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Splenic lymphoma with villous lymphocytes complicated by autoimmune haemolytic anaemia
1106
Splenic lymphoma with villous lymphocytes complicated by autoimmune haemolytic anaemia SIR,-Dr Bates and colleagues (Sept 5, p 575) report 10 patients with splenic lymphoma with villous lymphocytes (SLVL), 1 of whom died from "acute haemolysis". We report 2 patients with SLVL complicated by autoimmune haemolytic anaemia. Patient 1(70-year-old woman, 3-month history of fatigue and breathlessness on exertion). On examination she was pale, her spleen was palpable at 19 cm, and liver edge was 2 cm below the costal margins. She had no lymphadenopathy. Initial investigations confirmed anaemia (haemoglobin [Hb] 65 g/dl; mean corpuscular volume [MCV] 101 fl). The reticulocyte count was 24% and serum haptoglobins were absent. The direct antiglobulin test was positive and a warm reacting IgG autoantibody of no discernible blood group specificity was found in serum and eluted from cells. The white blood cell count was 4-9 x 109/1 (73% lymphocytes) and the peripheral blood film showed polychromasia, microspherocytes, basophilic stippling, and lymphocytes with polar cytoplasmic projections morphologically resembling SLVL cells (figure). Bone marrow trephine biopsy showed infiltration by small lymphocytes. Immunophenotyping of peripheral blood lymphocytes confirmed SLVL (table).1 After initially responding to prednisolone and combination chemotherapy, she subsequently relapsed. She then underwent splenectomy, and SLVL and autoimmune haemolytic anaemia have since remained in remission.
Lymphocytes with polar cytoplasmic projections
in
patient 1.
Patient 2 (94-year-old woman, about 6 kg weight loss over 1 year, and a 6-month history of increasing weakness, dizziness, and dyspnoea on exertion). On examination she was pale and her spleen was palpable at 8 cm below the costal margin. She had no hepatomegaly or lymphadenopathy. Investigations confirmed anaemia (Hb 7-8 g/dl; MCV 105 fl), with unconjugated hyperbilirubinaemia, absent serum haptoglobins, and a positive direct antiglobulin test (IgG and complement). The results were consistent with autoimmune haemolytic anaemia. The white cell count was 32 x 109/1 (90% lymphocytes), and peripheral blood morphology showed polychromasia, microspherocytes, and SLVL-type cells. Immunophenotyping of peripheral blood lymphocytes confirmed SLVL (table). She initially responded to treatment with prednisolone, cyclophosphamide, and blood
IMMUNOPHENOTYPE ANALYSIS
transfusion; however, her general condition deteriorated and she died of bronchopneumonia 3 months later. The association of autoimmune haemolytic anaemia with B-cell neoplasms is well recognised. Immunological abnormalities occur in SLVL, with 60% of patients having a serum paraprotein,2 although neither of our patients showed this feature. As far as we are aware, however, the association of autoimmune haemolytic anaemia with SLVL has not previously been reported. We thank Ms Faith
Wright for immunophenotyping.
Department of Haematology, Royal Free Hospital and School of Medicine, London NW3 2QG, UK 1. 2.
RICHARD GALE OWEN P. SMITH MARION WOOD A. B. MEHTA
Catovsky D. The classification of leukaemias and leukaemia/lymphoma syndromes of mature B and T cells. Br J Haematol 1992; 82: 226-29. Melo JV, Hegde U, Parreira A, Thompson I, Lampert IA, Catovsky D. Splenic B cell lymphoma with circulating villous lymphocytes: differential diagnosis of B cell leukaemias with large spleens. J Clin Pathol 1987; 40: 642-51.
