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WHEN IS AIDS NOT AIDS?
986 FOURTH CASE OF AIDS IN HAEMOPHILIC CHILDREN IN SEVILLE
SIR,-Haemophiliacs, though thought of as a risk population for1 acquired immunodeficiency syndrome (AIDS), are rarely affected.’ By July, 1983, the US Centers for Disease Control had recorded only twenty-two cases.2Of the four European cases one was in Wales, and the other three were all in Spanish patients in the care of Virgen del Rocio Hospital in Seville. We describe here another case of AIDS in a haemophiliac child at the same hospital. This 5-year-old boy who had been transfused several times with commercial factor VIII had inhibitor against this factor. In January, 1984, he was symptom-free, but lymphocyte subpopulation studies revealed a decrease in the helper/suppressor ratio (0’ 6). 2 months later he had a persistent cough, anorexia, weight loss, and unexplained transient fever, followed, 1 month after that, by fever, marked
intense
asthenia,
vomiting,
and
generalised
lymphadenopathy. He
was
cutaneous
admitted anergy,
hospital seriously ill and emaciated. He had polyclonal hypergammaglobulinaemia, and a
to
0-2. In sputum and urine cultures Candida albicans and Proteus mirabilis were isolated, respectively. Significant seroconversion to Bordetella titres, serological evidence of previous infection with Epstein-Barr and hepatitis B viruses and high antibody titres to C albicans and Toxoplasma were also noted. Ganglion biopsy indicated lymphoid follicular hyperplasia. Endoscopy and culture pointed to oesophagcal candidiasis. In addition we found autoimmune haemolytic anaemia with cryoantibodies and slightly raised transaminase levels. There was
helper/suppressor ratio of
no
lymphocytopenia.
Treatment with 5-fluocytosine, ketoconazole, and AM3was started and the patient is currently symptom-free with negative
microbiological tests. The accumulation of AIDS cases in the haemophilic population served by this hospital contrasts with epidemiological findings in the United States and elsewhere in Europe.5The detection of an abnormal helper/suppressor ratio when the patient was symptomfree reinforces the idea that this anomaly can sometimes suggest a preclinical stage of AIDS.6 Concomitant gram-negative bacterial infections and absence of lymphopenia have been described in other children with AIDS.7 Department of Haematology, Children’s Hospital, CS "Virgen del Rocio", Seville, Spain
P. NOGUEROL M. LEAL R. SOSA
1. Centers for Disease Control: Update: Acquired immunodeficiency syndrome (AIDS)-
United States. MMWR 1984; 32: 688-91. 2. Evatt BL, Ramsey RB, Lawrence DN, Zyla LD, Curran JW. The acquired immunodeficiency syndrome in patients with haemophilia. Ann Intern Med 1984; 100: 499-504: 3. Leal M, Lissen E, Wichmann I, Jimenez JM, Andreu-Kern F. AIDS in haemophilia patients in Spain. Lancet 1983; i: 992-93 4. Canavate ML, Ponton J, Amurrio C, Regulez P, Canada JL, Saura A, Cisterna R, Pivel JP, Sada G. Effect of a new immunomodulator on mouse macrophage activity. Rev Clin Esp 1984; 3: 159-62. 5. Pinching AJ Un aperçu sur le SIDA au Roysume-Uni Méd Hyg 1984; 42: 1570-74 6. Lederman MM, Ratnoff OD, Scillian JJ, Jones PK, Schacter B. Impaired cell-mediated immunity in patients with classic hemophilia. N Engl J Med 1983, 308: 79-83. 7. Scott G, Buck BE, Leterman JB, Bloom FL, Parks W. Acquired immunodeficiency syndrome in infants. N Engl J Med 1984; 310: 75-80.
