Kazuaki Moridaira

Myelodysplastic syndromes with nephrotic syndrome

It is sometimes reported that the immunological abnormalities in myelodysplastic syndromes (MDS) induce autoimmune disease (i.e., acute systemic vasculitic syndrome, chronic cutaneous vasculitis, polyneuropathy, relapsing polychondritis, and steroid‐responsive pulmonary disorders). We investigated the clinical features of patients with MDS accompanied by nephrotic syndrome. We enrolled 125 patients with MDS who were admitted between January 1979 and May 1996 in this study. The renal function was assessed based on the laboratory data and the findings at the physical examination. The diagnoses of nephrotic syndrome and glomerular disease were established when 24‐hr urinary excretion was more than 3.5 g and serum total protein was less than 6.0 g/dl, and when the 24‐hr protein excretion was more than 1.5 g. Five patients (4%) had glomerular disease, and three (2.4%) had nephrotic syndrome. Of the five patients with glomerular disease, two had refractory anemia (RA), and three had chronic myelomonocytic leukemia (CMMOL). Three of the total 11 patients with CMMOL were diagnosed as having nephrotic syndrome. Among the CMMOL patients, those with nephrotic syndrome showed higher absolute monocyte numbers than did those without nephrotic syndrome (8830 ± 4677/μl vs. 3061 ± 2887/μl, P = 0.03). One CMMOL patient was treated with VP‐16 and hydroxyurea. As the white blood cell count in this patient decreased, the 24‐hr urine protein excretion and the serum tumor necrosis factor alpha level decreased. The relationship between nephrotic syndrome and CMMOL was not clear. High monocyte count and the serum cytokines in MDS patients may play a partial role in the evolution of glomerulonephritis, and CMMOL may be closely related to nephrotic syndrome. Am. J. Hematol. 60:200–204, 1999.

Primary Hodgkin's disease of the bone presenting with an extradural tumor

We report a very rare case of primary Hodgkins disease of the bone in a 56-year-old male. It presented with sudden onset of paraparesis which suggested an extradural tumor. Early appropriate therapy for similar patients with Hodgkins disease can improve their prognosis.

A staging system for multiple myeloma based on the morphology of myeloma cells

Abstract: The morphology of myeloma cells is reported to be one of the prognostic factors in multiple myeloma (MM) patients. We analyzed the prognostic factors, including morphological classification, in 292 patients with MM in order to select poor‐risk patients who should be considered candidates for early intensive chemotherapy, including stem cell transplantation. Multivariate analysis was applied to 90 patients diagnosed between 1989 and 1996, because serum beta‐2‐microglobulin (β2M) has been measured regularly since 1989, and showed that serum albumin, serum β2M, and the morphology of myeloma cells predicted survival. According to these factors, patients were divided into 3 risk groups; a high‐risk group (14%), a intermediate‐risk group (46%) and a low‐risk group (40%). There were significant differences between survival times in these 3 groups (median survival: high‐risk, 16; intermediate‐risk, 22; and low‐risk, 44 months).

In long-term bedridden elderly patients with dietary copper deficiency, biochemical markers of bone resorption are increased with copper supplementation during 12 weeks

Background: Although the effect of copper on bone has been tested in animals and healthy subjects, no studies concerning the effect of copper supplementation on bone metabolism in patients with copper deficiency have been reported because of the rarity of these patients. This study was conducted to investigate the effect of copper supplementation on bone metabolism in copper-deficient patients. Method: This study included 10 patients (83.7 ± 8.3 years) with dietary copper deficiency under long-term bed rest for more than 12 months. They had their diets supplemented with copper sulfate (3 mg/day) over 12 weeks in addition to their diet of only one kind of enteral food with a low concentration of copper. Serum copper and ceruloplasmin, urinary deoxypyridinoline (DPD) and collagen-type 1 N-telopeptide (NTX) (biomarkers of bone resorption), serum osteocalcin (OC) and bone-specific alkaline phosphatase (Bone ALP) (biomarkers of bone formation) were analyzed at baseline, 4 and 12 weeks after copper supplementation. Results: DPD and NTX excretion were significantly increased 4 weeks after copper supplementation (p = 0.009 and p = 0.013, respectively). Serum bone ALP and OC were not significantly changed 12 weeks after copper supplementation (p = 0.051 and p = 0.594). Conclusions: In patients with nutritional copper deficiency, bone resorption markers are increased with copper supplementation.

Spurious macrocytosis associated with non-Hodgkin's lymphoma: report of two cases.

SummaryWe report two rare cases of spurious macrocytosis (SM) associated with non-Hodgkins lymphoma caused by cold agglutinin. SM was reversed, accompanied by the regression of lymphoma in case 1 but not in case 2, where the lymphoma was refractory to the chemotherapy. It is strongly suggested that lymphoma cells were related to the production of cold agglutinin and the cause of SM.

Non-Hodgkin's Lymphoma following Acute Myeloid Leukemia in Complete Remission

A 57-year-old female was admitted with low grade fever, leukocytosis, anemia and thrombocytopenia. Her white blood cell count was 59.7 ! 109/l, with 83% leukemic cells, some of which had Auer rods. The blasts were myeloperoxidase-positive with a myeloid immunophenotype (CD33: 99.1%, CD13: 8.5%, CD19: !1.0%, CD20: !1.0%, HLA-DR: 18.4%). She was diagnosed as having AML (M2) and underwent combination chemotherapy (the Japan Adult Leukemia Study GroupAML89 regimen which includes enocitabine, cytarabine, mercaptopurine, methotrexate, daunorubicin, aclarubicin, mitoxantrone, vindesine, etoposide, prednisolone), resulting in complete remission. She was well thereafter and was followed as an outpatient without treatment. Thirty-seven months following the diagnosis of AML, however, she complained of nausea and abdominal pain. Her serum lactate dehydrogenase level rose from 313 to 844 and 1,371 IU/l within 2 months (normal: less than 310). When she was readmitted for investigation, the peripheral blood counts were normal and examination of her bone marrow showed complete remission. Serum CA 19-9 and CA 125 levels were elevated to 145.6 ng/ml (normal: less than 60) and 315 U/ml (normal: less than 35), respectively. The chest X-ray revealed a small amount of bilateral pleural effusion, and the computed tomography scan of the abdomen showed multiple intrahepatic tumors and a moderate amount of ascites (fig. 1). There was no evidence of another tumor or lymphadenopathy. We suspected that she had carcinoma of the digestive or gynecologic system because of the high levels of the two characteristic tumor markers. However, no abnormality was detected by the gastrointestinal and gynecologic study. Examination of the pleural effusion and ascites showed no malignant or clonal cells, but 1 month later, reexamination of the patient’s ascites showed atypical cells (fig. 2). She died within 1 week of disseminated intravascular coagulation. The autopsy revealed diffuse, medium-sized, B cell non-Hodgkin’s lymphoma [LCA (CD 45); L26 (CD20)and UCHL1 (CD45RO)-positive but neutrophilic esterase-negative] in the liver tumors (maximum diameter 6 cm). There was also microscopic infiltration by the lymphoma cells into the diaphragm, omentum, peritoneum, stomach, parapancreatic region and para-aortic lymph nodes.