[IgA RIPA inhibitor in a case of acquired von Willebrand syndrome].

Acquired inhibitor of von Willebrand factor-platelet interaction occurring in a 57 year-old female has been partially characterized. She had no personal or familial bleeding tendencies, but presented a subcutaneous hematoma of recent origin. She was diagnosed as having an acquired von Willebrand syndrome because she had low levels of FVIII complex in plasma, with platelet adhesiveness to glassbeads and RIPA decreased. This inhibitor was classified as an IgA immunoglobulin, and had no activity against any component of FVIII complex. The purified IgA by the chromatographic technology interacted with normal platelets to inhibit RIPA. Following 1-deamino-8-D-arginine vasopressin (DDAVP) infusion, she had higher immediate rise in all components of FVIII complex in plasma, with no rapid decline. Plasma von Willebrand factor (vWF) multimers analyzed by 1.5% SDS-AGE technology revealed to be identical with those of normal plasma. These studies suggest that the abnormality of ristocetin-induced vWF-platelet interaction by IgA RIPA inhibitor and the reduction of all vWF multimers (like type IA von Willebrand disease) may have a relationship with the pathogenesis of bleeding diathesis in this case.

[Three cases of pernicious anemia complicated with chronic thyroiditis--the evaluations of immunological abnormalities].

We reported 3 patients with pernicious anemia associated with chronic thyroiditis and evaluated their immunological abnormalities in this paper. Two patients were females and another was male. They were all advanced in age. Levels of gammaglobulin including IgG was elevated in patients' sera. Values of complement components were within normal ranges. Although organ specific autoantibodies against intrinsic factors, parietal cells and thyroidal tissue antigens were detected, organ non-specific autoantibodies such as anti-DNA and/or anti-ENA antibodies were negative in the patients' sera. Percentage of T cells in peripheral blood remained within normal ranges. However, ratio of helper T/suppressor T cells was reduced considering patients' age. Moreover, Mantoux tests were negative in all of three patients. These results suggested that impaired cellular immunity, particularly imbalance of T cell subsets, caused by senescence, simultaneously induced pernicious anemia and chronic thyroiditis in these patients.

[Clinical evaluation of monotherapy with ceftazidime for severe infections complicating hematological disorders. Hyogo Cooperative Study Group of Infectious Diseases Complicating Hematological Disorders].

Clinical effects of the monotherapy with ceftazidime (CAZ) were evaluated in patients with severe infections associated with febrile granulocytopenia in hematological disorders in 10 institutions. CAZ (4-6g/day) was administered intravenously by drip infusion divided into 2 to 4 doses. 83% of the underlying diseases were hematological malignancies. Infections mainly consisted of documented sepsis (10%), presumed sepsis (60%). Overall efficacy rate was 65%, and that of septic patients was 83.3%. Adverse reactions were minimal, and this study revealed safety of CAZ.

[Acquired von Willebrand syndrome associated with Hashitoxicosis and pernicious anemia combined].

A new case of acquired von Willebrand syndrome (AvWS) with Hashitoxicosis and pernicious anemia combined in a 73-years-old male is reported. He was admitted because of appetite loss and general malaise. Physical examination showed severe anemia and general edema. The red-cell count was 103 X 10(4)/microliters with a MCV of 122 fl; the white-cell count was 2,900/microliters with 24.5% hypersegmented neutrophils; the platelet count was 17.2 X 10(4)/microliters. the lactate dehydrogenase was 9,513 U/ml and vitamin B12 was 87 pg/dl. An aspirated specimen of bone marrow was diagnostic of megaloblastic anemia. The thyroid hormones were decreased with the thyroid stimulating hormone increased. From the immunological findings, the thyroid-test, microsome-test, and anti-intrinsic factor were positive, but M proteinemia and Bence Jones proteinuria were absent. Histology of the thyroid gland and the gastric mucosa established the diagnosis of chronic thyroiditis and chronic atrophic gastritis. Subcutaneous hemorrhages after veni-puncture were observed on admission. He had a normal bleeding time, but the coagulation studies indicated the presence of von Willebrand disease, but as his family and past history were negative, this suggested the presence of an AvWS. The analysis of von Willebrand factor (vWF) multimeric composition had showed the lack of the larger multimers in the plasma, but it was normalized after the administration of levothyroxine sodium and hydroxocobalamin with vWF: Ag/RCo ratio paralleled. As far as we know, this is the first report of AvWS with Hashitoxicosis and pernicious anemia combined.

Hemopoietic recovery in bone marrow of lethally irradiated rats following parabiosis. I. Granulopoiesis.

Aplasia was induced in rats by total body irradiation. Three days later, the animal was conjugated by aortic anastomoses with a healthy untreated litter-mate. 6 h after parabiosis, the bone marrow of irradiated animals contained some granulocytes showing RNA synthesis. At 18 h, many myelocytes and promyelocytes were present but no myeloblast was encountered. These myeloid precursor cells showed active DNA synthesis but no mitoses, and no erythroblasts were observed at this time period. At 24 h, mitoses of myeloblasts were found. At 42--60 h, erythropoiesis was evident. Chromosome analysis and investigations of cells of irradiated parabionts conjugated with partners having labeled cells, revealed that these newly formed myeloid and erythroid cells originated from the untreated parabiont. The mechanism of triggering myelopoiesis in the aplastic bone marrow by parabiosis is discussed.