WHEN IS AIDS NOT AIDS?
of B cell origin have an increased incidence men with the acquired immunodeficiency syndrome (AIDS).1 ,In AIDS T helper cells (OKT4) are usually reduced with a reduction in helper to suppressor (OKT8) ratios to below 1-0, but this is not an absolute criterion.2 Delayed type 2 hypersensitivity skin testing is also compromised.2 In June, 1984 we saw a 24-year-old homosexual with a swelling in the left side of his neck which proved to be a 2 cm lymph node. The node was removed and the histologist reported a centroblastic 3 lymphoma, which is of B cell origin.3 Did this man have AIDS? He had none of the other symptoms or signs suggestive of the syndrome. He gave no history of contact with an AIDS patient but he had had several homosexual contacts in the previous year, none from the USA but some from London. Delayed
SIR,-Lymphomas
among homosexual
type hypersensitivity testing to streptodornase and Candida a/bieans was positive, that to tuberculin was negative. The differential white cell count was normal, with an OKT4 count of 1 - 06 x 109/1 and an OKT8 count of 0.53 x 109/1. Despite these results we were not satisfied that this was not a case of AIDS. Blood samples were sent to London and Paris for serological studies of human T cell leukaemia virus type III (HTLV-III) and lymphadenopathy virus (LAV).4.) For 4 weeks, while the serological results were awaited, this patient and his samples were handled in accordance with the recommendations of Conte et al. Shortly after radiotherapy began the patient complained of pain in his throat and a small mass was identified in the left tonsillar bed. Biopsy was arranged but when it came to admitting him as a suspected AIDS case, there was such illinformed resistance that he had to be transferred to another unit. Adler and Weller have described similar experiences.7 HTLV-111 and LAV antibody titres were negative and we think that this man does not have AIDS. Bearing in mind the implications for management and possible disruption of the services a patient may receive, at what point of investigation should a case such as this be declared "AIDS-free"? We thank Dr R. S. Tedder for HTLV-III for LAV serology.
Royal Victoria Hospital, Belfast BT 12 6BA
serology and Dr F. Brun-Vezinet R. D. MAW S. MCKELVEY
J. SLOAN
Miner RC, et al. Outbreak of Burkitt’s like lymphoma in homosexual men. Lancet 1982; ii: 631-33. 2. Siegal FP. Sem Oncol 1984; 11(1): 29-39. 3. Wright DH, Isaacson PG. Biopsy pathology of the lymphoreticular system. London Chapman and Hall, 1983: 119-27. 4. Safai B, Sarngadharan MG, Groopman JE, et al. Seroepidemiological studies of human T lymphotropic retrovirus type III in acquired immunodeficiency syndrome Lancet 1984; i: 1438-40. 5. Brun-Vezmet F, Rouzioux C, Barré-Sinoussi F, et al. Detection of IgG antibodies to lymphadenopathy associated virus in patients with AIDS or lymphadenopathy syndrome. Lancet 1984; i: 1253-56. 6. Conte JE, Hadley WK, Sande M, and the University of California, San Francisco, Task Force on the Acquired Immune Deficiency Syndrome Infection control guidelines for patients with the acquired immunodeficiency syndrome AIDS N Engl J Med 1983; 309: 740-44. 7. Adler MW, Weller IV. AIDS-Sense not fear. Br Med J 1984; 288: 1777-78. 1.
Ziegler JL, Drew WL,
FRUCTOSE AND MISLEADING GLYCOSYLATION DATA
SIR,-Fructose and fructose-containing substances are thought to be safe in diabetics despite the calorific content. I have been treating a diabetic aged 17 who, I believe, demonstrates the dangers of taking fructose in the diet. Diabetes had been diagnosed at the age of 5 years and she was managed at first by a free diet in association with soluble and protamine-zinc insulin. By the age of 14 she had changed to a sugarfree but otherwise liberal diet and then started routine blood testing, readings being consistently 1-7 mmol/1 (’BM 20-800’). Her technique and readings were checked by doctors, diabetic specialist health visitors, and by her family and re-checked by measurements on random sugars in the clinic, which confirmed good control. However, urine analysis (’Clinitest’) remained positive and glycosylated haemoglobin remained high (10-5-15-- 8%, normal up to 8 - 5 O/o). When reducing substances other than glucose were sought in her urine fructose was often found. She regularly ate jam, mints, and chocolate biscuits formulated for diabetics and plenty of usually an orange and an apple a day. She was asked to stop taking these fructose-containing substances (with a reasonable increase in other forms of carbohydrate to take their place), and 2 months later her glycosylated haemoglobin value is 6 - 5% with no change in selfmeasured blood glucose. Glycosylation can occur from many sugars and fructose would be an ideal sugar to do this. This case suggests an explanation for glycosylated haemoglobin values that seem incompatible with blood glucose estimations: diabetic patients with raised glycosylated haemoglobin values are usually accused of cheating.
