ABSTRACT
We report two patients with a distinctive biphenotypic hematologic disorder characterized by lymphoblastic lymphoma (LBL), eosinophilia, and myeloid malignancy and/or hyperplasia associated with a t(8;13)(p11;q11) chromosomal translocation in both bone marrow and lymph node specimens. Both patients presented with lymphadenopathy pathologically classified as LBL with a T-cell immunophenotype, myeloid hyperplasia of the bone marrow, and peripheral blood eosinophilia. The first patient achieved clinical complete remission after receiving several regimens of chemotherapy and remains disease-free 16 months after undergoing allogeneic bone marrow transplantation. The second patient developed progressive lymphadenopathy despite several courses of chemotherapy directed against non-Hodgkin's lymphoma. Eight months after his initial presentation, he developed acute myelogenous leukemia that was refractory to therapy. Comparison of these patients with four similar cases recently reported in the literature suggests that this constellation of findings constitutes a distinctive clinicopathologic syndrome. Molecular analysis of the t(8;13) translocation breakpoint may identify genes located in this region and provide insight into the pathogenesis of this interesting biphenotypic hematologic malignancy.
Subject(s)
Bone Marrow/pathology , Chromosomes, Human, Pair 13/ultrastructure , Chromosomes, Human, Pair 8/ultrastructure , Eosinophilia/genetics , Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics , Pregnancy Complications, Neoplastic/drug therapy , Translocation, Genetic , Acute Disease , Adolescent , Adult , Aged , Aged, 80 and over , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Bone Marrow Transplantation , Carboplatin/administration & dosage , Combined Modality Therapy , Cyclophosphamide/administration & dosage , Cytarabine/administration & dosage , Daunorubicin/administration & dosage , Doxorubicin/administration & dosage , Etoposide/administration & dosage , Fatal Outcome , Female , Humans , Hydroxyurea/therapeutic use , Hyperplasia , Ifosfamide/administration & dosage , Leukemia, Myeloid/genetics , Lymph Nodes/pathology , Male , Middle Aged , Mitoxantrone/administration & dosage , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Prednisone/administration & dosage , Pregnancy , Pregnancy Complications, Neoplastic/therapy , Remission Induction , Syndrome , Vincristine/administration & dosageABSTRACT
Threshold Limit Values (TLVs) represent conditions under which the TLV Committee of the American Conference of Governmental Industrial Hygienists (ACGIH) believes that nearly all workers may be repeatedly exposed without adverse effect. A detailed research was made of the references in the 1976 Documentation to data on "industrial experience" and "experimental human studies." The references, sorted for those including both the incidence of adverse effects and the corresponding exposure, yielded 158 paired sets of data. Upon analysis it was found that, where the exposure was at or below the TLV, only a minority of studies showed no adverse effects (11 instances) and the remainder indicated that up to 100% of those exposed had been affected (8 instances of 100%). Although, the TLVs were poorly correlated with the incidence of adverse effects, a surprisingly strong correlation was found between the TLVs and the exposures reported in the corresponding studies cited in the Documentation. Upon repeating the search of references to human experience, at or below the TLVs, listed in the more recent, 1986 edition of the Documentation, a very similar picture has emerged from the 72 sets of clear data which were found. Again, only a minority of studies showed no adverse effects and TLVs were poorly correlated with the incidence of adverse effect and well correlated with the measured exposure. Finally, a careful analysis revealed that authors' conclusions in the references (cited in the 1976 Documentation) regarding exposure-response relationships at or below the TLVs were generally found to be at odds with the conclusions of the TLV Committee. These findings suggest that those TLVs which are justified on the basis of "industrial experience" are not based purely upon health considerations. Rather, those TLVs appear to reflect the levels of exposure which were perceived at the time to be achievable in industry. Thus, ACGIH TLVs may represent guides of levels which have been achieved, but they are certainly not thresholds.