A 66-year-old woman with acute monoblastic leukemia with t(11;22)(q23;q11), which was subsequently transformed into myeloblastic leukemia is reported. Such karyotype abnormality has not been reported in acute monoblastic...A 66-year-old woman with acute monoblastic leukemia with t(11;22)(q23;q11), which was subsequently transformed into myeloblastic leukemia is reported. Such karyotype abnormality has not been reported in acute monoblastic leukemia. Cytogenetic analysis revealed 47,XX,+8,t(11;22)(q23;q11) in 19 out of 20 metaphases and 46,XX,del(7)(q22-q36),t(11;22)(q23;q11) in the remaining metaphase. Variant Ph1 was ruled out due to the absence of chimeric bcr-alb mRNAs. In remission cytogenetic findings showed normal karyotype. After 4 months, the patient relapsed with 20% myeloblasts replacing monoblasts in bone marrow. Cytogenetic analysis revealed 46,XX,del(7)(q22-q36),t(11;22)(q23;q11) in all metaphases. A possible explanation for this lineage switch may be the involvement of myelomonocytic progenitor cells.
A previously well 53-year-old man with fulminant myocarditis is presented. Cardiogenic shock and medically refractory low cardiac output syndrome were unsuccessfully treated using an intraaortic balloon pump. A percutane...A previously well 53-year-old man with fulminant myocarditis is presented. Cardiogenic shock and medically refractory low cardiac output syndrome were unsuccessfully treated using an intraaortic balloon pump. A percutaneous extracorporeal bypass system improved hemodynamics, DIC and other multiorgan injuries following acute myocarditis.
A patient with plasma cell myeloma refractory to both MP (melphalan-prednisolone) and VAD (vincristine-adriamycin-dexamethasone) was treated with a combination chemotherapy consisting of VP-16, ifosfamide and dexamethaso...A patient with plasma cell myeloma refractory to both MP (melphalan-prednisolone) and VAD (vincristine-adriamycin-dexamethasone) was treated with a combination chemotherapy consisting of VP-16, ifosfamide and dexamethasone (VID). A partial remission of 12-month duration was achieved. VID combination might be considered for myeloma patients who are refractory to both MP and VAD, and not eligible for high-dose melphalan with bone marrow transplantation.
We report a case of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura (Evans' syndrome) associated with gastric plasmacytoma. Recognition of this rare entity is important because surgery and chemotherap...We report a case of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura (Evans' syndrome) associated with gastric plasmacytoma. Recognition of this rare entity is important because surgery and chemotherapy together may achieve a cure. Possible mechanisms causing the hemolytic anemia and thrombocytopenia are discussed.
We investigated computed tomographic (CT) films of 4 pathologically documented cases of progressive supranuclear palsy (PSP) in which the clinical presentations were atypical and compared the findings with those of 15 pa...We investigated computed tomographic (CT) films of 4 pathologically documented cases of progressive supranuclear palsy (PSP) in which the clinical presentations were atypical and compared the findings with those of 15 patients with Parkinson's disease (PD). Dilatation of the third ventricle, atrophy of the midbrain tegmentum, and enlargement of the interpeduncular cistern toward the aqueduct were found to be the characteristic findings in PSP. Thus, radiological findings can be useful when the differential diagnosis between PSP and PD is clinically difficult.
To evaluate the cardiovascular and plasma catecholamine responses to dynamic exercise in patients with cardiac neurosis (CN), treadmill testing was performed. Thirty-four patients with CN were chosen for this study based...To evaluate the cardiovascular and plasma catecholamine responses to dynamic exercise in patients with cardiac neurosis (CN), treadmill testing was performed. Thirty-four patients with CN were chosen for this study based on exercise tolerance and the results were compared with those in 31 patients with organic heart disease and 12 normal subjects. Patients with CN showed an augmentation of cardiovascular and plasma catecholamine responses. The augmentation of the norepinephrine response in patients with CN was not as remarkable as that in patients with organic heart disease. On the other hand, the augmentation of the epinephrine response was greater in patients with CN than in those with organic heart disease. Administration of metoprolol (40 mg/day) for two weeks improved exercise tolerance in patients with CN. We suggest that anxiety augments both sympatho-neural and sympatho-adrenal activity and that it is the symptoms induced by the augmented cardiovascular response which reduce exercise tolerance in patients with CN.
