B cell XXXX in adults. Clinical, morphologic, and immunologic findings.

@entity1 @entity981 @entity882 @entity2621 @entity5 @entity1778 @entity791
@entity981 ( @entity981 ) is a heterogeneous group of disorders, clinically, immunologically, and pathologically. @entity981 of a B cell phenotype (B- @entity981 ) is the least common. We have studied ten adult @entity1 with B- @entity981 , none of whom had a @entity5 mass. The median age was 56 years (range, 30 to 90). A history of an altered immune state was noted in four cases: a distant history of @entity2621 in one, pregnancy in one, and @entity791 in two. Two @entity1 presented with CNS involvement, and in two additional @entity1 @entity1778 developed during the course of disease. By the French-American-British (FAB) classification system, L3 leukemic morphology was present in nine, whereas L2 was present in one. Circulating @entity1778 varied from less than 500/dL to greater than 15,000/dL. Eight @entity1 were @entity882 , and eight were anemic at presentation. Immunologic marker studies on @entity1778 revealed monoclonal kappa light chain marking in nine and monoclonal lambda in one. Following chemotherapy, complete remission was achieved in three @entity1 , two of whom experienced relapse within 9 months. The median survival for the group was 3 months, and only one @entity1 experienced long-term, disease-free survival. We conclude that B- @entity981 in the adult presents with the classic L3 morphologic picture in the majority and is associated with extremely short survival.
