To distinguish hypoplasitc myelodysplasitc syndrome (MDS) from aplastic anemia (AA), morphological abnormalities of bone marrow hematopoietic cells in 8 patients with MDS and 39 patients with AA were studied. All the patients with MDS and AA showed prolonged plasma iron disappearance time, (PIDT_1/2)>120 min. Five hundred erythloid and myeloid cells, as well as 20 megakaryocytes were counted. Dysplastic changes were defined if morphological changes were present in more than 1.0% cells with only one lineage, or in more than 0.6% cells with more than two lineages. Twenty six of 39 patients with AA showed morphological abnormalities. In MDS cases, morphological abnormalities were prominent in trilineage cells in some cases, in bilineage (erythoroid and megakaryocytic or myeloid and megakaryocytic cells), in others, or solely in myeloid cells or in megakaryocytic cells in other cases. Morphological abnormalities seen solely in erythoroid cells, especially those with segmented nuclei were considered to be less significant for the diagnosis of MDS. The findings were considered to be useful to distinguish hypoplastic MDS from AA.

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For the differential diagnosis of pulmonary infiltrates after bone marrow transplantation, cytmegalovirus (CMV) antigenemia was evaluated in 9 episodes of pneumonia which developed in 7 allogeneic marrow transplant patients between 9 and 495 days after transplant. The diagnosis of lung infiltration was made based on clinical findings including histological, cytological or microbiological examinations using bronchoalveolar lavage fluid specimens, sputum or lung tissue. The CMV antigen-positive leukocytes were detected with a direct immunoperoxidase technique using a peroxidase-labeled monoclonal antibody (HRP-C7) against CMV immediate early antigen. The episodes included 2 CMV pneumonias, 1 pneumocystis carinii pneumonia, 1 adenovirus pneumonia, 1 bacterial pneumonia, 1 bacterial and fungal pneumonia, 2 idiopathic pneumonias and 1 capillary leak syndrome associated with hyper acute GVHD. The CMV antigenemia became positive only in two patients with CMV pneumonia and the number of CMV antigen-positive leukocytes exceeded 10 per 50000 WBCs. The CMV antigenemia test required only 24 hours to obtain results. Theses observations suggest that the detection of CMV antigenemia is of great value in the differential diagnosis of pulmonary infiltrates in marrow transplant patients.

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To evaluate the relationship between initial therapy and clinical prognosis, retrospective analyses were performed in 73 children with idiopathic thrombocytopenic purpura treated in our hospital during the past 12 years. The patients were classified into three groups as follows: group A, 34 patients recovered from thrombocytopenia within six weeks after onset; group B, 9 patients recovered in a period of six weeks to six months; group C, 30 patients remained thrombocytopenia beyond six months. The majority of the patients in group A (82%) and group B (56%) provided a history of some infection within the preceding three weeks. The patients in group A and group B had epistaxis or purpura of mucous membranes more frequently than the patients in group C. Although, in gourp A and group B, steroid therapy and intravenous high-dose immunoglobulin (IVIG) therapy has been shown to rapidly increase platelet counts, many patients had become thrombocytopenia again from ten days after starting treatment. Therefore, there was no evidence that steroid therapy and IVIG therapy could reduce a total period of the thrombocytopenia compared with no treatment. The evidence suggested that those therapies, steroid therapy and IVIG therapy, might have influenced the essential natural history of the condition. The mean platelet associated IgG values in group C was significantly greater than in group A and group B with the platelet counts exceeds 100,000/μl.

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Summary: A case of Waldenström's macroglobulinemia (WM) (IgM-κ type) associated with acuteonset demyelinating peripheral neuropathy is reported. A 49-year-old woman was admitted to our hospital because of general fatigue and recurrent syncope attacks. She was treated with vincristine, cyclophosphamide, epirubicin and prednisolone. By 10th hospital day, her clinical condition improved and serum viscosity was reduced. However, on the 21th hospital day, she suffered from rapidly progressive writing and gait disturbance. Neurological examination showed muscular atrophy and weakness in the distal part of four extremities. Deep tendon reflexes were diminished. There was no sensory deficit. Cerebrospinal fluid was normal. Anti-myelin associated glycoprotein activity of her serum was negative. Both motor and sensory nerve conduction velocities were markedly decreased. Biopsy of sural nerve revealed marked demyelination and onion bulb formation. There was IgM deposition on myelin sheath. Minimal axonal changes excluded the possibility of vincristine neuropathy. Plasmapheresis improved her symptoms, but nerve conduction velocities remained unchanged. Polyneuropathy associated with WM is usually gradual onset and sensory dominant. In this case, associated neuropathy was acute onset, progressive and motor dominant. This type of neuropathy in patients with WM is very rare.

