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Central nervous system leukaemia in children
72
THE ROYAL COLLEGE OF PATHOLOGISTS OF AUSTRALIA
In spite of this concentrated and costly effort, the mortality from cervical cancer has not apparently been influenced to any extent, either in Tasmania or in the whole of Australia. These findings are in keeping with recent reports from New Zealand and British Columbia. Suggested modifications to the mass screening programme were discussed and recommendations made : a. If the cervix is abnormal, a smear should be taken no matter what the patient's age. b. If the cervix appears normal, and the woman is aged between 25 and 65 yr., a smear need be taken only every 3rd or even 5th year. A REVIEW OF ACUTE LEUKAEMIA CASES AT ROYAL PRINCE ALFRED HOSPITAL, 1965-1970
WILKINSON, T., KRONENBERG, H. & RICKARD, K. A. Hospital, Sydney, New South Wales
Royal Prince Alfred
During the years 1965-1970 (inclusive) 159 patients were diagnosed as acute leukaemia. After excluding children and atypical cases, 135 adults with acute leukaemia remained and these were reviewed, being analysed according to age and sex distribution, leukaemic classification, survival after diagnosis, remission rate and its relation to forms of therapy. An initial remission was obtained in 27'!,, and when the patients were divided into lymphoblastic and non-lymphoblastic categories the initial remission rates were 79"; and 21 (,It, respectively. T h e median survival for the series was 2 mth. and the duration of life after diagnosis was 10 wk. or less in 50°,, of patients. Arising from experience in the management of these cases, the authors discussed decisions to withdraw anti-leukaemic agents, factors associated with remission rates, the significance of marrow lymphocytosis, long survivors and the quality of life in these patients.
MENINGEAL INVOLVEMENT IN ACUTE LEUKAEMIA: A REVIEW OF 4 ADULTS AND 2 ADOLESCENT CASES
LYTTON,D. G., KRONENBERG, H., RICKARD, K . A. & WILKINSON T. Royal Prince Alfred Hospital, Sydney, New South Wales With the advent of new drug regimens in the treatment of patients with acute leukaemia and the consequently increased length of survival, it is to be expected there will be an increase in the incidence of meningeal leukaemia. T h e authors reviewed in some detail the features of meningeal leukaemia. Of the 134 cases of acute leukaemia in adults diagnosed between the years 1965-1970, there were 6 patients exhibiting meningeal involvement during some stage of their illness. Usually the principal presenting symptom was headache and other manifestations included vomiting, tinnitus, nominal aphasia and hyperacusis. T h e onset of symptoms bore no constant relationship to the state of haematological remission or relapse confirming the findings of other investigators. The CSF findings were considered and the importance of correct diagnosis was stressed, as these troublesome symptoms are invariably relieved by the administration of intrathecal methotrexate. It was found that meningeal involvement is not an indication of the terminal phase of the illness.
CENTRAL NERVOUS SYSTEM LEUKAEMIA I N CHILDREN
O'GORMAN HUGHES, D. W., CLUGSTON, G., CHOWE. & LAMPo TANG, P. R. School of Paediatrics, University of New South Wales and Division of Paediatric Haematology, Prince of Wales Hospital, Sydney, New South Wales Infiltration of the nervous system is the most common extramedullary manifestation of acute leukaemia in childhood. Of 28 children with acute lymphoblastic leukaemia (ALL) diagnosed between 1964 and 1969 and followed regularly thereafter, 21 (75",,) developed neurological leukaemia. T h e first episodes occurred between 20 days and 274 mth. from diagnosis, but 64",, of all cases of leukaemia and 86", of all patients destined to develop neurological leukaemia had evidence of infiltration by 15 mth. from
ABSTRACTS OF ANNUAL SCIENTIFIC MEETING
1971
73
