[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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Clinicopathological analyses of 6 cases of mantle zone lymphoma (MZL) were carried out. The median age of the patients was 62 years with a range of 41 to 71 years and the male-to-female ratio was 1:1. Superficial lymph node (LN) swelling was present only in 2 patients. Giant LN swellings of the mesenteric or inguinal regions were present in 4, and bone marrow involvement by lymphoma cells in 5. Serum protein electrophoresis revealed a monoclonal protein of IgM κ type in 2 patients. One of these also had polyclonal hypergammaglobulinemia. An immunohistochemical study of 6 patients revealed LN-1-∼+, LN-2+∼++, sIgM+, sIgD+, CALLA±∼+, DRC-1+∼++. The immunohisto-chemical features of the cases were similar to those of small lymphocytic lymphoma or follicular lymphoma. Only 1 patients out of 6 achieved complete remission. Two patients died, one of pneumonia after chemotherapy and the other of cancer. The others were alive 4 to 100 months after the diagnoses. Although giant LN swelling and bone marrow involvement of lymphoma cells which were refractory to treatments were frequently observed, we consider MZL to be a slowly progressive and low-grade type of non-Hodgkin's lymphoma.

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The authors evaluated the efficacy of a daily administration of recombinant human α 2a interferon (IFN), given at a dose of 300MU for 12 consecutive days, in patients with steroid-nonresponsive or-dependent idiopathic thrombocytopenic purpura (ITP). Nine patients received courses of IFN therapy. Mean platelet counts rose from 1.39 to 10.9×10⁴/μl and PAIgG decreased from 151.7 to 59.7 ng/10⁷ cells. The maximum rise in platelet counts occurred from 10 to 42 days (mean 19.1) after the initiation of IFN. Complete response (CR) was achieved in 3 of 13 courses (23.1%), and partial response (PR) in 8 (61.5%). One CR case continued for longer than 20 months without further treatment, but intermittent IFN therapy was necessary for the other. The increment of the platelet counts was transient in all of the partial responders. No severe side effect requiring interruption of the course of IFN was experienced. Both serum IgG and PAIgG significantly correlated with the increment of platelet counts, therefore the mechanism of IFN on ITP was presumed to be associated with the inhibition of autoantibody production. Daily administration of IFN appears to be an effective and safe treatment protocol for refractory ITP.

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We established the diagnostic criteria of pure erythrocytosis (PE) and spurious polycythemia (SP), and investigated the different points between 4 PE patients and 4 SP patients who had clinical course longer than 5 years from diagnosis. The red cell mass (RCM) was greater in PE patients than in SP patients, but the difference was not significant. However, the actual measurement of RCM was significantly greater in PE than their predicted values (p<0.01), and was not significant in SP. Such laboratory data as red cell counts, Hb, Hct, reticulocyte counts, M/E ratio of bone marrow pictures, the positive rate and score of NAP, and bone marrow CFU-E numbers were not significantly different in the two groups, and serum EPO levels in patients with PE were significantly lower than those in patients with SP (p<0.01). When in hospital, the Hct levels of patients of both groups decreased linearly. Sustained erythrocytosis was found in two patients with PE and one patient with SP. Vascular complications in patients of both groups have not been observed. There was no essential difference between the two groups. Therefore, it is suggested that PE patients without endogenous erythroid colonies may follow almost the same clinical course as SP patients.

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We measured appearance rates of RNA-rich reticulocytes in 8 patients undergoing allogeneic bone marrow transplantation, using a Sysmex R-1000 reticulocyte counter which utilizes laser flow cytometry. The changes in proportion of RNA-rich reticulocytes (high fluorescence ratio: HFR) and maturation index (MI: HFR+middle flourescence ratio/low fluorescence ratio) were compared with those of WBC, neutrophil or reticulocyte counts. Engraftment was defined as an HFR of ≥5% or a MI of ≥15%. Engraftment was confirmed significantly earlier by HFR (13.5±2.4days) and MI (13.1±2.9days) than by neutrophils (17.1±3.2days) or reticulocytes (20.4±6.2days). The maturation of reticulocytes would be a useful indicator for engraftment or recovery from marrow aplasia in cases of bone marrow transplantation.

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Busulfan (BU) concentrations in the blood and spinal fluid of 7 patients pretreated with BU for bone marrow transplantation (BMT) were measured using gas chromatography. The data from these periodically obtained samples were used to study the relationship between the BU concentration and complications (e.g. vomiting), indicating that vomiting leads to a lower maximum BU level. The trough level of BU concentration at 6 hours after administration was stable at around 500 ng/ml, not showing little effect of vomiting. The BU concentrations in the spinal fluid were virtually the same as those in plasma collected at the same time and the spinal fluid/plasma ratio of the BU concentration averaged 1.06. No veno-occlusive disease (VOD) was noted. Failure of engraftment occurred in one case of myelofibrosis, however, as the plasma BU concentration in this case was not lower than the others, the graft failure was not considered to be due to the preconditioning regimen including BU.