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A 78 year old female was found to have pancytopenia in February 1991. Bone marrow was normocellular with 11.7% blasts and showed dysmegakaryopoietic changes. A diagnosis of MDS (RAEB) was made and she was treated with transfusions and ubenimex. Leukemic transformation was noted in July. On Admission in Octover 1991, her laboratory examinations revealed the following: WBC 38,900/μl with 93% blast, Hb 8.0 g/dl, Plt 2.1×10⁴/μl, a hypercellular bone marrow with 74% blasts which were negative for myeloperoxidase (MPO) by light microscopy, but were positive by electron microscopy. Surface marker for CD13 was positive. These findings corresponded to M0 of the FAB subtype. Chromosome analysis revealed Ph¹ chromosome with 46XX, t(9;22)(q34;q11) in 3 of 3 cells examined, Southern analysis showed the rearrangement of the break point cluster region (bcr). Reverse transcriptase polymerase chain reaction technique demonstrated the presence of major bcr/abl mRNA. She was treated with transfusions and methyl-prednisolone. Her blast counts declined and Ph¹ chromosome was only positive in 1 of 12 metaphses examined. She died of pneumonia in December 1991. Eleven cases with MDS showing Ph¹ chromosome have previously been reported. The observations indicate that Ph¹ chromosome positive acute leukemias were heterogenous in nature.

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A 66-year-old female was admitted to our hospital because of leukocytosis, anemia and splenomegaly in August 1989. The white cell count was 3.49×10¹⁰/l with 88.5% of the leukemic cells which were morphologically similar to prolymphocytes. On flowcytometric analysis, the leukemic cells were found to be positive for B-cell markers such as CD19, CD20, FMC7, Sm-IgM and Sm-IgD and negative for CD5 and CD25. The chromosome analysis demonstrated hyperdiploidy of 48, XX, (+3, +18). She was diagnosed as having B-cell prolymphocytic leukemia, and treated with α-interferon and VP therapy with progression. Complete remission was achieved after three courses of ranimustine (MCNU) administration. She relapsed after about one year without therapy, but when MCNU was administered again, a secondary remission followed. The prolymphocytes during the relapse stage also had the phenotypes of CD11b, CD13 and CD25. This case is considered to be rare with respect to both complete remission by MCNU and the immunophenotypic change of leukemic cells during the relapse period.

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Extramedullary plasmacytoma arising in the kidney is uncommon. So far only 11 cases have been reported. Recently, we noted the 12th case, the third case seen at the Kyoto University Hospital. The literature is reviewed. A 43-year-old man was hospitalized because of paraplegia due to spinal cord compression by the solitary plasmacytoma arising in the cervical spine, producing an IgG-λ paraprotein. The tumor excision was followed by radiotherapy and standard chemotherapy. Two years later, new lesions on the thoracic spine, the left clavicle, and the occipital bone successively developed with Bence Jones proteinuria (λ), but without IgG-λ paraproteinemia. Ga-67 scanning was performed to detect extramedullary tumors. The marked accumulation of the radionuclide revealed intraabdominal tumors. A CT scan of the abdomen revealed a large soft-tissue mass (73×50mm) emanating from the right kidney and several enlarged lymph nodes. Ultrasound of the right kidney revealed a solid mass with hydronephrotic change. RI-renogram showed delayed excretion from the right kidney. Clusters of myeloma cells were repeatedly observed in the urinary sediment. Thus, a diagnosis of plasmacytoma of the kidney was made. Treatment with radiotherapy alone was sufficient.

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A 34-year-old man was admitted with lumbago and anemia in November 1992. Hematological examination revealed an Hb 9.2g/dl, WBC count 13,500/μl (33% blasts), and monocyte count 3,400/μl. Bone marrow examination showed hyperplasia with dysplasia in trilineage blood cells and increased blasts (21.8%). A diagnosis of refractory anemia with excess of blasts in transformation (RAEB in T) was made. Cytochemical examination revealed the neutrophils in the peripheral blood were 66.5% positive for alpha-naphthyl butyrate esterase inhibited by sodium fluoride, 4.0% positive for peroxidase and 75% positive for alkaline phosphatase. The results of immuno-alkaline phosphatase stainings (avidin biotin alkaline phosphatase complex method) of neutrophils were as follows; CD16 (94.5%), CD24 (91.0%), CD13 (93.0%), CD14 (52.5%), CD33 (39.0%), CD36 (16.5%), HLA-DR (17.0%). These neutrophils exhibited monocyte-specific features and failed to show characteristics of neutrophils.