diagnosis. T h e common features of the initial episode were headaches (lo), vomiting (13), drowsiness (9), mood changes (lo), cranial nerve palsies (8), spinal or peripheral nerve involvement ( l ) , papilloedema (14), raised CSF pressure (10 17), CSF pleocytosis (17 20), elevated CSF protein above 40 mg. 100 ml. (8 17), and CSF glucose levels below 35 m g . J l 0 0 ml. (5116). T h e initial complication occurred during haematological remission in 11, during frank relapse in 8, during partial control in 1, and during the remission induction phase in one patient. The 21 patients suffered from neurological leukaemia on 82 occasions, the greatest number being 13 episodes in one child. On 74 occasions there was evidence of brain and or meningeal (CNS) involvement, on 4 occasions spinal or peripheral nerve infiltration, and in 4 patients a combination was present. Six of the children had a prolonged survival of from 3-5yr. 11 mth., and the longest survivor remains in continuous haematological remission. The six children who did not develop neurological leukaemia survived from 18 to 86 mth., 3 being still alive at 34, 63 and 68 mth. from diagnosis. Of 18 patients with ALL diagnosed between January 1970 and June 1971, 4 have already developed CNS leukaemia at intervals of 4.: to 12 mth. from diagnosis. As only 3 have been followed for more than 15 mth., the incidence of neurological leukaemia is likely to increase. In view of the extremely high incidence of neurological infiltration in acute lymphoblastic leukaemia, prophylactic treatment with intrathecal methotrexate and radiotherapy soon after diagnosis appears warranted. PROGNOSTIC FACTORS IN CHRONIC LYMPHATIC LEUKAEMIA
HURDLE, A. D. F. & EPSTEIN, I . S. Repatriation General Hospital, Heidelbrrx, and Prince Henry’s Hospital, Melbourm, Victoria It is well recognized that the morphology of the peripheral blood lymphocytes in chronic lymphatic leukaemia (CLL) may show considerable variation. Many cases have a proportion of cells with indented nuclei and or nucleoli. T h e records and blood films of 69 cases of CLL at presentation were reviewed to see if a relationship could be demonstrated between survival and the maturity of lymphocytes in the blood. At the same time cases of lymphosarcoma were reviewed in an attempt to define the common ground between this disease and CLL. Only two aspects of lymphocyte morphology were considered, the presence of nucleoli and deep nuclear clefts. T h e haemoglobin, white cell count, platelet count, ESR, the presence of lymphadenopathy and splenomegaly were recorded and the patients’ survival in months from diagnosis. Results were grouped according to lymphocyte morphology as shown: Mean survival (mth.) Nucleoli alone 13 cases) Nucleoli cletts :7 cases)
26.5
deep
-
Nucleoli (20 cases)
23.9
19.3
--
Deep clefts alone (6 cases)
66 2
Neither nucleoli nor deep clefts 43 cases)
41.3
No nucleoli (49 cases)
44.4
THE ROYAL COLLEGE OF PATHOLOGISTS OF AUSTRALIA
In spite of this concentrated and costly effort, the mortality from cervical cancer has not apparently been influenced to any extent, either in Tasmania or in the whole of Australia. These findings are in keeping with recent reports from New Zealand and British Columbia. Suggested modifications to the mass screening programme were discussed and recommendations made : a. If the cervix is abnormal, a smear should be taken no matter what the patient's age. b. If the cervix appears normal, and the woman is aged between 25 and 65 yr., a smear need be taken only every 3rd or even 5th year. A REVIEW OF ACUTE LEUKAEMIA CASES AT ROYAL PRINCE ALFRED HOSPITAL, 1965-1970
WILKINSON, T., KRONENBERG, H. & RICKARD, K. A. Hospital, Sydney, New South Wales
Royal Prince Alfred
During the years 1965-1970 (inclusive) 159 patients were diagnosed as acute leukaemia. After excluding children and atypical cases, 135 adults with acute leukaemia remained and these were reviewed, being analysed according to age and sex distribution, leukaemic classification, survival after diagnosis, remission rate and its relation to forms of therapy. An initial remission was obtained in 27'!,, and when the patients were divided into lymphoblastic and non-lymphoblastic categories the initial remission rates were 79"; and 21 (,It, respectively. T h e median survival for the series was 2 mth. and the duration of life after diagnosis was 10 wk. or less in 50°,, of patients. Arising from experience in the management of these cases, the authors discussed decisions to withdraw anti-leukaemic agents, factors associated with remission rates, the significance of marrow lymphocytosis, long survivors and the quality of life in these patients.
