Fifty leukemia patients were given bone marrow transplants (BMTs) from unrelated donors at Meitetsu Hospital. We studied the outcomes of their transplants from two perspectives: leukemia disease stage and acute graft versus host disease (GVHD). The probability of disease-free survival for standard-risk, high-risk, and super-high risk patients was 65%, 29%, and 8%, respectively. The main causes of death were septicemia, cardiac and renal failure, and relapse of leukemia in the high- and super-high risk patients, and grade III∼IV acute GVHD in the standard-risk patients. The incidence of grade II∼IV and grade III∼IV acute GVHD was 32% and 17%, respectively. All 7 patients in whom grade III∼IV severe acute GVHD developed died. We conclude that better control of acute GVHD and treatment of early stage complications are clearly important to improving the outcome of BMTs from unrelated donors, especially for high and super-high risk patients.

View full abstract

To evaluate the prognostic significance of CD7 expression in de novo acute myeloid leukemia (AML), we studied 63 patients with AML who had been admitted to our hospital between September 1989 and January 1996. Eleven of the patients were later eliminated from the study (9 due to insufficient surface marker analyses, and 2 due to early death). The remaining 52 patients (median age: 42.5 years) were evaluated for morphologic subtype, immunophenotypic classification, complete remission (CR), diseasefree survival (DFS) and overall survival (OS). All 52 patients were grouped by the French-American-British classification system: 10 as M1, 16 as M2, 11 as M3, 8 as M4, 5 as M5, and 2 as M6. Ten of the patients expressed CD7 on their leukemic cells (positive rate ≥25) and were classified as CD7 (+) AML, with morphological subtypes as follows: 3 as M1, 6 as M2, and 1 as M3. Thirty-three of the 42 patients with CD7-AML (78.6%) and 6 of the 10 patients with CD7+AML (40%) achieved CR. DFS and OS rates for the patients with CD7 (-) AML were 22.1% and 35.4%, respectively; those for the CD7 (+) AML patients were 53.3% and 44.4%, respectively. No significant differences in gender, hematological findings, clinical manifestations such as hepatosplenomegaly, lymphadenopathy, or incidence of cemtral nervous system involvement, CR rate, and DFS distinguished patients with CD7 (+) AML from those with CD7 (-) AML. These results suggest that CD7 expression is unlikely to be a prognostic factor in AML.

View full abstract

We report a 48-year-old woman with adult T-cell leukemia who had refractory arthralgia, intense headaches, and fever. Leukemic cell infiltration of the cerebrospinal fluid was detected but no other acute signs were observed. Abnormal lymphocytes with lobulated nuclei were found in the synovial fluid, and a histologic examination revealed proliferation into the synovium. Because combination chemotherapy did not elicit a favorable respose, the patient was treated with a pentostatin bolus injection. The articular symptoms disappeared and complete remission was obtained. Six months later, she experienced arthralgia again together with a gradual increase of abnormal lymphocytes in peripheral blood. Sixteen months later, the patient was given pentostatin and achieved a complete remission again. She is still free from relapse without further therapy after 36 months, and her articular symptoms have not returned either. There were no adverse effects due to pentostatin. The patient's serum IL-6 level was elevated, suggesting that IL-6 may play a role in arthropathy.

View full abstract

A 49-year-old man was admitted to our hospital for investigation of splenomegaly and lymphocytosis. He had no significant past history and was not a smoker. Physical examination revealed massive splenomegaly and no palpable superficial lymph nodes. Hematological examination showed a hemoglobin concentration of 10.5g/dl, a platelet count of 9.8×10⁴/μl, and a leukocyte count of 21.2×10³/μl with 70% abnormal lymphocytes. In May-Giemsa stained blood films, the abnormal lymphocytes had round nuclei, abundant, pale cytoplasm, and slightly serrated edges. Phase-contrast microscopic and scanning electron microscopic examinations revealed many long surface villi. Tartrate-resistant acid phosphatase activity in these cells was negative. The abnormal lymphocytes had a CD5^-, CD10^-, CD11a⁺, CD11c⁺, CD19⁺, CD20⁺, CD22⁺ phenotype. These features were similar to those described for a variant form of hairy cell leukemia (HCL-Japanese variant). However, studies of Ig gene rearrangement and expression of sIg revealed a polyclonal proliferation of B cells. On the basis of these findings, this case was diagnosed as hairy B-cell lymphoproliferative disorder, a recently described condition characterized by polyclonal B-cell lymphocytosis and features resembling HCL-Japanese variant. Serological assays for antibodies against Epstein-Barr virus suggested a past infection. Splenectomy alleviated the anemia and thrombocytopenia, but not the lymphocytosis.

