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Ovalocytosis with hypersplenism: Report of two cases and observations on the pathogenesis of the hypersplenism
534
American Federation for Clinical Research
volume, no significant physiologic differences were attributable to aureomycin in the doses employed nor were findings at autopsy altered. OVALOCYTOSISWITH HYPERSPLENISM:REPORT OF Two CASES AND OBSERVATIONSON THE PATHOGENESISOF THE HYPERSPLENISM.H. E. Wilson, M.D. and M. J. Long, M.D. ” (From the Northwestern University, Chicago, Ill.) Two of five siblings were found to have ovalocytosis. The first, a sixty-three year old female, entered in an attack of acute cholecystitis with a history of long-standing anemia. Significant findings on admission were fever, icterus, moderate hepatomegaly and splenomegaly, ovalocytosis with anemia and a relative leucopenia. Bone marrow was hyperplastic in all elements. After subsidence of fever and jaundice an adrenalin test produced marked splenic contraction concurrent with significant increases in all of the peripheral blood cell elements. Erythrocyte osmotic fragility was normal, Coombs’ test negative. A gallbladder full of pigment stones and a 300 gm. spleen showing fibrosis and hemosiderosis were removed. Postoperatively, all of the peripheral blood cells rose to high normal levels. Coincident with the recovery from the pancytopenia was the appearance of increasing numbers of ovalocytic and spherocytic microcytes. Erythrocyte mechanical fragility was greatly increased, the osmotic fragility moderately increased. This patient’s fifty-eight year old brother shows splenomegaly, anemia and a uniform ovalocytosis similar to that observed in his sister preoperatively. Mechanical fragility is slightly but significantly increased. Comparison of these two cases and the effects of splenectomy demonstrate the importance of red cell shape and size on mechanical fragility and on splenic sequestration. These observations support Bjorkman’s experimental work on splenic filtration and circulation. HUMAN STUDIESWITH ACTX, A HIGH-POTENCY CORTICOTROPIN SUB-TYPE CHARACTERIZED BY A HIGH RATIO OF INTRAMUSCULAR TO INTRAVENOUS ACTIVITY. W. Q. Wolfson, M.D.,
W. D. Robinson, M.D., J. R. Quinn,?M.D. * and I. F. Duf, M.D. (From the Rackham Arthritis Research Unit, University of Michigan School, Ann Arbor, Mich.)
Medical
ACTX is a high-potency corticotropin prepared by chromatographic fractionation. When given intramuscularly each U.S.P. unit of
ACTX appears metabolically and therapeutically equivalent to approximately 3 U.S.P. units of intramuscular U.S.P. corticotropin (ACTH). ACTX in Light Gelatin (ACTX-LG) q.6.h. i.m.: Four patients receiving 2.5 to 5.0 units of ACTX-LG q.6.h. consistently showed circulating eosinophils below 10 per cent of pre-treatment levels. Under assay conditions in one suhject 2.5 units of ACTX-LG q.6.h. produced metabolic effects greater than those of q.6.h. administration of 12.5 units of two aqueous ACTH preparations. ACTX in Heavy Gelatin (ACTX-HG) once daily: in initiating and maintaining remissions in comparable patients, effectiveness of ACTX-HG averaged better than three times that of ACTH in heavy gelatin. Twenty-four hours after receiving 35 to 40 unit doses of ACTX-HG medium eosinophil count in twelve subjects was 0 per cu. mm., a result not duplicated by 100 units of long-acting ACTH preparations. Under assay conditions in one subject ACTX-HG preparations from two different manufacturers were closely similar. Distinctive labelling and dosage instructions for ACTX appear advisable. Since intermittent intramuscular ACTX-LG approaches the effectiveness of intravenous ACTH infusions, ACTX probably undergoes less extravascular inactivation than ACTH. AORTIC STENOSIS MASKING AS CHRONIC COR PIJLMONALE. J. Zatuchni, M.D. ” and L. A. Solo$, M.D. (From the Dept. of Medicine, Temple University Hospital and School of Medicine, Philadelphia, Pa.) The postmortem discovery of aortic stenosis in an individual free of cardiac murmurs who had presented for years the clinical syndrome of chronic car pulmonale prompted an investigation of the circulatory hemodynamics of aortic stenosis. Nine consecutive subjects were studied. The right heart circulation time was prolonged in all. The venous pressure may be elevated. The aortic murmur may or may not be characteristic and with failure may disappear entirely. Only with failure is the left heart time prolonged and even then it is disproportionately slow compared to the right. With increasing venous hypertension an absent or insignificant aortic murmur, an emphysematous chest SO frequent in the elderly and pulmonary symptoms, the picture of chronic car pulmonale is AMERICAN