In order to detect diagnostic clues to identify patients with chronic immune thrombocytopenia which is likely to develop systemic lupus erythematosus (SLE), a retrospective study was conducted. Of 39 patients with chroni...In order to detect diagnostic clues to identify patients with chronic immune thrombocytopenia which is likely to develop systemic lupus erythematosus (SLE), a retrospective study was conducted. Of 39 patients with chronic immune thrombocytopenia, 4 fulfilled the diagnostic criteria being between 4.5 and 14 years after the initial diagnosis of "idiopathic" thrombocytopenic purpura. The remaining 35 patients were found to be idiopathic thrombocytopenic purpura (ITP) after at least a 5-year follow-up. It was noted that in patients with SLE who had thrombocytopenia at presentation, only 4 or 5 findings of diagnostic criteria were observed. Of particular interest, all patients who developed SLE were positive for anti-SS-A antibody, in contrast none of the patients with ITP had this antibody. Furthermore, despite the few clinical manifestations, we noted lupus peritonitis in 1 patient and pulmonary hypertension in another patient. In conclusion, the anti-SS-A antibody is an excellent clue for the early detection of SLE occurring as "idiopathic" thrombocytopenic purpura.
Coronary risk factors were assessed in 186 consecutive patients who received coronary angiography. The severity of coronary luminal narrowing was scored as the coronary sclerosis index (CSI). Patients were divided into n...Coronary risk factors were assessed in 186 consecutive patients who received coronary angiography. The severity of coronary luminal narrowing was scored as the coronary sclerosis index (CSI). Patients were divided into normal coronary arteries (N, n = 72), coronary sclerosis without infarction (C, n = 73) and previous myocardial infarction (MI, n = 41). The CSI increased with age. A significant difference in serum triglycerides, HDL cholesterol and atherogenic index was observed between Groups C or MI and N. Multivariate analysis revealed that CSI had correlated with total- and HDL-cholesterol, uric acid and age in subjects under 55 years; and with age, blood sugar, factor H and HDL cholesterol in those of 55 years or over. When patients were classified by their total and LDL cholesterol level, a significantly different CSI was found between the desirable and high cholesterol levels in subjects under the age of 55, but it was not significant in those over 55. Therefore, disorders in lipid metabolism should be corrected in early middle age.
Ten patients with human T lymphotropic virus type I-associated myelopathy (HAM), 5 asymptomatic HTLV-I carriers and 11 healthy normal volunteers were studied to determine if peripheral blood lymphocytes spontaneously rel...Ten patients with human T lymphotropic virus type I-associated myelopathy (HAM), 5 asymptomatic HTLV-I carriers and 11 healthy normal volunteers were studied to determine if peripheral blood lymphocytes spontaneously release IL-2 and soluble IL-2 receptors. Peripheral blood lymphocytes obtained from HAM patients proliferated spontaneously when cultured for 5 days in vitro. Proliferating cells were CD3+ lymphocytes and both CD4+ cells and CD8+ cells as shown by morphologic and immunohistochemical observations. These T cell responses were also found in asymptomatic carriers, but the responses were not as marked as those of HAM patients. IL-2 activity in the culture supernatants was much higher in HAM patients than in asymptomatic carriers; IL-2 activity correlated well with the intensity of spontaneous proliferation of lymphocytes. Furthermore, soluble IL-2 receptors in the cell-free supernatants from HAM patients were markedly increased compared to those from asymptomatic carriers. These results indicate that spontaneous proliferation of T cells is intimately related to HTLV-I infection and is probably due to autocrine or paracrine pathways which involve IL-2 and IL-2 receptor system.
We examined several immunological parameters such as the number of T cells with CD4 antigen and the receptor for the Fc portion of IgA (T alpha cells), in vitro immunoglobulin production by peripheral blood lymphocytes w...We examined several immunological parameters such as the number of T cells with CD4 antigen and the receptor for the Fc portion of IgA (T alpha cells), in vitro immunoglobulin production by peripheral blood lymphocytes with or without pokeweed mitogen and serum levels of immune complexes in 19 healthy family members of patients with IgA nephropathy (IgAN). It was shown that the levels of serum IgA, T4 cells, CD4/CD8 ratio of T cell subsets, T alpha cells, IgA circulating immune-complexes and spontaneous synthesis of immunoglobulins were significantly increased in family members of patients with IgAN. No family members had proteinuria or hematuria. It was concluded that immunological abnormalities, including abnormalities of T cells and B cells, were found not only in patients with IgAN but also in their healthy family members. It was suggested that some factors in addition to these immunological abnormalities may be involved in the development of IgAN.
