Indolent T-lymphoblastic proliferation: report of a case with a 16-year course without cytotoxic therapy.

T-lymphoblastic lymphoma is a high-grade malignant lymphoma. Clinically indolent T-lymphoblastic proliferations have not been described. We present a case report of an indolent T-cell lymphoblastic proliferation studied by histopathology, immunohistochemistry, flow cytometry, antigen receptor gene rearrangement studies, and cytogenetics. The patient had recurrent masses in the upper aerodigestive tract over a 16-year period, was treated by multiple surgical excisions, and never received either chemotherapy or radiotherapy. A proliferation of lymphoblasts was present histologically. The cells were positive for terminal deoxynucleotidyl transferase, CD1, and CD3, and coexpressed CD4 and CD8. No clonal rearrangements of the T-cell receptor beta or gamma chain genes were identified. Cytogenetic studies revealed a questionable inversion of the short arm of chromosome 9, affecting the 9p21-22 region. Although ectopic thymic tissue was considered, the case was considered to be an indolent lymphoblastic proliferation. It should be recognized that rare lymphoblastic proliferations may not behave in a high grade fashion as typically seen in T-lymphoblastic lymphoma.

Major activity of cladribine in patients with de novo B-cell prolymphocytic leukemia.

PURPOSE: De novo B-cell prolymphocytic leukemia (B-PLL) is a distinct clinicopathologic entity usually characterized by marked lymphocytosis, massive splenomegaly, an aggressive course, and refractoriness to therapy. Cladribine (2-chlorodeoxyadenosine [2-CdA]; Ortho Biotech, Raritan, NJ) is a newer purine analog with potent activity against indolent lymphoproliferative disorders. PATIENTS AND METHODS: We treated eight patients with cladribine 0.1 mg/kg/d for 7 days by continuous infusion or 0.14 mg/kg/d over 2 hours for 5 days, every 28 to 35 days, for a median of three courses (range, two to five). There were five men and three women, with a median age of 62 years and a median pretreatment duration of 6 months; four patients were previously untreated. RESULTS: All eight patients were assessable: five achieved a complete response with a median response duration of 14 months (range, 1+ to 55+), and three achieved a partial response with a median duration of 3 months (range, 1 to 3). Of four patients who achieved a complete response and in whom a peripheral-blood immunophenotypic analysis was performed, two had no circulating B-PLL cells and one had no residual disease on Southern blot analysis. Myelosuppression and infection were the major toxicities: three patients developed grade 3 or 4 myelosuppression, four had bacterial infections, and two had herpes zoster infections. CONCLUSION: In this small study of patients with de novo B-PLL, cladribine was an active agent that induced a high overall and complete response rate. These results require confirmation in larger numbers of B-PLL patients.

Heritable alpha 2-macroglobulin deficiency in a patient with arterial thrombosis: alpha 2-macroglobulin deficiency Irvine.

A heritable deficiency in α(2)-macroglobulin (α(2)M) was identified in a 61-year-old man with arterial thrombosis. Plasma α(2)M levels among the patient's symptom-free relatives consistently ranged from 43 to 55 percent of laboratory mean-normal values. The new α(2)M variant displayed retarded anodal immunoelectrophoretic mobility when studied in plasma and serum. The affected members of this lineage showed no evidence of acquired or inherited thrombotic or consumptive derangements involving other plasma proteins. The significance of a possible causal association between α(2)M deficiency and the predisposition to arterial thrombosis is considered. The uncomplicated use of streptokinase and urokinase to treat the reference patient's arterial thrombosis is described. Recommendations are made for the adoption of a descriptive nomenclature. The new familial deficiency is tentatively designated α(2) (+)-macroglobulin deficiency Irvine.

Remission of pure red cell aplasia associated with nonthymic malignancy.

Two patients with acquired pure red cell aplasia associated with malignancy are presented. One patient has breast cancer and the other had poorly differentiated nodular lymphoma; neither patient had evidence of a serum inhibitor of hemoglobin synthesis and both achieved complete hematologic remission following prolonged immunosuppressive therapy. The literature describing the association of pure red cell aplasia and nonthymic malignancy is reviewed and potential for responsiveness discussed.