JOURNAL
OF
MEDICINE
American Federation for Clinical Research
volume, no significant physiologic differences were attributable to aureomycin in the doses employed nor were findings at autopsy altered. OVALOCYTOSISWITH HYPERSPLENISM:REPORT OF Two CASES AND OBSERVATIONSON THE PATHOGENESISOF THE HYPERSPLENISM.H. E. Wilson, M.D. and M. J. Long, M.D. ” (From the Northwestern University, Chicago, Ill.) Two of five siblings were found to have ovalocytosis. The first, a sixty-three year old female, entered in an attack of acute cholecystitis with a history of long-standing anemia. Significant findings on admission were fever, icterus, moderate hepatomegaly and splenomegaly, ovalocytosis with anemia and a relative leucopenia. Bone marrow was hyperplastic in all elements. After subsidence of fever and jaundice an adrenalin test produced marked splenic contraction concurrent with significant increases in all of the peripheral blood cell elements. Erythrocyte osmotic fragility was normal, Coombs’ test negative. A gallbladder full of pigment stones and a 300 gm. spleen showing fibrosis and hemosiderosis were removed. Postoperatively, all of the peripheral blood cells rose to high normal levels. Coincident with the recovery from the pancytopenia was the appearance of increasing numbers of ovalocytic and spherocytic microcytes. Erythrocyte mechanical fragility was greatly increased, the osmotic fragility moderately increased. This patient’s fifty-eight year old brother shows splenomegaly, anemia and a uniform ovalocytosis similar to that observed in his sister preoperatively. Mechanical fragility is slightly but significantly increased. Comparison of these two cases and the effects of splenectomy demonstrate the importance of red cell shape and size on mechanical fragility and on splenic sequestration. These observations support Bjorkman’s experimental work on splenic filtration and circulation. HUMAN STUDIESWITH ACTX, A HIGH-POTENCY CORTICOTROPIN SUB-TYPE CHARACTERIZED BY A HIGH RATIO OF INTRAMUSCULAR TO INTRAVENOUS ACTIVITY. W. Q. Wolfson, M.D.,
W. D. Robinson, M.D., J. R. Quinn,?M.D. * and I. F. Duf, M.D. (From the Rackham Arthritis Research Unit, University of Michigan School, Ann Arbor, Mich.)
Medical
ACTX is a high-potency corticotropin prepared by chromatographic fractionation. When given intramuscularly each U.S.P. unit of
ACTX appears metabolically and therapeutically equivalent to approximately 3 U.S.P. units of intramuscular U.S.P. corticotropin (ACTH). ACTX in Light Gelatin (ACTX-LG) q.6.h. i.m.: Four patients receiving 2.5 to 5.0 units of ACTX-LG q.6.h. consistently showed circulating eosinophils below 10 per cent of pre-treatment levels. Under assay conditions in one suhject 2.5 units of ACTX-LG q.6.h. produced metabolic effects greater than those of q.6.h. administration of 12.5 units of two aqueous ACTH preparations. ACTX in Heavy Gelatin (ACTX-HG) once daily: in initiating and maintaining remissions in comparable patients, effectiveness of ACTX-HG averaged better than three times that of ACTH in heavy gelatin. Twenty-four hours after receiving 35 to 40 unit doses of ACTX-HG medium eosinophil count in twelve subjects was 0 per cu. mm., a result not duplicated by 100 units of long-acting ACTH preparations. Under assay conditions in one subject ACTX-HG preparations from two different manufacturers were closely similar. Distinctive labelling and dosage instructions for ACTX appear advisable. Since intermittent intramuscular ACTX-LG approaches the effectiveness of intravenous ACTH infusions, ACTX probably undergoes less extravascular inactivation than ACTH. AORTIC STENOSIS MASKING AS CHRONIC COR PIJLMONALE. J. Zatuchni, M.D. ” and L. A. Solo$, M.D. (From the Dept. of Medicine, Temple University Hospital and School of Medicine, Philadelphia, Pa.) The postmortem discovery of aortic stenosis in an individual free of cardiac murmurs who had presented for years the clinical syndrome of chronic car pulmonale prompted an investigation of the circulatory hemodynamics of aortic stenosis. Nine consecutive subjects were studied. The right heart circulation time was prolonged in all. The venous pressure may be elevated. The aortic murmur may or may not be characteristic and with failure may disappear entirely. Only with failure is the left heart time prolonged and even then it is disproportionately slow compared to the right. With increasing venous hypertension an absent or insignificant aortic murmur, an emphysematous chest SO frequent in the elderly and pulmonary symptoms, the picture of chronic car pulmonale is AMERICAN
JOURNAL
OF
MEDICINE