The clinical usefulness of ultrasound scanning in the assessment of thyroid nodule was examined. The total diagnostic accuracy for aspiration biopsy cytology (ABC) in the differentiation of benign from malignant thyroid...The clinical usefulness of ultrasound scanning in the assessment of thyroid nodule was examined. The total diagnostic accuracy for aspiration biopsy cytology (ABC) in the differentiation of benign from malignant thyroid nodules was 92.4% in 26 patients, which is similar to that of previous reports when compared with the findings of pathological examination by surgery. Additionally, the diagnostic efficacy of high resolution ultrasonography was evaluated based on the results of ABC. When the echogram of 90 patients with a thyroid nodule was divided into eight patterns according to Obara's classification, only 64.3% of the carcinoma exhibited the typical malignant pattern of ultrasonography and 21.4% of the carcinoma exhibited the ultrasonographically homogenous nodule with a clear margin, which was often observed in the benign nodules. Therefore, it is necessary to be very careful when differentiating malignant from benign thyroid nodules by ultrasonography. In conclusion, although high resolution ultrasonography provides useful information for the assessment of most thyroid nodules, all ultrasonography (including high resolution ultrasonography) should be combined with ABC for the final diagnosis.
A 74-year-old man complained of a cough and left chest pain. Chest X-ray showed marked pleural effusion and a large mass in the left lower lobe, and air bronchograms within the mass were observed by tomography and comput...A 74-year-old man complained of a cough and left chest pain. Chest X-ray showed marked pleural effusion and a large mass in the left lower lobe, and air bronchograms within the mass were observed by tomography and computed tomography (CT). About 3 months later, the patient died of left atelectasis and pneumonia. Autopsy revealed a localized tumor in the lower lobe of the left lung. Histologically, proliferation of lymphoma cells was noted. Immunoglobulin staining showed B cell-type monoclonality. No metastasis was evident except for a very small nodular area in the left renal cortex.
An autopsy case of hypertrophic obstructive cardiomyopathy (HOCM), in which the clinical features of dilated cardiomyopathy (DCM) developed after 10 years is described. Three serial left ventriculograms (LVG) and bi-vent...An autopsy case of hypertrophic obstructive cardiomyopathy (HOCM), in which the clinical features of dilated cardiomyopathy (DCM) developed after 10 years is described. Three serial left ventriculograms (LVG) and bi-ventriculograms (BVG) were performed during the 10-year follow-up. At the time of the first observation, the ECG revealed a giant negative T wave (GNT) in V5 (-1.5mV) and high voltage (SV1 + RV5 = 8.1mV). The LVG and BVG showed concentric left ventricular wall hypertrophy and a pressure gradient in the left ventricular out-flow tract. However, follow-up studies disclosed resolution of the GNT with decreased high voltage. All wall thicknesses and the ejection fraction were significantly decreased and the wall motion was reduced serially. These findings were consistent with the clinical profile of DCM. At autopsy, the heart weighed 610 g and showed dilated change of the left ventricular cavity. Significant myocardial fibrosis, bizarre myocardial hypertrophy with disorganization (BMHD) and severe narrowing of the intramural small arteries due to intimal thickening were found in the left ventricular wall, especially in the anterior and interventricular septal wall. This autopsy case suggests that some cases of HCM, with serial disappearance of the deep negative T wave and high voltage, deteriorate to the clinical profile of DCM.
