Rinsho Ketsueki

In this study we investigated the frequency and the type of manifestations of antiprothrombin antibodies (aFII) in patients with antiphospholipid syndrome (APS). In 16 (84.2%) of 19 patients with lupus anticoagulant (LA) and either anticardiolipin antibodies or antiphosphatidylserine antibodies, two types of abnormal patterns were shown on a crossed immuno-electrophoresis technique using anti-human prothrombin murine IgG. The slow-moving peak of prothrombin was detected in 8 patients, while a peak in the other patients had the slow-moving shoulder. These slow-moving materials might represent prothrombin-aFII complexes. In 13 patients who were studied on Western blots, IgGs of 11 patients (84.6%) bound to human purified prothrombin, and the IgGs of 7 (53.8%) of these patients also bound to human purified α-thrombin. Our results indicate that aFII detected in patients with APS may explain part of the mechanism of LA

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A 72-year-old man was admitted because of general weakness. On physical examination, marked splenomegaly was found. Blood tests revealed anemia, thrombocytopenia and two-peak hypergammaglobulinemia composed of κ type IgG and IgA monoclonal proteins. Peripheral blood and bone marrow (BM) contained abnormal lymphocytes including plasmacytoid lymphocytes and/or plasma cells. Pathological examination of the biopsied BM showed non-Hodgkin lymphoma consistent with the lymphoplasmacytoid type. Immunohistochemical staining revealed two different populations of cells, one with IgG and the other with IgA; no cell stained both and no solitary cluster of either IgG or IgA positive cell was seen. The surface phenotype of the lymphoma cells was CD19⁺, CD20⁺, HLADR⁺. Double immunofluorescense staining of the BM smear showed IgG or IgA positive plasma cells, whereas small lymphocytes were negative for IgG and IgA. Analysis of immunoglobulin genes in the BM cells showed 2 rearranged bands in each of heavy chain genes and κ light chain genes. The patient was treated with modified MVCP theray and the two monoclonal proteins decreased in parallel with the improvement of splenomegaly. These findings strongly suggest that the two-peak monoclonal protein was produced by monoclonal lymphoma cells. The patients has been disease-free without any therapy since August, 1995.

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A 30-year-old man with a diagnosis of acute promyelocytic leukemia (APL) was admitted. Laboratory findings were as follows: WBC 32,900/μl with 88% promyelocytes, Hb 10.4 g/dl, platelets 2.6×10⁴/μl. Coagulaion tests revealed DIC. Bone marrow was hypercellular with 91.8% promyelocytes which were strongly positive for peroxidase and positive for α-naphthyl butyrate esterase. Cytogenetic study revealed 46, XY, t(15;17)(q22:q11). He was treated with all-trans retinoic acid (ATRA) along with hydroxyurea (HU) and low-molecular weight heparin (LMH). Because his WBC increased to 93,700/μl on day 6 of ATRA therapy, DCMP chemotherapy was given, while ATRA was withheld. He developed enterocolitis due to myelosuppression. ATRA was restarted along with granulocyte-colony stimulating factor (G-CSF). His WBC rose to 10,400/μl with a marked, but temporary predominance of myelomonocytes both in peripheral blood and in bone marrow. These myelomonocytoid cells were positive for specific and nonspecific esterase double stainings. Then he entered complete remission. It was of interest that myelomonocytoid differentiation of APL cells was induced by ATRA. The etiology was discussed.

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A 34-year-old man was found to have leukocytosis and thrombocytosis in 1983. In 1988, his leukocyte count was 10,400/μl, Hb 16.5g and a platelet was 73×10⁴/μl. A bone marrow examination showed megakaryocyte hyperplasia. Essential thrombocythemia (ET) was diagnosed but no treatment was given. In February 1993, anemia and hepatosplenomegaly developed and cytogenetic study of the peripheral blood demonstrated t(1;7)(q10;p10). Myelofibrosis was diagnosed as by bone marrow biopsy. The patient was treated with blood transfusion, oxymetholone and prednisolone, but without effect. In 1995, acute myeloid leukemia developed, and he died in December 1995 due to septicemia. We report here a case of that ET developed myelofibrosis with t(1;7)(q10;p10) anomaly and acute leukemia.

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