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The marks of preleukaemia
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T H E R O Y A L COLLEGE O F P A T H O L O G I S T S O F A U S T R A L I A
This enzyme was measured in 191 patients with hepatobiliary disease and also in 58 patients in whom a high serum alkaline phosphatase (AP) was due to a bony cause. 92:” of patients with liver disease had high G G T P values but the test was of little use in differentiating between different types. G G T P is elevated in the same circumstances as AP, but has no advantage over AP as a routine liver function test. Only 2.5”; of patients with bone disease had elevated values. GGTP was extremely useful in the differentiation of liver from bone disease, and its greatest use would seem to be in making such a differentiation in patients with an unexplained elevation in AP. CONGENITAL IDIOPATHIC ADRENAL HYPOPLASIA LAVERTY, C. R. A. & FORTUNE, D. W. Department of Pathology, Royal Women’s Hospital, Carlton, Victoria
This paper reported the largest series yet from a single institution of congenital idiopathic or primary adrenal hypoplasia confirmed at autopsy. In the 13 yr 1959-1971, 11 cases were documented in a series of 5,687 consecutive perinatal autopsies at the Royal Women’s Hospital, Melbourne. One of the cases was stillborn, 7 died during the first day, and the longest survival was 24 days. Seven were male, and 4 female, no sibships were included. The disease has a distinct male preponderance and sex-linked inheritance has been thought to account for the majority of the less than 50 previously reported cases. Three different histological patterns were seen in the hypoplastic adrenal cortices. In 3 cases, the glands were simply miniature with a normal ratio of foetal to adult cortex. In 7 cases, the foetal cortex was selectively reduced (as in secondary adrenal hypoplasia seen typically in anencephalus) while in one case, the cortex was composed solely of disorganized cytomegalic foetal cortical tissue. Attention was drawn to the association of adrenal hypoplasia with low maternal urinary estriol excretion in the last trimester of pregnancy, with post-maturity, difficulty in induction of labour, poor progress during labour, and to the difficulties of clinical and biochemical confirmation of adrenal hypoplasia during life. In two infants the diagnosis was suspected and corticosteroid therapy given unsuccessfully. THE MARKS OF PRELEUKAEMIA
GUNZ,F. W. Medical Research Department, Kanematsu Memorial Institute, Sydney Hospital, Sydney, New South Wales The term preleukaemia suggests a state which precedes true leukaemia and can be clearly distinguished from it. However, since there are no absolute criteria of what constitutes leukaemia, it is impossible to draw a sharp demarcation line between the disease and any pathological processes leading up to it. Preleukaemia can thus only be diagnosed retrospectively. The discovery of acquired cytogenetic changes is often believed to indicate the imminence or presence of malignancy. In this paper the question is posed if clonal abnormalities in marrow cells denote preleukaemia and forecast the later development of leukaemia. Details are presented of the case of a man with lymphosarcoma whose marrow, when first seen before treatment, was mildly hypoplastic; but without abnormal chromosomes. The lymphoma was controlled by chemo- and radiotherapy, but 7 mth after treatment was discontinued, the patient developed pancytopenia. A hypodiploid clone was now observed in the marrow, and additional hyperdiploid and polyploid lines were found 4 mth later, together with the clear features of acute granulocytic leukaemia. This case showed the concurrent development of leukaemia and of chromosome abnormalities. I t is contrasted with others where clonal changes were found in haematologically abnormal patients but leukaemia failed to develop, and with a second group in which leukaemia followed longstanding blood dyscrasias but without any observable chromosome abnormalities. It was concluded that new cytogenetic techniques are required before the precise rattionship between leukaemia and its pathological antecedents can be properly assessed. CLINICAL AND MORPHOLOGICAL FEATURES OF A CASE LEU KAEM I C R ETICU LO EN DOTH ELI0s IS
OF
IRONSIDE, P. Cancer Institute, Melbourne, Victoria
