphoma cells), fluorescence in situ hybridization analysis for MYC rearrangement, with break-apart probe, and for MYC/IGH translocation, in addition to chromosome analysis, should be performed. MYC rearrangement associated with a more rapid progression of the neoplasia, might warrant a more aggressive treatment.

Primary, extranodal, follicular non-Hodgkin lymphoma of the gallbladder: case report and a review of the literature.

We report the first case of isolated primary extranodal non-Hodgkin lymphoma follicular grade 2 limited to the gallbladder, found in the laparoscopic cholecystectomy specimen from a 70 year old woman with symptomatic cholelithiasis. The pericystic duct lymph node, surgical margins, and other lymph nodes, were not involved with lymphoma. According to the medical literature in English language, mucosa associated lymphoid tissue lymphoma (6 cases) is the most frequent type (38%) of primary gallbladder lymphomas (15 reported cases plus our case). Our case demonstrates that follicular lymphoma can be limited to the gallbladder, and confirm that it can occur in an organ normally devoid of lymphoid tissue.

Correlation between the percentages of myeloblasts in bone marrow obtained by flow cytometry and manual counting on glass slide smears in 74 hematologic patients.

To investigate reliability of calculating percentage of myeloblasts by flow cytometric method, data were obtained from 74 hematologic patients (76 paired data). Myeloblast counts obtained by manual count versus flow cytometry were compared. Our data show that the percentage of myeloblasts in the bone marrow obtained with flow cytometric method correlates well with manual count (correlation coefficient is 0.9912). A very high correlation coefficient means that reliable percentage of myeloblasts in the bone marrow can be obtained by either method alone. Flow cytometry is a useful adjunct (or quality control) to validate manual myeloblast count and vice versa.

Hematogones in the peripheral blood of a 5½-month-old boy with cyclic neutropenia due to heterozygous, novel ELANE gene mutation p.Q97P, c.290 A>C.

We have identified a novel point mutation in the ELANE gene of a 5.5-month-old boy with severe cyclic neutropenia, and we are reporting for the first time, to our knowledge, the presence of hematogones in the peripheral blood of an infant. The novel point mutation occurred at base number 290 in codon 97, where adenine was replaced with cytosine. The mutation caused the replacement of amino acid glutamine with amino acid proline in the activation domain of the elastase 2 enzyme. The heterozygous mutation generated severe cyclic neutropenia, granulocytic maturation arrest, an increased number of hematogones (26% of marrow cells) in the bone marrow, an absence of neutrophils, and the presence of stage 3 (mature) hematogones in the peripheral blood. The percentage of hematogones in the peripheral blood was inversely proportional to the absolute number of neutrophils. Leukemic number of blast-like cells (hematogones) in the bone marrow, blast-like cells in the peripheral blood, marked neutropenia, and the arrest of granulopoiesis might suggest an acute leukemia. However, the finding of characteristic flow cytometric features of hematogones should help to avoid a wrong diagnosis.