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LYMPHOCYTIC LYMPHOMA AFTER IDIOPATHIC HYPERSPLENISM
881 between the pattern of 1966 and that of the preceding years. Indeed in the U.S.A. conceptions tend to be concentrated in the months from October to January.2 These were the months in which there was clustering of conceptions with trisomic
karyotypes. A seasonal fluctuation in ovulation-rate, at least in women with a temporarily or a chronically unstable hypothalamic/ pituitary/ovarian axis, has been claimed to be responsible for seasonality of conception-rate. 1,4 The apparently simultaneous clustering of disproportionate rates of pathological conceptions with or without chromosomal aberrations4. suggests to us that at the time of the breakthrough from anovulation to ovulation and vice versa, fertilisation of overripe eggs may occur, leading to non-disjunction or otherwise abnormal conceptions. Huize Mana Roepaan, Centre for Observation and Treatment of Mental Retardates, Ottersum (L.), Netherlands
P. H.
Department of Human Genetics, Free University, Amsterdam,
J. H. J. VAN ERKELENS-ZWETS
JONGBLOET
LYMPHOCYTIC LYMPHOMA AFTER IDIOPATHIC HYPERSPLENISM
SIR,-An enlarged spleen is generally secondary to some other condition, but in a few patients no underlying disease is found and in these cases splenectomy usually corrects abnormalities in the blood-count. We have seen a patient who had a lymphocytic lymphoma associated with an immune haemolyticanaemia 6 years after "idiopathic" hyerpsplenism. This 50-year-old woman presented in 1970 with a spleen extending 9 cm below the left ribs. There was no lymphadenopathy and no sign of portal hypertension. The liver was just palpable. She has never been abroad. She had pancytopenia (hemoglobin 12.0g/dl, white blood-cells 2.4x109/1, platelets 55x10/1). The bone-marrow was hyperplastic without evidence of infiltration with malignant cells. Immunoelectrophoresis of serum was normal. No cause for the splenic enlargement was found and the spleen was removed, with liver and retroperitoneal lymph-node biopsy. The liver, spleen, and lymphnodes showed features of reactive hyperplasia only. 4 weeks later the haemoglobin was 14.2g/dl, white blood-cells 10 x 109/1 (69% polymorphs), and platelets 206 x 109/1. Since 1971 she had been on methyldopa for mild essential hypertension. The daily dose was increased in July, 1976, to 1.5 g, when she complained of headaches and fatigue. In December, 1976, she was admitted to hospital with progressive decrease in exercise tolerance, weakness, fatigue, and ankle swelling. She was pale and icteric. Blood-pressure 110/70 mm Hg. Liver palpable 2 cm below the ribs. There were no other abnormal physical signs. Haemoglobin 7-1g/dl, with 11% reticulocytes, white cells 17-2x 109/1 (76% polymorphs), and platelets 440x10/1. Direct Coombs test positive (1/256), due to the binding to red cells of complement only. A non-specific red-cell agglutinin active in saline at room temperature and at 37°C was detected in the serum. Further investigations failed to reveal a cause for the haemolysis. Treatment with methyldopa was discontinued and she was given prednisolone 60 mg/day, but this did not control the haemolytic process. Mean red-cell lifespan was 18 days. Serum-immunoglobulins were below normal. Bipedal lymphangiography demonstrated para-aortic 2 Vital and Health Statistics: Seasonal Variation of Births, United States, 1933-1963, series 21, no. 9. National Center for Health Statistics, Washington, 1966. 3 Jongbloet, P. H. Mental and Physical Handicaps in Connection with Overripeness Ovopathy. Leiden, 1971. 4 Jongbloet, P. H. in Aging Gametes (edited by H. E. Stenfert and N. V. Kroese); p. 300. Basle, 1975. 5. Jongbloet, P. H., van Erkelens-Zwets, J. H. J., Holleman-van der Woude, REAP (Int, J. Res. Exch. Pract. ment. Retard.), 1976, 2, 243.