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A case of Mycoplasma pneumonia complicated with severe hemolytic anemia, which occurred as a result of a high titer of cold agglutinin is presented. A 49 year-old male was admitted because of fever, jaundice and dyspnea. Chest x-ray showed diffuse small nodular infiltrates throughout both lung fields. Laboratory studies disclosed the following values: Hb 4.6 g/dl, Ht 13.9%, reticulocyte 11.5%, direct and indirect Coombs' test positive, haptoglobin 38 mg/dl, ESR 145 mm/hr, cold agglutinin titer 1: 2,048 mycoplasma antibody titer 1: 640, PPD negative. The diagnosis of autoimmune hemolytic anemia associated with Mycoplasma pneumonia was made, and treatment with minocycline and prednisolone observed striking clinical improvement. It was sussested that the cold exposure was possibly a major factor in the pathogenesis of hemolysis in this patient.

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A patient with CML showed monoblastic crisis which started with extramedullary tumor formation in a rib before medullary involvement. She was diagnosed as having CML in 1984 at the age of 57. In February 1990, she was admitted to Furukawa City Hospital because of extramedullary blastic crisis beginning at the right 5th rib. At that time, the bone marrow revealed 4.6% blasts. On March 5, after one coures of chemotherapy, she was transferred to our hospital for radiotherapy. Hematological findings were WBC 10,100/μl with 10% blasts, Hb 10.9 g/dl, platelet 3.7×10⁴/μl. Bone marrow aspiration was unsuccessful. The blasts in the peripheral blood were negative for peroxidase and chloroacetate esterase; but positive for naphtylbutyrate esterase. The leukemic cells were positive for CD13, CD33, and had phagocytic activity. Chromosomal analysis revealed 46XX with Ph¹ chromosome and some additional anomalies. Southernblot analysis of tumor cells shows BCR rearrangement. These findings suggest that the blasts were immature monocytic cells, and we conclude that this is a rare case of extramedullary monoblastic crisis of CML.

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In order to evaluate the usefulness of MRI in estimating bone marrow cellularity, we performed MRI of the lumbar spine in two patients with severe aplastic anemia, before and after successful treatment with antithymocyte globulin (ATG). Case 1, a 25-year-old man with idiopathic aplastic anemia, was treated with ATG 6 months after the onset. One month after treatment, his peripheral blood count and bone marrow cellularity recovered, and the MRI bone marrow pattern became normal. Case 2, a 78-year-old woman with drug-induced aplastic anemia, was treated with ATG 4 months after the onset. Three months after treatment, her peripheral blood count improved. Five months after treatment, her bone marrow cellularity recovered and the MRI bone marrow pattern was normal for her age. Seven months after treatment, when her peripheral blood count was almost normal, we observed hypercellular bone marrow restoration at the periphery of the vertebrae. MRI seems to be an effective method of evaluating bone marrow recovery in aplastic anemia.

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A 54-year-old man who had been known to have a high prolymphocyte count for four years was admitted to our hospital because of dyspnea in September, 1990. Physical examination revealed skin eruption, lymphadenopathy and hepatosplenomegaly. Chest X-ray demonstrated bilateral pleural effusions. The leukocyte count was 232,900/μl with 99% lymphoid cells possessing single nucleoli. The cells expressed the phenotype CD2+, CD3-, CD4+, CD7+, and CD8-. Southern blot analysis of DNA from these cells revealed monoclonal rearrangement of T-cell receptor beta-chain genes. Anti-human T-cell lymphotropic virus type 1 (HTLV-1) antibody and HTLV-1 proviral DNA were not detected. A biopsy specimen from the skin lesions showed infiltration of the leukemic cells which were positive for anti-MT1 antibody. Histological finding of the axillary lymph node was malignant lymphoma, diffuse, medium-sized, T-cell type. Combination chemotherapy resulted in the improvement of skin eruption, lymphadenopathy, hepatosplenomegaly and pleural effusions, although his prolymphocyte count increased to 910,000/μl. He died of cerebral bleeding in July, 1991. We diagnosed this case as T-cell prolymphocytic leukemia, observed for five years.