Disseminated mycobacterial infection was found at autopsy in a male patient with hemophilia B and acquired immunodeficiency syndrome (AIDS). In May 1986, 23 months before death, the patient had encephalitis for one month...Disseminated mycobacterial infection was found at autopsy in a male patient with hemophilia B and acquired immunodeficiency syndrome (AIDS). In May 1986, 23 months before death, the patient had encephalitis for one month and in July he developed a fever, malaise and generalized lymphoadenopathy. Human immunosuppressive virus (HIV) was positive and the CD 4/8 ratio of lymphocyte surface markers was 0.1, but mycobacterium was not detected. In September 1986, he had severe dyspnea due to interstitial pneumonia and he was treated with high-dose methylprednisolone. He died after a 23-month course of fever, severe weight loss and terminal progressive deterioration, although he was treated with antibiotics, antifungal agents, gamma-globulin, steroid and a Azidothymidine.
We report a case of a 43-year-old woman with pseudocoarctation of the aorta manifested by a superior mediastinal mass on the chest roentgenogram. Evaluation was made using computed tomography (CT), magnetic resonance ima...We report a case of a 43-year-old woman with pseudocoarctation of the aorta manifested by a superior mediastinal mass on the chest roentgenogram. Evaluation was made using computed tomography (CT), magnetic resonance imaging (MRI) and digital subtraction angiography (DSA). No other congenital anomalies or aneurysms below the kinked area of the aorta could be found. The characteristic "kink" of the aorta and the absence of true coarctation were evident in the sagittal plane by MRI. MRI is of great value for examining disorders of the aorta.
A case of primary aldosteronism associated with renovascular hypertension is reported. The patient, a 46-year-old woman, developed hypertension to the level of 210/110 mmHg. Laboratory data included serum potassium 3.4mE...A case of primary aldosteronism associated with renovascular hypertension is reported. The patient, a 46-year-old woman, developed hypertension to the level of 210/110 mmHg. Laboratory data included serum potassium 3.4mEq/l, plasma renin activity 25ng/ml/h and plasma concentration of aldosterone 330 pg/ml. Occlusion of the left renal artery and left adrenal aldosteronoma was diagnosed by radiographic and hormone analysis findings. Left adrenalectomy and nephrectomy corrected the hypertension. The possibility of tertiary aldosteronism is discussed.
An 18-year-old woman with systemic lupus erythematosus developed neuropsychiatric disorders, including aseptic meningoencephalitis, organic brain syndrome and seizure. A series of computed axial tomography scans revealed...An 18-year-old woman with systemic lupus erythematosus developed neuropsychiatric disorders, including aseptic meningoencephalitis, organic brain syndrome and seizure. A series of computed axial tomography scans revealed the progression of marked atrophy of the right cerebral hemisphere for a period of 3 years without occlusion or stenosis of large vessels on cerebral angiography. I-123 IMP single photon emission computed tomography disclosed a markedly decreased uptake of I-123 IMP in the right cerebral hemisphere, and also in the left cerebellar hemisphere (crossed cerebellar diaschisis), which disappeared within 2 years.
Danazol was administered to two patients with paroxysmal nocturnal hemoglobinuria (PNH) with a dramatic effect on the hematological findings. The patients, 31- and 41-year-old females, were initially diagnosed as having...Danazol was administered to two patients with paroxysmal nocturnal hemoglobinuria (PNH) with a dramatic effect on the hematological findings. The patients, 31- and 41-year-old females, were initially diagnosed as having aplastic anemia, and were initially treated with anabolic steroid and immunosuppressive therapy, respectively. Sugar water and Ham tests turned positive at the start of danazol therapy in the former patient and after two months in the latter patient. This drug produced a dramatic improvement in the hemoglobin level and the platelet count and showed few side effects in the patients. A possible mechanism of action of danazol for PNH is briefly discussed.
A 32-year-old female was suffering from intractable lupus nephritis (LN). Prednisolone at 30 mg per day had been prescribed for two months because of massive proteinuria and edema. After that, anasarca and orthopnea were...A 32-year-old female was suffering from intractable lupus nephritis (LN). Prednisolone at 30 mg per day had been prescribed for two months because of massive proteinuria and edema. After that, anasarca and orthopnea were induced, and hypoproteinemia, hypercholesteremia, and anti-cardiolipin antibody were observed. Prednisolone at a dose of 60 mg per day was necessary. However, there was no improvement of the nephrotic state and renal pathological changes shown as diffuse proliferative lupus nephritis were followed by moderate crescent formation as determined by serial needle biopsy. Cyclophosphamide (CYP) pulse therapy was started and marked improvement of LN was obtained clinically and serologically without significant adverse effects.