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Non-Specific Immunological Function in Acute Myeloblastic Leukaemia
ABSTRACTS OF A N N U A L M E E T I N G
1973
95
NON-SPECIFIC IMMUNOLOGICAL FUNCTION I N ACUTE M Y EL0 B LASTlC LEU KAEM IA
CAUCHI, M. N., FOY, A. & WHITESIDE,M . Department of Pathology, Monush University Medical School &JAlfred Hospital, Melbourne Patients with acute myeloblastic leukaemia were investigated in the untreated stage of the disease and in remission using several tests of immunological function. IgM levels were found to be raised in 7/29 patients during the acute stage. There were only minimal changes in IgG, IgA and C' levels, or in the incidence of autoantibodies to normal tissue components. Reaction to standard skin tests was considerably impaired: all controls tested responded to 2 or more of these tests, whereas only 2/10 leukaemic patients responded in this fashion. In general the response in leukaemic patients was much weaker than the corresponding controls. PHA stimulation of lymphocytes in patients during remission showed considerable variation from near normal levels to gross impairment. I n general, levels below 40", of normal were asscciated with a short remission period, indicating that the FHA stimulation test may be a useful additional criterion of the likelihood of relapse. DEATH FROM FULM INANT AUTO-IMMU N E TH ROM BOCYTOPENIC PU R PU RA
SAGE,R. E.
The Queen Elizabeth Hospital, Woodville, South Australia
Acute auto-immune thrombocytopenic purpura is a self-limited disorder responding well in most adult patients to treatment with immune suppressive agents alone or with splenectomy. T h e present report illustrates the extreme rapidity with which the disorder can progress with resistance to all forms of therapy to death. A 22-yr-old female presented with a one-day history of spontaneous bruising and minor haemoptysis. There was no preceding history of bleeding tendency, respiratory tract infection or drug ingestion. Examination revealed only bruises over the trunk and limbs. T h e platelet count was 37,000 'mm3 and bone marrow examination was compatible with auto-immune thrombocytopenic purpura. During the next 60 hr the platelet count fell to zero despite massive doses of steroids and multiple platelet transfusions. Purpura appeared and there was bleeding into the subarachnoid space. Epistaxes, haematuria, menorrhagia and bright blood per rectum were followed by pulmonary haemorrhage. Emergency splenectomy was performed. T h e patient survived for 16 h r post-splenectomy, but succumbed to the pre-operative pulmonary haemorrhage. There was no further bleeding after the operation. At the time of death the platelet count had risen to 16,000,/mm3. This case illustrates the extremely rapid progress that may occur and supports the premise that early splenectomy must be considered in these cases. Failure of response to massive platelet transfusion may indicate need for early splenectomy in the presence of clinical deterioration. ABNORMAL CYANIDE ASCORBATE TEST IN URAEMIA
CRONAN, K. ROZENBERG, M . & JEREMY, D. Little Ba-y, New Scuth Wales
Prince Henry Hospital,
T h e anaemia of renal failure is associated with increased activity of the hexose monophosphate shunt in the erythrocytes and with increased in virro formation of sulphaemoglobin during incubation of the red cells with cyanide and ascorbate. T h e presence of a contaminant in the water used for haemodialysis in renal failure in one unit has been reported to worsen the anaemia and enhance the formation of sulphaemoglobin in the cyanide ascorbate test. This test was performed on the red cells in patients with renal failure to determine the extent of the defect and to observe the effect, if any, of haemodialysis on anaemia. T h e result of the cyanide ascorbate test was expressed as the ratio of sulphaemoglobin to total haemoglobin after 2-hr incubation under stress. T h e normal range was 0.12 to 0.44 with a mean of 0.28 (SD 0.08). Fifty-four patients with renal failure were studied. In 39, the mean ratio was 0.44 (0.24-0.91, S D 0.15). There was no apparent correlation between the abnormal test and the patient's haemoglobin, blood urea of serum creatinine. Fifteen patients established on recurrent haemodialysis for severe renal failure had a pre-dialysis mean ratio of 0.50 (0.29-1.00, S D 0.21) and a post-dialysis mean ratio of 0.57 (0.25-1.00, SD 0.26). There was no evidence of deterioration in either the cyanide ascorbate test ratio or in the level of haemoglobin in these patients as a result of a dialysis.