lymph-node enlargement. Liver scan showed even uptake throughout. At laparotomy grossly enlarged para-aortic lymph-nodes were found, and biopsy revealed diffuse replacement by a poorly differentiated lymphocytic lymphoma. Haemolysis persisted and was difficult to control despite treatment of the lymphoma with chemotherapy. Two of the ten patients with primary hypersplenism described by Dacie et al.I subsequently had lymphoma. Our patient resembles one of theirs both in the interval between presentation and development of lymphoma (6 years and 4 i years, respectively) and in the fact that the autoimmune hsemolytic ansemia was a’ major feature of both patients. Autoimmune haemolysis in our patient was at first thought to be related to treatment with methyldopa, but continuation of haemolysis several months after withdrawal of the drug and particularly the serological finding of a "complement only" pattern would essentially rule this out.2 We cannot say whether non-tropical idiopathic hypersplenism is a pre-lymphoma or merely an abnormal immune response to endogenous or exogenous stimuli, as suggested by Dacie et al. Long and Aisenberg3 suggested that idiopathic splenomegaly is a benign inflammatory lesion; none of their ten patients had had lymphoma after 1-8 years of follow-up. Skarin et al.4 described eleven patients with primary lymphosarcoma, eight of whom had hypersplenism, but our patient does not resemble those in Skarin’s series either histologically or clinically. Further study is required to elucidate criteria for predicting which patients with idiopathic splenomegaly are likely to develop lymphoma subsequently. Department of Haematology, University Hospital of Wales
S. A. D. AL-ISMAIL D. P. BENTLEY J. A. WHITTAKER
and Welsh National School of Medicine, Cardiff CF4 4XW
ROLE OF ANTIBIOTICS IN INFLAMMATORY DISC LESIONS IN CHILDREN
SIR,-None would question the use of antibiotics where a bacterial aetiology is clear or the clinical and laboratory evidence is strongly suggestive-e.g., early haematogenous osteomyelitis in children. Where such evidence is lacking, the routine use of antibiotics, some of which may have harmful side-effects, should be challenged. Inflammation of the intervertebral discs in young children ("discitis") falls into this category: it is an important, but uncommon, condition which runs a benign, self-limiting course and frequently poses diagnostic difficulties. We have reviewed thirty-five cases of inflammatory disc lesions treated at the Royal Alexandra Hospital for Children over the years 1960-75. The average age at onset was 2 years and 11 months for the twenty girls and fifteen boys in the series. The variable mode of presentation is well-recognised, and most of the children were admitted to wards other than orthopaedic. A battery of investigations was done in the search for an infectious aetiology, and, as in previous reports, positive results were notably absent. The one common positive finding was a raised erythrocyte-sedimentation rate (E.S.R.) which averaged 48 mm in the 1st hour (Westergren) with a range of 25-80. Seventeen children had mild leucocytosis. Management differed considerably according to the specialty under which the children were admitted. Immobilisation was either by bed rest or abduction frame. Antibiotics (many and various) did not accelerate the disappearance of symptoms and signs or the return of the E.s.R. to normal (see table). The same held true when the data for bed rest and frame immobilisation were compared, irrespective of whether or not antibiotics had been given. -
1. 2. 3. 4. 5.
Dacie, J. V., and others. Br. J. Hœmat. 1969, 17, 317. Brown, E. B. Progr. Hœmat. 1973, 3, 45. Long, J. C., Aisenberg, A. C. Cancer, 1973, 33, 1054. Skarin, A. T. Archs intern. Med. 1971, 127, 259. Menelaus, M. J. Bone Jt Surg. 1964, 46B, 16.
karyotypes. A seasonal fluctuation in ovulation-rate, at least in women with a temporarily or a chronically unstable hypothalamic/ pituitary/ovarian axis, has been claimed to be responsible for seasonality of conception-rate. 1,4 The apparently simultaneous clustering of disproportionate rates of pathological conceptions with or without chromosomal aberrations4. suggests to us that at the time of the breakthrough from anovulation to ovulation and vice versa, fertilisation of overripe eggs may occur, leading to non-disjunction or otherwise abnormal conceptions. Huize Mana Roepaan, Centre for Observation and Treatment of Mental Retardates, Ottersum (L.), Netherlands
P. H.
Department of Human Genetics, Free University, Amsterdam,
J. H. J. VAN ERKELENS-ZWETS
JONGBLOET
LYMPHOCYTIC LYMPHOMA AFTER IDIOPATHIC HYPERSPLENISM
SIR,-An enlarged spleen is generally secondary to some other condition, but in a few patients no underlying disease is found and in these cases splenectomy usually corrects abnormalities in the blood-count. We have seen a patient who had a lymphocytic lymphoma associated with an immune haemolyticanaemia 6 years after "idiopathic" hyerpsplenism. This 50-year-old woman presented in 1970 with a spleen extending 9 cm below the left ribs. There was no lymphadenopathy and no sign of portal hypertension. The liver was just palpable. She has never been abroad. She had pancytopenia (hemoglobin 12.0g/dl, white blood-cells 2.4x109/1, platelets 55x10/1). The bone-marrow was hyperplastic without evidence of infiltration with malignant cells. Immunoelectrophoresis of serum was normal. No cause for the splenic enlargement was found and the spleen was removed, with liver and retroperitoneal lymph-node biopsy. The liver, spleen, and lymphnodes showed features of reactive hyperplasia only. 4 weeks later the haemoglobin was 14.2g/dl, white blood-cells 10 x 109/1 (69% polymorphs), and platelets 206 x 109/1. Since 1971 she had been on methyldopa for mild essential hypertension. The daily dose was increased in July, 1976, to 1.5 g, when she complained of headaches and fatigue. In December, 1976, she was admitted to hospital with progressive decrease in exercise tolerance, weakness, fatigue, and ankle swelling. She was pale and icteric. Blood-pressure 110/70 mm Hg. Liver palpable 2 cm below the ribs. There were no other abnormal physical signs. Haemoglobin 7-1g/dl, with 11% reticulocytes, white cells 17-2x 109/1 (76% polymorphs), and platelets 440x10/1. Direct Coombs test positive (1/256), due to the binding to red cells of complement only. A non-specific red-cell agglutinin active in saline at room temperature and at 37°C was detected in the serum. Further investigations failed to reveal a cause for the haemolysis. Treatment with methyldopa was discontinued and she was given prednisolone 60 mg/day, but this did not control the haemolytic process. Mean red-cell lifespan was 18 days. Serum-immunoglobulins were below normal. Bipedal lymphangiography demonstrated para-aortic 2 Vital and Health Statistics: Seasonal Variation of Births, United States, 1933-1963, series 21, no. 9. National Center for Health Statistics, Washington, 1966. 3 Jongbloet, P. H. Mental and Physical Handicaps in Connection with Overripeness Ovopathy. Leiden, 1971. 4 Jongbloet, P. H. in Aging Gametes (edited by H. E. Stenfert and N. V. Kroese); p. 300. Basle, 1975. 5. Jongbloet, P. H., van Erkelens-Zwets, J. H. J., Holleman-van der Woude, REAP (Int, J. Res. Exch. Pract. ment. Retard.), 1976, 2, 243.