fruit,
Leicester Royal Infirmary, Leicester LE1 5WW
A.C.BURDEN
SIR,-Haemophiliacs, though thought of as a risk population for1 acquired immunodeficiency syndrome (AIDS), are rarely affected.’ By July, 1983, the US Centers for Disease Control had recorded only twenty-two cases.2Of the four European cases one was in Wales, and the other three were all in Spanish patients in the care of Virgen del Rocio Hospital in Seville. We describe here another case of AIDS in a haemophiliac child at the same hospital. This 5-year-old boy who had been transfused several times with commercial factor VIII had inhibitor against this factor. In January, 1984, he was symptom-free, but lymphocyte subpopulation studies revealed a decrease in the helper/suppressor ratio (0’ 6). 2 months later he had a persistent cough, anorexia, weight loss, and unexplained transient fever, followed, 1 month after that, by fever, marked
intense
asthenia,
vomiting,
and
generalised
lymphadenopathy. He
was
cutaneous
admitted anergy,
hospital seriously ill and emaciated. He had polyclonal hypergammaglobulinaemia, and a
to
0-2. In sputum and urine cultures Candida albicans and Proteus mirabilis were isolated, respectively. Significant seroconversion to Bordetella titres, serological evidence of previous infection with Epstein-Barr and hepatitis B viruses and high antibody titres to C albicans and Toxoplasma were also noted. Ganglion biopsy indicated lymphoid follicular hyperplasia. Endoscopy and culture pointed to oesophagcal candidiasis. In addition we found autoimmune haemolytic anaemia with cryoantibodies and slightly raised transaminase levels. There was
helper/suppressor ratio of
no
lymphocytopenia.
Treatment with 5-fluocytosine, ketoconazole, and AM3was started and the patient is currently symptom-free with negative
microbiological tests. The accumulation of AIDS cases in the haemophilic population served by this hospital contrasts with epidemiological findings in the United States and elsewhere in Europe.5The detection of an abnormal helper/suppressor ratio when the patient was symptomfree reinforces the idea that this anomaly can sometimes suggest a preclinical stage of AIDS.6 Concomitant gram-negative bacterial infections and absence of lymphopenia have been described in other children with AIDS.7 Department of Haematology, Children’s Hospital, CS "Virgen del Rocio", Seville, Spain
P. NOGUEROL M. LEAL R. SOSA
1. Centers for Disease Control: Update: Acquired immunodeficiency syndrome (AIDS)-
United States. MMWR 1984; 32: 688-91. 2. Evatt BL, Ramsey RB, Lawrence DN, Zyla LD, Curran JW. The acquired immunodeficiency syndrome in patients with haemophilia. Ann Intern Med 1984; 100: 499-504: 3. Leal M, Lissen E, Wichmann I, Jimenez JM, Andreu-Kern F. AIDS in haemophilia patients in Spain. Lancet 1983; i: 992-93 4. Canavate ML, Ponton J, Amurrio C, Regulez P, Canada JL, Saura A, Cisterna R, Pivel JP, Sada G. Effect of a new immunomodulator on mouse macrophage activity. Rev Clin Esp 1984; 3: 159-62. 5. Pinching AJ Un aperçu sur le SIDA au Roysume-Uni Méd Hyg 1984; 42: 1570-74 6. Lederman MM, Ratnoff OD, Scillian JJ, Jones PK, Schacter B. Impaired cell-mediated immunity in patients with classic hemophilia. N Engl J Med 1983, 308: 79-83. 7. Scott G, Buck BE, Leterman JB, Bloom FL, Parks W. Acquired immunodeficiency syndrome in infants. N Engl J Med 1984; 310: 75-80.
WHEN IS AIDS NOT AIDS?