A case of leukaemic reticuloendotheliosis was described to indicate the individuality of the disease and its
T H E R O Y A L COLLEGE O F P A T H O L O G I S T S O F A U S T R A L I A
This enzyme was measured in 191 patients with hepatobiliary disease and also in 58 patients in whom a high serum alkaline phosphatase (AP) was due to a bony cause. 92:” of patients with liver disease had high G G T P values but the test was of little use in differentiating between different types. G G T P is elevated in the same circumstances as AP, but has no advantage over AP as a routine liver function test. Only 2.5”; of patients with bone disease had elevated values. GGTP was extremely useful in the differentiation of liver from bone disease, and its greatest use would seem to be in making such a differentiation in patients with an unexplained elevation in AP. CONGENITAL IDIOPATHIC ADRENAL HYPOPLASIA LAVERTY, C. R. A. & FORTUNE, D. W. Department of Pathology, Royal Women’s Hospital, Carlton, Victoria
This paper reported the largest series yet from a single institution of congenital idiopathic or primary adrenal hypoplasia confirmed at autopsy. In the 13 yr 1959-1971, 11 cases were documented in a series of 5,687 consecutive perinatal autopsies at the Royal Women’s Hospital, Melbourne. One of the cases was stillborn, 7 died during the first day, and the longest survival was 24 days. Seven were male, and 4 female, no sibships were included. The disease has a distinct male preponderance and sex-linked inheritance has been thought to account for the majority of the less than 50 previously reported cases. Three different histological patterns were seen in the hypoplastic adrenal cortices. In 3 cases, the glands were simply miniature with a normal ratio of foetal to adult cortex. In 7 cases, the foetal cortex was selectively reduced (as in secondary adrenal hypoplasia seen typically in anencephalus) while in one case, the cortex was composed solely of disorganized cytomegalic foetal cortical tissue. Attention was drawn to the association of adrenal hypoplasia with low maternal urinary estriol excretion in the last trimester of pregnancy, with post-maturity, difficulty in induction of labour, poor progress during labour, and to the difficulties of clinical and biochemical confirmation of adrenal hypoplasia during life. In two infants the diagnosis was suspected and corticosteroid therapy given unsuccessfully. THE MARKS OF PRELEUKAEMIA
GUNZ,F. W. Medical Research Department, Kanematsu Memorial Institute, Sydney Hospital, Sydney, New South Wales The term preleukaemia suggests a state which precedes true leukaemia and can be clearly distinguished from it. However, since there are no absolute criteria of what constitutes leukaemia, it is impossible to draw a sharp demarcation line between the disease and any pathological processes leading up to it. Preleukaemia can thus only be diagnosed retrospectively. The discovery of acquired cytogenetic changes is often believed to indicate the imminence or presence of malignancy. In this paper the question is posed if clonal abnormalities in marrow cells denote preleukaemia and forecast the later development of leukaemia. Details are presented of the case of a man with lymphosarcoma whose marrow, when first seen before treatment, was mildly hypoplastic; but without abnormal chromosomes. The lymphoma was controlled by chemo- and radiotherapy, but 7 mth after treatment was discontinued, the patient developed pancytopenia. A hypodiploid clone was now observed in the marrow, and additional hyperdiploid and polyploid lines were found 4 mth later, together with the clear features of acute granulocytic leukaemia. This case showed the concurrent development of leukaemia and of chromosome abnormalities. I t is contrasted with others where clonal changes were found in haematologically abnormal patients but leukaemia failed to develop, and with a second group in which leukaemia followed longstanding blood dyscrasias but without any observable chromosome abnormalities. It was concluded that new cytogenetic techniques are required before the precise rattionship between leukaemia and its pathological antecedents can be properly assessed. CLINICAL AND MORPHOLOGICAL FEATURES OF A CASE LEU KAEM I C R ETICU LO EN DOTH ELI0s IS
OF
IRONSIDE, P. Cancer Institute, Melbourne, Victoria
A case of leukaemic reticuloendotheliosis was described to indicate the individuality of the disease and its