lymph-node enlargement. Liver scan showed even uptake throughout. At laparotomy grossly enlarged para-aortic lymph-nodes were found, and biopsy revealed diffuse replacement by a poorly differentiated lymphocytic lymphoma. Haemolysis persisted and was difficult to control despite treatment of the lymphoma with chemotherapy. Two of the ten patients with primary hypersplenism described by Dacie et al.I subsequently had lymphoma. Our patient resembles one of theirs both in the interval between presentation and development of lymphoma (6 years and 4 i years, respectively) and in the fact that the autoimmune hsemolytic ansemia was a’ major feature of both patients. Autoimmune haemolysis in our patient was at first thought to be related to treatment with methyldopa, but continuation of haemolysis several months after withdrawal of the drug and particularly the serological finding of a "complement only" pattern would essentially rule this out.2 We cannot say whether non-tropical idiopathic hypersplenism is a pre-lymphoma or merely an abnormal immune response to endogenous or exogenous stimuli, as suggested by Dacie et al. Long and Aisenberg3 suggested that idiopathic splenomegaly is a benign inflammatory lesion; none of their ten patients had had lymphoma after 1-8 years of follow-up. Skarin et al.4 described eleven patients with primary lymphosarcoma, eight of whom had hypersplenism, but our patient does not resemble those in Skarin’s series either histologically or clinically. Further study is required to elucidate criteria for predicting which patients with idiopathic splenomegaly are likely to develop lymphoma subsequently. Department of Haematology, University Hospital of Wales
S. A. D. AL-ISMAIL D. P. BENTLEY J. A. WHITTAKER
and Welsh National School of Medicine, Cardiff CF4 4XW
ROLE OF ANTIBIOTICS IN INFLAMMATORY DISC LESIONS IN CHILDREN
SIR,-None would question the use of antibiotics where a bacterial aetiology is clear or the clinical and laboratory evidence is strongly suggestive-e.g., early haematogenous osteomyelitis in children. Where such evidence is lacking, the routine use of antibiotics, some of which may have harmful side-effects, should be challenged. Inflammation of the intervertebral discs in young children ("discitis") falls into this category: it is an important, but uncommon, condition which runs a benign, self-limiting course and frequently poses diagnostic difficulties. We have reviewed thirty-five cases of inflammatory disc lesions treated at the Royal Alexandra Hospital for Children over the years 1960-75. The average age at onset was 2 years and 11 months for the twenty girls and fifteen boys in the series. The variable mode of presentation is well-recognised, and most of the children were admitted to wards other than orthopaedic. A battery of investigations was done in the search for an infectious aetiology, and, as in previous reports, positive results were notably absent. The one common positive finding was a raised erythrocyte-sedimentation rate (E.S.R.) which averaged 48 mm in the 1st hour (Westergren) with a range of 25-80. Seventeen children had mild leucocytosis. Management differed considerably according to the specialty under which the children were admitted. Immobilisation was either by bed rest or abduction frame. Antibiotics (many and various) did not accelerate the disappearance of symptoms and signs or the return of the E.s.R. to normal (see table). The same held true when the data for bed rest and frame immobilisation were compared, irrespective of whether or not antibiotics had been given. -
1. 2. 3. 4. 5.
Dacie, J. V., and others. Br. J. Hœmat. 1969, 17, 317. Brown, E. B. Progr. Hœmat. 1973, 3, 45. Long, J. C., Aisenberg, A. C. Cancer, 1973, 33, 1054. Skarin, A. T. Archs intern. Med. 1971, 127, 259. Menelaus, M. J. Bone Jt Surg. 1964, 46B, 16.