Harold R. Schumacher

Hand-mirror variant of acute lymphoblastic leukemia. Evidence for early T-cell lineage in two cases by evaluation with monoclonal antibodies

Lymphoid cells from two patients with hand‐mirror variant of acute lymphoblastic leukemia (ALL) were studied with various monoclonal antibodies in attempts to determine their derivation and differentiation. The predominant feature of the malignant bone marrow cells was strong reactivity with antibody 3A1, which stains the majority of normal T‐cells and is apparently present on all T‐ALL cells. In addition, a less intense binding was observed with antibody 4F2, which reacts with activated or rapidly dividing cells, and antibody 10.2, which reacts with all thymocytes and most peripheral T‐cells. Most other antibodies with a wide variety of specificities were not reactive or, in the case of a few anti‐T‐cell antibodies showed, by fluorescence‐activated cell sorter analysis only weak staining on some cells. Sequential bone marrow studies in one patient, before and during treatment with chemotherapy, revealed a reduction of 3A1‐positive cells, concordant with a decrease of malignant cells in the marrow. When involved lymph nodes, peripheral blood, or marrow were studied, similar reactivity patterns were found in all locations. The data obtained suggest that in both patients with hand‐mirror variant of ALL, the malignant lymphoid cells were immature cells of early T‐lymphocyte lineage. The relation of phenotype by monoclonal antibody analysis to hand‐mirror morphologic type and biologic function is discussed.

Terminal-Deoxynucleotidyl Transferase (TdT): Serial Observations on Patients with Leukemia

Terminal-deoxynucleotidyl transferase (TdT) bone marrow determinations were performed on 67 patients with leukemia using the indirect immunofluorescence technique. A total of 103 smears were evaluated on 32 patients with acute lymphoblastic leukemia. With some exceptions, TdT levels were elevated at onset, declined during induction except in resistant cases, decreased during remission on chemotherapy, showed slight elevation during remission off chemotherapy, and rose during relapse in those cases previously positive. The most important finding was that patients in remission may have elevated TdT levels. Those were usually less than 10%. A total of 124 bone marrow smears were evaluated on 29 patients with acute myeloid leukemia. In general, values in all categories were below 1%, with a few elevated between 1% to 10%. Six patients with chronic myelogenous leukemia in blast crisis had 13 bone marrow smears evaluated. Five were in myeloblastic crisis and had values of less than 1%; 1 was lymphoblastic which had 50% positive cells at onset. In our experience, TdT determinations are of value in lymphoblastic leukemia in diagnosis, in predicting response to therapy, and in detecting early relapse.

Acute erythroleukemia, M6b.

T patient was a 47-year-old man whose past medical history was significant for renal transplant, as well as refractory anemia with ringed sideroblasts (Figure 1) diagnosed 1 year previously. He re-presented to his hematologist/oncologist with fatigue and weight loss. The only medications he was taking at the time of presentation were cyclosporine and prednisone. Physical examination was noncontributory. A complete blood count revealed anemia, thrombocytopenia, and rare blasts. A bone marrow aspirate and biopsy were performed and revealed 51% erythrocytic precursors (Figure 2). Characteristic features included the presence of 41% pronormoblasts by FrenchAmerican-British (FAB) exclusion criteria, with marked dysplastic change and ‘‘block-and-blush’’ periodic acid– Schiff positivity (Figure 3) of the erythroid cell lineage. There were 22% myeloblasts by FAB exclusion criteria, with minimal dysplasia of the granulocytic cell line. A diagnosis of acute erythroleukemia, M6b (pure erythroleukemia, Di Guglielmo disease) was established. Cytogenetic analysis demonstrated a complex karyotype with abnormalities of both chromosomes 5 and 7. Review of the previous bone marrow slides confirmed the original diagnosis. The patient underwent 6 cycles of arabinosylcytosine (cytarabine)/daunorubicin therapy. Sequential intrachemotherapy and postchemotherapy bone marrow specimens revealed ablation of all hematopoietic elements, with the exception of the pronormoblasts. The patient died 21⁄2 months after initial diagnosis. A leukemia composed of purely erythrocytic precursors was first described by Di Guglielmo in 1926 and was designated Di Guglielmo disease. Until recently, this fulminant acute leukemia was alternately classified as a myeloproliferative disorder or a myelodysplastic syndrome. Nevertheless, a number of articles and books have described the complex cytogenetics and extremely poor outcome associated with this disease, which represents a distinct leukemic entity.1–4 The overall presentation and outcome of the patient described here are in keeping with previous descriptions of this condition.1,2 Investigators studying this leukemic process proposed that this disorder be renamed

Hematologic Complications of Cancer from the Species Specific View

In this chapter, pertinent hematologic alterations developing in man and animals with associated malignant disorders are discussed.

Lymph Node Biopsy in Thrombotic Thrombocytopenic Purpura

Excerpt To the editor: In the article by Goodman and colleagues (1) on the use of gingival biopsy in the diagnosis of thrombotic thrombocytopenic purpura, the authors mention the use of lymph node ...