View full abstract

A 9-year old boy was admitted to our hospital due to a relapse of acute myelogenous leukemia (AML). A chromosomal analysis at the time of relapse revealed abnormalities in addition to 45, X, -Y, t(8;21)(q22;q22) when AML was first diagnosed. The patient was given granulocyte-colony stimulating factor (G-CSF), cytosine arabinoside (Ara-C) and aclarubicin (CAG therapy), but this treatment regimen was not effective. He was next treated with G-CSF (started 3 days prior to the administration of anticancer drugs), Ara-C, (200 mg/mm² for 7 days), Etoposide (VP-16, 150 mg/mm² for 5 days) and Idarubicin (8 mg/mm² for 5 days) according to the modified Japan Cooperative Protocol ANLL 91 for children. Although his condition had been septic and he had experienced renal and respiratory failure, he achieved a complete remission after 140 days without additional therapy. The patient returned to a condition of health and received a bone marrow transplant from an unrelated donor. We concluded that this treatment regimen is effective for the relapse of AML in children.

View full abstract

A 30-year-old man who had been given a diagnosis of IgG-κ multiple myeloma by another hospital and treated with melphalan, prednisone, and cyclophosphamide 6 months earlier, was admitted to our hospital in July 1994 because of progressively impaired hearing in both ears, vertigo, and worsening fatigue. Peripheral blood examination showed a white blood cell count 25,000/μl, with 77.5% atypical plasma cells. Examination at the time of hospitalization also revealed retinal hemorrhages and serum hyperviscosity. The diagnosis was plasma cell leukemia with hyperviscosity syndrome. Subsequent treatment consisted of vincristine, doxorubicine, and prednisone and repeated plasmapheresis. This resulted in a partial response and a reduction of serum viscosity but no reversal of hearing loss. One month after admission, left sixth cranial nerve palsy was demonstrated. Cranial computed tomography studies disclosed a tumoral mass in the sphenoid sinus. The patient received local radiotherapy and intensive chemotherapy, but exhibited no notable alleviation of his cranial nerve palsy. He died of septicemia and progressive disease in August 1994. This case was rare in that it involved plasma cell leukemia and bilateral neurosensory hearing loss associated with serum hyperviscosity and sixth cranial nerve palsy due to plasmacytoma within the sphenoid sinus.

View full abstract

We report a case of a 63-year-old woman with γ heavy chain disease (HCD) associated with mucosa-associated lymphoid tissue (MALT) lymphoma of the duodenum. She was suffering from drug-resistant tonsillitis with high fever. Examination on admission showed leukocytopenia and thrombocytopenia. Bone marrow aspirate revealed granulocytosis and a hypocellular marrow with no increase in plasma cells or atypical lymphocytes. Serum electrophoresis disclosed, in addition to hypogamma-globulinemia, an abnormal band due to the presence of γHCD protein. This abnormal protein was a molecular weight of approximately 40kd as determined by Western blots technique, and belonged to the IgG1 subclass as determined by ELISA with monoclonal antibodies against IgG. An endoscopic examination of the patient's duodenum found a small tumorous lesion, which was confirmed pathologically to be MALT lymphoma. HCD is known to be associated with lymphoproliferative diseases. In this case, γHCD had developed as a secondary complication of MALT lymphoma. γHCD associated with MALT lymphoma of the duodenum is rare in the literature.

View full abstract

A 66-year-old man was given a peripheral blood test because of low grade fever. Leukocytosis was detected, and the blood and bone marrow findings were consistent with those of chronic myelomonocytic leukemia. Three months later the hematological findings were: WBC 58,800/μl (19% blastoid cells, 22% monocytes), Hb 9.0 g/dl, and a platelet count of 116×10⁴/μl. A bone marrow examination revealed the presence of 52.6% blastoid cells and dysmegakaryocytopoiesis, including micromegakaryocytes. Serum and urinary lysozyme levels were elevated. Karyotypic analysis detected t(1;3)(p36;q21), but not major bcr/abl mRNA. The patient was given a diagnosis of acute transformation of chronic myelomonocytic leukemia. Despite treatment, he died about 3 months later. t(1;3) is occasionally observed in cases of myelodysplastic syndrome (MDS) and leukemia. Patients with t(1;3) often exhibit dysmegakaryocytopoiesis; furthermore, acute leukemia develops more readily in those who also have MDS. Cases of long-term survival are rare.

View full abstract

A 59-year-old man was referred to our hospital because of pancytopenia. Peripheral blood examination showed a WBC of 1,500/μl with 2% blasts, Hb 8.1 g/dl and a platelet count of 4.1×10⁴/μl. A bone marrow aspiration revealed hyperplasia with proliferation of blasts (15.7%) and myelodysplasia. Chromosome analysis revealed multiple aberrations, including -5, -7, +8. The patient was given a diagnosis of refractory anemia with excess of blasts (RAEB) and treated with combination chemotherapy. Agranulocytosis and high fever remained after chemotherapy, and abdominal pain and diarrhea developed. An abdominal X-ray film and computed tomography scan demonstrated dilated small bowel, thickness of the bowel wall, and ascites. A diagnosis of neutropenic enterocolitis was given. During the WBC recovery period from nadir, massive hematochezia developed in the patient. Angiography detected the leakage of contrast medium from a peripheral region of the first jejunal artery into the jejunal lumen. A partial resection of the jejunum was thus performed, and a histological examination revealed the presence of irregularly dilated blood vessels in the submucosal layer. These findings were consistent with the features of angiodysplasia, and indicate that angiodysplasia should be considered one cause of intestinal hemmorrhage in elderly patients during intensive chemotherapy.

View full abstract