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A case of pure red cell aplasia (PRCA) with various complications polyarthritis, angitis, acute renal failure and DIC was successfully treated with steroid pulse therapy was described. A 55-year-old woman was hospitalized with a 9-month of intermittent but progressive joint pain, morning stiffness, general fatigue, and fever. Her initial laboratory evaluation revealed a hemoglobin of 4.4g/dl and absence of reticulocyte. Her bone marrow aspirate showed no erythroblast which was compatible with a diagnosis of PRCA. Marked leukocytosis and thrombocytosis, positive antinuclear antigen, elevation of gammaglobulin and C-reactive protein and the presence of polyarthritis and angitis which was confirmed by renal angiography, indicated an underlying autoimmune disorders. Steroid pulse therapy was administered at 500 mg/day for 3 days, resulting in the complete response in both red cell aplasia and above findings. PRCA is known to be associated with systemic lupus erythematosus and rheumatoid arthritis very rarely, but this case did not fulfill the criteria of known collagen diseases, and there is no previous report representing PRCA with various complications such as polyarthritis, angitis and acute renal failure. This case may help us to understand more about the relationship between PRCA and autoimmune disorders.

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A 60-year-old hemophiliac man complaining of abdominal discomfort was admitted on suspicion of hepatocellular carcinoma (HCC) by ultrasonography (US). When he was 22-year-old, blood transfusion was given for intra-abdominal hematoma due to an external wound and a diagnosis of moderate hemophilia A and non-A non-B type chronic hepatitis (later, C type chronic hepatitis) was made at the age of 40. After admission, HCC in the S₆ segment of the liver was diagnosed by US, computerized tomography, magnetic resonance imaging and angiography. He was treated with transcatheter arterial embolization (TAE) and partial resection of the liver. Later, percutaneous ethanol injection therapy and TAE was performed because of the recurrence of HCC. He is now alive with no evidence of recurrence. The rate of infection with hepatitis C virus (HCV) is significantly high in hemophiliacs but, because of the replacement therapy, their life expectation has dramatically improved in last two decades. Therefore, it is speculated that the incidence of HCC in hemophiliacs will increase. We emphasize that regular US examination for HCV-positive hemophiliacs is important for the early detection of HCC.

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An 83-year-old male was admitted with pulmonary tuberculosis. He was started on rifampicin, isoniazid, and stereptomycin (SM). The hematological data at 12 weeks after the treatment showed pancytopenia (RBC: 2.14×10⁶/μl, Hb: 7.2g/dl, Plt: 1.8×10⁴/μl, WBC: 700/μl). All the above medicines were discontinued and he received bolus methylprednisolone (bmPSL) and recombinant human granulocyte-colony stimulating factor (rhG-CSF). After 3 cycles of bmPSL, red blood cells and platelets gradually increased. White blood cells also increased in response to rhG-CSF. Bone marrow aspirate and biopsy specimens showed normocellularity, indicating recovery from aplastic anemia. Drug lymphocyte stimulation test was positive for SM.

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In order to evaluate the usefullness of magnetic resonance imaging (MRI) in estimating bone marrow cellularity, we performed T1-weighted MRI of the lumbar vertebral marrow in two patients with severe aplastic anemia, before and after bone marrow transplantation (BMT). High signal intensity was detected in the bone marrow before BMT, but the bone marrow pattern of MRI became normal after BMT. MRI seems to be effective for evaluating bone marrow cellularity and recovery, and for monitoring therapeutic effects in patients with aplastic anemia before and after treatment with BMT.

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A 58-year-old woman was hospitalized in March, 1991, with slight breathlessness on exertion. Laboratory investigations revealed M-protein (IgG λ type, 7,575mg/dl) in serum, and Bence-Jones proteinuria. Osteolytic bone lesions were noted roentgeno-logically. Bone marrow aspiration showed the presence of 19% of atypical plasma cells, and the case was diagnosed as multiple myeloma. The chest X-ray film showed bilateral diffuse micronodules, which were found by a transbronchial lung biopsy. to be diffuse parenchymal amyloid deposits The patient was treated with melphalan, prednisolone and interferon-α for 6 courses. Clinical symptoms improved but no decrese of the level of M-protein was observed. The patient is still alive without changes of pulmonary shadows on X-ray films for the past 2 years.

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