The characteristics of diastolic pressure-volume relationships in primary myocardial disease.

Utilizing the pressure-volume relationship, the indices of left ventricular diastolic compliance, SV/EDP-DP, dV/dP, and dV/VdP, ventricular stiffness index, I/P(dV/dP), and myocardial stiffness index, I/S(dD/dS) were evaluated of seven patients with normal left ventricle, eleven patients with hypertrophic and seven with congestive type of primary myocardial disease. Myocardial stiffness index was obtained from the equation of S = aekD, where S is a wall stress, a; a constant, D; a length of short axis, K; a stiffness constant. The mean values and standard errors of the means of SV/EDP DP, dV/dP, dV/VdP, I/P(dV/dP), and I/S(dD/dS) for normal group are 16.7 +/- 8.7 (ml/mmHg), 16.03 +/- 6.98 (ml/mmHg), 0.1259 +/- 0.0447 (mmHg-1), 0.0059 +/- 0.0032 (ml-1) and 0.603 +/- 0.153 (cm-1), for hypertrophic type are 10.5 +/- 5.5 (ml/mmHg), 7.23 +/- 7.42 (ml/mmHg), 0.0599 +/- 0.0736 (mmHg-1), 0.0174 +/- 0.0097 (ml-1) and 1.3555 +/- 0.3395 (cm-1), and for congestive type are 17.5 +/- 3.4 (ml/mmHg), 3.91 +/- 2.13 (ml/mmHg), 0.0250 +/- 0.0239 (mmHg-1), 0.0308 +/- 0.0126 (ml-1), and 3.287 +/- 1.272 (cm-1). There were highly significant differences between normal and primary myocardial disease in each indices except SV/EDP-DP. I/P(dV/dP), and I/S(dD/dS) had highly significant differences between hypertrophic and congestive type, the latter is stiffer than the former. The compliance index was not sensitive enough to separate two types of primary myocardial disease.     

Multiple lymphomatous polyposis of the colon originating from T-cells: a case report

Multiple lymphomatous polyposis is an unusual form of non-Hodgkin's lymphoma characterised by myriad polyps throughout the alimentary tract. Most multiple lymphomatous polyposis cases are derived from B-cell, and there has been little information on multiple lymphomatous polyposis of T-cell origin. A 67-year-old Japanese man presented with lower abdominal pain and diarrhoea of 4-week duration. Colonoscopy revealed numerous small umbilicated polyps and several raised erosions in the colorectum. Biopsy specimens showed diffuse proliferation of lymphoma cells negative for B-cell markers but positive for T-cell markers. Polymerase chain reaction using extracted chromosomal deoxyribonucleic acid from paraffin-embedded samples identified T-cell receptor gamma and delta gene recombination. The patient was treated with combined chemotherapy, leading to complete resolution of the lesions.     

Lymphoid crisis with T-cell phenotypes in a patient with Philadelphia chromosome negative chronic myeloid leukaemia

A case of Philadelphia (Ph1) chromosome negative chronic myeloid leukaemia (CML) developed lymphoid crisis. Immunological marker studies disclosed that the lymphoid cells were sheep erythrocyte-rosetting-, Leu-1+, Leu-5+, OKT-4+, OKT-8+, common ALL antigen-, HLA-DR-, cytoplasmic and surface immunoglobulin-, MAS 036C(antithymocyte)+ (after in vitro culture) and terminal deoxynucleotidyl transferase-, indicating T-cell phenotypes, probably of common thymocytes. Cytochemical staining also demonstrated immature T-cell characters: dot-positivity for acid phosphatase and beta-glucuronidase, and negative for acid alpha-naphthyl acetate esterase. All bone marrow metaphases exhibited normal karyotypes. Our observation suggests that the neoplastic features of a common stem cell for myeloid and lymphoid cell lines are very similar in Ph1 positive and negative CMLs, and that the stem cell can differentiate towards T-lineage.     

Clinicopathological features of acute megakaryoblastic leukaemia: Relationship between fibrosis and platelet‐derived growth factor

Acute megakaryoblastic leukaemia (AMGL) is an uncommon disease with poor prognosis. Histopathologically, AMGL cases show variable degree of fibrosis and the presence of uniform blasts or mature dysplastic megakaryocytes. Here we examined 18 cases of AMGL, including idiopathic (n = 9) and secondary (n = 9) cases. Fourteen cases were males and four were females, ranging in age from 14 to 87 years (median, 58). All cases had anaemia, but leukocyte and platelet counts varied. Blast cells were detected in the peripheral blood of 14 cases. Fourteen of 16 cases showed chromosomal abnormalities. The median survival was 6 months (range, 1–48 months). Survival rates did not correlate with the severity of fibrosis, proportion of blast cells and cause of AMGL. Nine of the 11 cases examined immunohistochemically were positive for platelet‐derived growth factor (PDGF)(‐BB), especially megakaryoblasts and a few fibroblasts. The PDGF‐positive cases showed various degrees of fibrosis, while the negative cases showed no evidence of fibrosis. Our results confirmed the poor prognosis of patients with AMGL, irrespective of the degrees of fibrosis, and demonstrated that PDGF could play an important role in the pathogenesis of marrow fibrosis.     

Empiric therapy with aztreonam for febrile neutropenic patients with hematological malignancies

Combination of aztreonam/amikacin/ticarcillin (AAT) and latamoxef/amikacin/ticarcillin (LAT) were compared in a prospective randomized trial of empiric therapy for febrile neutropenic patients with hematological malignancies. Low dose amphotericin B was also added to each regimen from the beginning. Of 45 evaluable episodes, 23 were treated with AAT and 22 with LAT. The response rates were 61 percent for AAT and 50 percent for LAT, statistically not significant. There was one infection-related death among patients assigned to AAT therapy and also one among those assigned to LAT therapy. Dose escalation of amphotericin B seemed to be effective for those patients who did not improve with initial therapy.     

A case of unusual longevity of tetralogy of Fallot confirmed by cardiac catheterization.

We describe a 61-year-old woman with tetralogy of Fallot and dextrocardia with complete situs inversus. The functional status of this patient was New York Heart Association (NYHA) class II and the systemic blood pressure was 100/54 mmHg. The hematocrit was 54.4% and the arterial partial pressure of oxygen was 53 mmHg. On cardiac catheterization, both pulmonary valvular and infundibular stenoses were of equal severity and the aorta and main pulmonary artery were of equal size. We think that this patient has survived to this unusual age for tetralogy of Fallot because of unusually low systemic pressure and a proper balance between the ventricular septal defect and the pulmonary stenosis.