Pre-pregnancy care in diabetes SIR,- That those most in need of clinical care are often least likely voluntarily to use the clinical services provided is well-recognised, and few would dispute Dr Gregory and Professor Tattersall’s contention (Sept 12, p 656) that this fact is relevant to the management of diabetic pregnancy. We certainly agree that the delivery of effective and cost-efficient care to diabetic women can take many forms and that the method used has to be tailored to the individual patient as well as to local conditions, such as the number of patients dealt with at the centre, the geography of and the social and educational mix within the catchment area, the resources available, and the existing overall level of diabetic care and metabolic control. Your Oct 10 correspondents (p 918) raise important points, but we are concerned that the most important message-namely, that all diabetic women need prospective advice and care in relation to both metabolic regulation and all aspects of reproduction and that every effort must be made to get this to them-may be lost, firstly by the ambiguous and provocative title of this paper, and secondly by rapid scanning of the text which focuses on one method of delivering such care (ie, in a formal pre-pregnancy clinic) and on one particular outcome (malformation). That our fear of confusion and misunderstanding arising is indeed justified is indicated by the title and opening sentence of your talking-point for the report which states that, "The value of pregnancy care for diabetic women in the prevention of congenital abnormalities and perinatal deaths remains controversial". In fact nothing could be further from the truth. The main causes of the very high perinatal mortality and morbidity rates seen universally in diabetic pregnancy until the sixties are late intra-uterine death, prematurity, low birthweight, and malformation. There is general agreement that, as shown by the national data for Sweden,l the main factor responsible for the striking fall in these rates over the past two decades is improved control of maternal blood glucose instituted at diagnosis of pregnancy, with the main impact of better metabolic regulation seen on the stillbirth rate and little or no effect observed on the frequency of malformation, spontaneous premature labour, or infants light for gestational age-the last two contributing most of the serious neonatal morbidity seen. Although the precise mechanisms underlying the development of these complications of diabetic pregnancy have not been defined, it is now known that they tend to be associated with each other and to occur particularly in diabetic mothers with clinically evident microangiopathy,that ultrasound evidence of fetal early growth delay is associated with an increased risk of malformationand that
they are seen significantly less often, though are not abolished, when good metabolic control has been established before conception and maintained throughout the period of major organogenesis (6-8 weeks’ gestation).’ This fact is the basis of the recommendation in the St Vincent Declaration Implementation Documents that diabetic women should receive closely controlled care at a specialist centre before, during, and after pregnancy.
Splenic lymphoma with villous lymphocytes complicated by autoimmune haemolytic anaemia SIR,-Dr Bates and colleagues (Sept 5, p 575) report 10 patients with splenic lymphoma with villous lymphocytes (SLVL), 1 of whom died from "acute haemolysis". We report 2 patients with SLVL complicated by autoimmune haemolytic anaemia. Patient 1(70-year-old woman, 3-month history of fatigue and breathlessness on exertion). On examination she was pale, her spleen was palpable at 19 cm, and liver edge was 2 cm below the costal margins. She had no lymphadenopathy. Initial investigations confirmed anaemia (haemoglobin [Hb] 65 g/dl; mean corpuscular volume [MCV] 101 fl). The reticulocyte count was 24% and serum haptoglobins were absent. The direct antiglobulin test was positive and a warm reacting IgG autoantibody of no discernible blood group specificity was found in serum and eluted from cells. The white blood cell count was 4-9 x 109/1 (73% lymphocytes) and the peripheral blood film showed polychromasia, microspherocytes, basophilic stippling, and lymphocytes with polar cytoplasmic projections morphologically resembling SLVL cells (figure). Bone marrow trephine biopsy showed infiltration by small lymphocytes. Immunophenotyping of peripheral blood lymphocytes confirmed SLVL (table).1 After initially responding to prednisolone and combination chemotherapy, she subsequently relapsed. She then underwent splenectomy, and SLVL and autoimmune haemolytic anaemia have since remained in remission.
Lymphocytes with polar cytoplasmic projections
in
patient 1.