1973
95
NON-SPECIFIC IMMUNOLOGICAL FUNCTION I N ACUTE M Y EL0 B LASTlC LEU KAEM IA
CAUCHI, M. N., FOY, A. & WHITESIDE,M . Department of Pathology, Monush University Medical School &JAlfred Hospital, Melbourne Patients with acute myeloblastic leukaemia were investigated in the untreated stage of the disease and in remission using several tests of immunological function. IgM levels were found to be raised in 7/29 patients during the acute stage. There were only minimal changes in IgG, IgA and C' levels, or in the incidence of autoantibodies to normal tissue components. Reaction to standard skin tests was considerably impaired: all controls tested responded to 2 or more of these tests, whereas only 2/10 leukaemic patients responded in this fashion. In general the response in leukaemic patients was much weaker than the corresponding controls. PHA stimulation of lymphocytes in patients during remission showed considerable variation from near normal levels to gross impairment. I n general, levels below 40", of normal were asscciated with a short remission period, indicating that the FHA stimulation test may be a useful additional criterion of the likelihood of relapse. DEATH FROM FULM INANT AUTO-IMMU N E TH ROM BOCYTOPENIC PU R PU RA
SAGE,R. E.
The Queen Elizabeth Hospital, Woodville, South Australia
Acute auto-immune thrombocytopenic purpura is a self-limited disorder responding well in most adult patients to treatment with immune suppressive agents alone or with splenectomy. T h e present report illustrates the extreme rapidity with which the disorder can progress with resistance to all forms of therapy to death. A 22-yr-old female presented with a one-day history of spontaneous bruising and minor haemoptysis. There was no preceding history of bleeding tendency, respiratory tract infection or drug ingestion. Examination revealed only bruises over the trunk and limbs. T h e platelet count was 37,000 'mm3 and bone marrow examination was compatible with auto-immune thrombocytopenic purpura. During the next 60 hr the platelet count fell to zero despite massive doses of steroids and multiple platelet transfusions. Purpura appeared and there was bleeding into the subarachnoid space. Epistaxes, haematuria, menorrhagia and bright blood per rectum were followed by pulmonary haemorrhage. Emergency splenectomy was performed. T h e patient survived for 16 h r post-splenectomy, but succumbed to the pre-operative pulmonary haemorrhage. There was no further bleeding after the operation. At the time of death the platelet count had risen to 16,000,/mm3. This case illustrates the extremely rapid progress that may occur and supports the premise that early splenectomy must be considered in these cases. Failure of response to massive platelet transfusion may indicate need for early splenectomy in the presence of clinical deterioration. ABNORMAL CYANIDE ASCORBATE TEST IN URAEMIA
CRONAN, K. ROZENBERG, M . & JEREMY, D. Little Ba-y, New Scuth Wales
Prince Henry Hospital,
T h e anaemia of renal failure is associated with increased activity of the hexose monophosphate shunt in the erythrocytes and with increased in virro formation of sulphaemoglobin during incubation of the red cells with cyanide and ascorbate. T h e presence of a contaminant in the water used for haemodialysis in renal failure in one unit has been reported to worsen the anaemia and enhance the formation of sulphaemoglobin in the cyanide ascorbate test. This test was performed on the red cells in patients with renal failure to determine the extent of the defect and to observe the effect, if any, of haemodialysis on anaemia. T h e result of the cyanide ascorbate test was expressed as the ratio of sulphaemoglobin to total haemoglobin after 2-hr incubation under stress. T h e normal range was 0.12 to 0.44 with a mean of 0.28 (SD 0.08). Fifty-four patients with renal failure were studied. In 39, the mean ratio was 0.44 (0.24-0.91, S D 0.15). There was no apparent correlation between the abnormal test and the patient's haemoglobin, blood urea of serum creatinine. Fifteen patients established on recurrent haemodialysis for severe renal failure had a pre-dialysis mean ratio of 0.50 (0.29-1.00, S D 0.21) and a post-dialysis mean ratio of 0.57 (0.25-1.00, SD 0.26). There was no evidence of deterioration in either the cyanide ascorbate test ratio or in the level of haemoglobin in these patients as a result of a dialysis.