MENINGEAL INVOLVEMENT IN ACUTE LEUKAEMIA: A REVIEW OF 4 ADULTS AND 2 ADOLESCENT CASES
LYTTON,D. G., KRONENBERG, H., RICKARD, K . A. & WILKINSON T. Royal Prince Alfred Hospital, Sydney, New South Wales With the advent of new drug regimens in the treatment of patients with acute leukaemia and the consequently increased length of survival, it is to be expected there will be an increase in the incidence of meningeal leukaemia. T h e authors reviewed in some detail the features of meningeal leukaemia. Of the 134 cases of acute leukaemia in adults diagnosed between the years 1965-1970, there were 6 patients exhibiting meningeal involvement during some stage of their illness. Usually the principal presenting symptom was headache and other manifestations included vomiting, tinnitus, nominal aphasia and hyperacusis. T h e onset of symptoms bore no constant relationship to the state of haematological remission or relapse confirming the findings of other investigators. The CSF findings were considered and the importance of correct diagnosis was stressed, as these troublesome symptoms are invariably relieved by the administration of intrathecal methotrexate. It was found that meningeal involvement is not an indication of the terminal phase of the illness.
CENTRAL NERVOUS SYSTEM LEUKAEMIA I N CHILDREN
O'GORMAN HUGHES, D. W., CLUGSTON, G., CHOWE. & LAMPo TANG, P. R. School of Paediatrics, University of New South Wales and Division of Paediatric Haematology, Prince of Wales Hospital, Sydney, New South Wales Infiltration of the nervous system is the most common extramedullary manifestation of acute leukaemia in childhood. Of 28 children with acute lymphoblastic leukaemia (ALL) diagnosed between 1964 and 1969 and followed regularly thereafter, 21 (75",,) developed neurological leukaemia. T h e first episodes occurred between 20 days and 274 mth. from diagnosis, but 64",, of all cases of leukaemia and 86", of all patients destined to develop neurological leukaemia had evidence of infiltration by 15 mth. from
ABSTRACTS OF ANNUAL SCIENTIFIC MEETING
1971
73
diagnosis. T h e common features of the initial episode were headaches (lo), vomiting (13), drowsiness (9), mood changes (lo), cranial nerve palsies (8), spinal or peripheral nerve involvement ( l ) , papilloedema (14), raised CSF pressure (10 17), CSF pleocytosis (17 20), elevated CSF protein above 40 mg. 100 ml. (8 17), and CSF glucose levels below 35 m g . J l 0 0 ml. (5116). T h e initial complication occurred during haematological remission in 11, during frank relapse in 8, during partial control in 1, and during the remission induction phase in one patient. The 21 patients suffered from neurological leukaemia on 82 occasions, the greatest number being 13 episodes in one child. On 74 occasions there was evidence of brain and or meningeal (CNS) involvement, on 4 occasions spinal or peripheral nerve infiltration, and in 4 patients a combination was present. Six of the children had a prolonged survival of from 3-5yr. 11 mth., and the longest survivor remains in continuous haematological remission. The six children who did not develop neurological leukaemia survived from 18 to 86 mth., 3 being still alive at 34, 63 and 68 mth. from diagnosis. Of 18 patients with ALL diagnosed between January 1970 and June 1971, 4 have already developed CNS leukaemia at intervals of 4.: to 12 mth. from diagnosis. As only 3 have been followed for more than 15 mth., the incidence of neurological leukaemia is likely to increase. In view of the extremely high incidence of neurological infiltration in acute lymphoblastic leukaemia, prophylactic treatment with intrathecal methotrexate and radiotherapy soon after diagnosis appears warranted. PROGNOSTIC FACTORS IN CHRONIC LYMPHATIC LEUKAEMIA
HURDLE, A. D. F. & EPSTEIN, I . S. Repatriation General Hospital, Heidelbrrx, and Prince Henry’s Hospital, Melbourm, Victoria It is well recognized that the morphology of the peripheral blood lymphocytes in chronic lymphatic leukaemia (CLL) may show considerable variation. Many cases have a proportion of cells with indented nuclei and or nucleoli. T h e records and blood films of 69 cases of CLL at presentation were reviewed to see if a relationship could be demonstrated between survival and the maturity of lymphocytes in the blood. At the same time cases of lymphosarcoma were reviewed in an attempt to define the common ground between this disease and CLL. Only two aspects of lymphocyte morphology were considered, the presence of nucleoli and deep nuclear clefts. T h e haemoglobin, white cell count, platelet count, ESR, the presence of lymphadenopathy and splenomegaly were recorded and the patients’ survival in months from diagnosis. Results were grouped according to lymphocyte morphology as shown: Mean survival (mth.) Nucleoli alone 13 cases) Nucleoli cletts :7 cases)
26.5
deep
-
Nucleoli (20 cases)
23.9
19.3
--
Deep clefts alone (6 cases)
66 2
Neither nucleoli nor deep clefts 43 cases)
41.3
No nucleoli (49 cases)
44.4