Patient 2 (94-year-old woman, about 6 kg weight loss over 1 year, and a 6-month history of increasing weakness, dizziness, and dyspnoea on exertion). On examination she was pale and her spleen was palpable at 8 cm below the costal margin. She had no hepatomegaly or lymphadenopathy. Investigations confirmed anaemia (Hb 7-8 g/dl; MCV 105 fl), with unconjugated hyperbilirubinaemia, absent serum haptoglobins, and a positive direct antiglobulin test (IgG and complement). The results were consistent with autoimmune haemolytic anaemia. The white cell count was 32 x 109/1 (90% lymphocytes), and peripheral blood morphology showed polychromasia, microspherocytes, and SLVL-type cells. Immunophenotyping of peripheral blood lymphocytes confirmed SLVL (table). She initially responded to treatment with prednisolone, cyclophosphamide, and blood
IMMUNOPHENOTYPE ANALYSIS
transfusion; however, her general condition deteriorated and she died of bronchopneumonia 3 months later. The association of autoimmune haemolytic anaemia with B-cell neoplasms is well recognised. Immunological abnormalities occur in SLVL, with 60% of patients having a serum paraprotein,2 although neither of our patients showed this feature. As far as we are aware, however, the association of autoimmune haemolytic anaemia with SLVL has not previously been reported. We thank Ms Faith
Wright for immunophenotyping.
Department of Haematology, Royal Free Hospital and School of Medicine, London NW3 2QG, UK 1. 2.
RICHARD GALE OWEN P. SMITH MARION WOOD A. B. MEHTA
Catovsky D. The classification of leukaemias and leukaemia/lymphoma syndromes of mature B and T cells. Br J Haematol 1992; 82: 226-29. Melo JV, Hegde U, Parreira A, Thompson I, Lampert IA, Catovsky D. Splenic B cell lymphoma with circulating villous lymphocytes: differential diagnosis of B cell leukaemias with large spleens. J Clin Pathol 1987; 40: 642-51.
Pre-pregnancy care in diabetes SIR,- That those most in need of clinical care are often least likely voluntarily to use the clinical services provided is well-recognised, and few would dispute Dr Gregory and Professor Tattersall’s contention (Sept 12, p 656) that this fact is relevant to the management of diabetic pregnancy. We certainly agree that the delivery of effective and cost-efficient care to diabetic women can take many forms and that the method used has to be tailored to the individual patient as well as to local conditions, such as the number of patients dealt with at the centre, the geography of and the social and educational mix within the catchment area, the resources available, and the existing overall level of diabetic care and metabolic control. Your Oct 10 correspondents (p 918) raise important points, but we are concerned that the most important message-namely, that all diabetic women need prospective advice and care in relation to both metabolic regulation and all aspects of reproduction and that every effort must be made to get this to them-may be lost, firstly by the ambiguous and provocative title of this paper, and secondly by rapid scanning of the text which focuses on one method of delivering such care (ie, in a formal pre-pregnancy clinic) and on one particular outcome (malformation). That our fear of confusion and misunderstanding arising is indeed justified is indicated by the title and opening sentence of your talking-point for the report which states that, "The value of pregnancy care for diabetic women in the prevention of congenital abnormalities and perinatal deaths remains controversial". In fact nothing could be further from the truth. The main causes of the very high perinatal mortality and morbidity rates seen universally in diabetic pregnancy until the sixties are late intra-uterine death, prematurity, low birthweight, and malformation. There is general agreement that, as shown by the national data for Sweden,l the main factor responsible for the striking fall in these rates over the past two decades is improved control of maternal blood glucose instituted at diagnosis of pregnancy, with the main impact of better metabolic regulation seen on the stillbirth rate and little or no effect observed on the frequency of malformation, spontaneous premature labour, or infants light for gestational age-the last two contributing most of the serious neonatal morbidity seen. Although the precise mechanisms underlying the development of these complications of diabetic pregnancy have not been defined, it is now known that they tend to be associated with each other and to occur particularly in diabetic mothers with clinically evident microangiopathy,that ultrasound evidence of fetal early growth delay is associated with an increased risk of malformationand that
they are seen significantly less often, though are not abolished, when good metabolic control has been established before conception and maintained throughout the period of major organogenesis (6-8 weeks’ gestation).’ This fact is the basis of the recommendation in the St Vincent Declaration Implementation Documents that diabetic women should receive closely controlled care at a specialist centre before, during, and after pregnancy.