of B cell origin have an increased incidence men with the acquired immunodeficiency syndrome (AIDS).1 ,In AIDS T helper cells (OKT4) are usually reduced with a reduction in helper to suppressor (OKT8) ratios to below 1-0, but this is not an absolute criterion.2 Delayed type 2 hypersensitivity skin testing is also compromised.2 In June, 1984 we saw a 24-year-old homosexual with a swelling in the left side of his neck which proved to be a 2 cm lymph node. The node was removed and the histologist reported a centroblastic 3 lymphoma, which is of B cell origin.3 Did this man have AIDS? He had none of the other symptoms or signs suggestive of the syndrome. He gave no history of contact with an AIDS patient but he had had several homosexual contacts in the previous year, none from the USA but some from London. Delayed
SIR,-Lymphomas
among homosexual
type hypersensitivity testing to streptodornase and Candida a/bieans was positive, that to tuberculin was negative. The differential white cell count was normal, with an OKT4 count of 1 - 06 x 109/1 and an OKT8 count of 0.53 x 109/1. Despite these results we were not satisfied that this was not a case of AIDS. Blood samples were sent to London and Paris for serological studies of human T cell leukaemia virus type III (HTLV-III) and lymphadenopathy virus (LAV).4.) For 4 weeks, while the serological results were awaited, this patient and his samples were handled in accordance with the recommendations of Conte et al. Shortly after radiotherapy began the patient complained of pain in his throat and a small mass was identified in the left tonsillar bed. Biopsy was arranged but when it came to admitting him as a suspected AIDS case, there was such illinformed resistance that he had to be transferred to another unit. Adler and Weller have described similar experiences.7 HTLV-111 and LAV antibody titres were negative and we think that this man does not have AIDS. Bearing in mind the implications for management and possible disruption of the services a patient may receive, at what point of investigation should a case such as this be declared "AIDS-free"? We thank Dr R. S. Tedder for HTLV-III for LAV serology.
Royal Victoria Hospital, Belfast BT 12 6BA
serology and Dr F. Brun-Vezinet R. D. MAW S. MCKELVEY
J. SLOAN
Miner RC, et al. Outbreak of Burkitt’s like lymphoma in homosexual men. Lancet 1982; ii: 631-33. 2. Siegal FP. Sem Oncol 1984; 11(1): 29-39. 3. Wright DH, Isaacson PG. Biopsy pathology of the lymphoreticular system. London Chapman and Hall, 1983: 119-27. 4. Safai B, Sarngadharan MG, Groopman JE, et al. Seroepidemiological studies of human T lymphotropic retrovirus type III in acquired immunodeficiency syndrome Lancet 1984; i: 1438-40. 5. Brun-Vezmet F, Rouzioux C, Barré-Sinoussi F, et al. Detection of IgG antibodies to lymphadenopathy associated virus in patients with AIDS or lymphadenopathy syndrome. Lancet 1984; i: 1253-56. 6. Conte JE, Hadley WK, Sande M, and the University of California, San Francisco, Task Force on the Acquired Immune Deficiency Syndrome Infection control guidelines for patients with the acquired immunodeficiency syndrome AIDS N Engl J Med 1983; 309: 740-44. 7. Adler MW, Weller IV. AIDS-Sense not fear. Br Med J 1984; 288: 1777-78. 1.
Ziegler JL, Drew WL,
FRUCTOSE AND MISLEADING GLYCOSYLATION DATA
SIR,-Fructose and fructose-containing substances are thought to be safe in diabetics despite the calorific content. I have been treating a diabetic aged 17 who, I believe, demonstrates the dangers of taking fructose in the diet. Diabetes had been diagnosed at the age of 5 years and she was managed at first by a free diet in association with soluble and protamine-zinc insulin. By the age of 14 she had changed to a sugarfree but otherwise liberal diet and then started routine blood testing, readings being consistently 1-7 mmol/1 (’BM 20-800’). Her technique and readings were checked by doctors, diabetic specialist health visitors, and by her family and re-checked by measurements on random sugars in the clinic, which confirmed good control. However, urine analysis (’Clinitest’) remained positive and glycosylated haemoglobin remained high (10-5-15-- 8%, normal up to 8 - 5 O/o). When reducing substances other than glucose were sought in her urine fructose was often found. She regularly ate jam, mints, and chocolate biscuits formulated for diabetics and plenty of usually an orange and an apple a day. She was asked to stop taking these fructose-containing substances (with a reasonable increase in other forms of carbohydrate to take their place), and 2 months later her glycosylated haemoglobin value is 6 - 5% with no change in selfmeasured blood glucose. Glycosylation can occur from many sugars and fructose would be an ideal sugar to do this. This case suggests an explanation for glycosylated haemoglobin values that seem incompatible with blood glucose estimations: diabetic patients with raised glycosylated haemoglobin values are usually accused of cheating.
fruit,
Leicester Royal Infirmary, Leicester LE1 5WW
A.C.BURDEN