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A 77-year-old male who had suffered from an upper respiratory infection and had been given Norfloxacin (NFLX) on May 2, 1990, developed generalized erythema which did not subside with prednisolone. He was hospitalized on May 8, and Stevens-Johnson syndrome was diagnosed. The WBC was 115,400/μl (Ly 61.0%, Aty Ly 39.5%). Sternal tap revealed hypercellular marrow with increased lymphocytes (48.5%; Aty Ly 24.5%) and eosinophils (7.0%). Clinical chemistry revealed slightly abnormal liver and renal function with LDH (1,745IU/l) and IgE (803IU/ml) elevation. No pathognomonic result was obtained with several viral antibodies. CD4+, CD8+ lymphocytes and the 4/8 ratio were 39%, 43% and 0.9, respectively. Clinical and laboratory abnormalities were normalized within 3 weeks after the discontinuance of all drugs. Positive lymphocyte stimulation test results were obtained by NFLX. While drug allergy is known to be a cause of IM-like syndrome, there are few reports regarding the subset characterization of the increased T lymphocytes. In this case, T lymphocytosis was remarkable, but the 4/8 ratio declined only slightly, indicating that CD4+ as well as C8+ cells were activated and increased, unlike IM. The record of this case helps to clarify the mechanisms of the lymphocyte activation shared and not shared by EBV-induced IM and IM-like syndrome.

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The authors report six cases of non-Hodgkin's lymphoma (NHL) (3B-cell type, one T-cell type, one non-T non-B cell type, one unclassified type) occurring subsequently to autoimmune diseases. The patients were females aged 43 to 70 (median 61). Rheumatoid arthritis was most frequent as the preceding autoimmune disease, and the intervals from the onset of an autoimmune desease to that of NHL were 10 to 36 years (median 20). Polyclonal hypergammaglobulinemia was seen in 4 cases, lymphocytopenia in 3 cases, and conversion to negative PPD reaction in 2 cases. Only one patient had been given corticosteroids, and immunosuppressive agents may not contribute much to the development of lymphoma in patients with autoimmune diseases.

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A 59-year-old woman was admitted in February 1991, because of abdominal distension. On admission, she had splenomegaly, autoimmune hemolytic anemia, and idiopathic thrombocytopenic purpura. Evans syndrome had been diagnosed and daily prednisolone therapy had been performed. After this therapy, a rapid increase of lymphocytes was observed accompanied with contraction of spleen. The monoclonal proliferation of B-lymphocytes and rearrangement of JH and J κ gene were detected and chronic lymphocytic leukemia was diagnosed. With reduction of prednisolone, the lymphocyte counts decreased and the size of the spleen returned to the previous state. It is suspected that the cause of rapid lymphocytosis in this case was due to the redistribution of lymphocytes from the spleen to the peripheral blood.

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The authors measured the level of interleukin-6 (IL-6), endotoxin and CRP from 7 patients of documented sepsis with hematological disorders. IL-6 was higher in patients who developed septic shock, compared with patients who had only sepsis. These data revealed the importance in the level of IL-6, rather than endotoxin and CRP, in managing the patients with septic shock.

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A 64-year-old woman was admitted for treatment of malignant lymphoma involving the pharynx and abdomen. Lymphoma disappeared after chemotherapy and radiotherapy, but she had central nervous system symptoms; euphorism, left facial nerve palsy, right hemiplesia, and disturbance of micturition. Magnetic resonance imaging (MRI) revealed a diffuse dural thicking and a subdural fluid retention. The subdural fluid was determined to be an exudate, and a biopsy of the dura mater revealed a normal dura and a fibrin clot containing lymphoma cells and neutrophils. MRI can be very useful to detect the meningeal involvement of malignant lymphoma.

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A 47-year-old male case of IgG-κ type multiple myeloma was treated with VMCP and recombinant human alpha-interferon (IFN-α2a). The direct Coombs test was positive before treatment. Hemolytic anemia associated with massive hematuria was observed during the administration of 9 million IU IFN-α2a per day for 2 weeks. The hemolytic symptoms rapidly improved after withdrawal of IFN-α2a. This clinical course suggests that IFN-α as an immunomodulator was responsible for the progression of autoimmune hemolytic anemia in a case of multiple myeloma.

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A 70-year-old female was found to have class V cytology on an endometrial smear, and a histological diagnosis of malignant lymphoma was made by endometrial biopsy. The pathological diagnosis was malignant lymphoma, diffuse large cell-type according to the Working Formulation classification. Immunohistochemical staining showed lymphoma cells to be positive for CD 20 (B 1), indicating B cell lineage. Two cervical lymph nodes were palpable, and swelling of a para-aortic lymph node was also found by abdominal CT scan. The clinical stage was determined to be III according to the Ann Arbor classification. After three courses of CHOP chemotherapy, lymphoma cells could no longer be detected by endometrial biopsy, and the para-aortic and cervical lymphadenopathy also disappeared. Primary malignant lymphoma of the uterus, especially of the uterine body, is very rare. Only eight cases of primary malignant lymphoma of the uterine body were reported in the literature. The cell lineage was decided in only one case, which was B cell type.

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