58
Journal of the Royal Society of Medicine Volume 85 January 1992
Letters to the Editor Preference is given to letters commenting on contributions recently published in the JRSM. They should not-exceed 300 words and should be typed double-spaced.
Alfred the Great: a diagnosis I read the article on Alfred the Great by Craig with interest (May 1991 -JRSM, p 303). The argument for Crohn's disease is very ingenious. However, haemorrhoidal disease and complications are much more common. Bouts ofthrombosis and prolapse, with a probably psychologic overlay, occur frequently enough to account for the intermittency of Alfred's symptoms, rather than Crohn's disease. His many pursuits (wars, Viking attacks, governing, etc) would tend to rule out an affliction with a chronic debilitating disease. According to Asser, Alfred contracted the malady in the 'first flowering of youth'. On one occasion, the attack had been cured after Alfred lay prostrate in silent prayer for a long while. This suggests a simple reduction of haemorrhoids as Asser goes on 'to substitute for the pangs of the present and agonizing infirmity some less severe illness, on the understanding that the new illness would not be outwardly visible on his body'. His attack of pain during the Great Feast after he married Ealhswith may well have been due to prolapse or thrombosis precipitated by his nuptial exertions. Certainly, even a king would have been embarrassed at such a time to have displayed the cause of his pain! The term used by Asser, 'ficus' (a fig') is generally translated as 'piles', 'haemorrhoids', or St Fiacre's disease2. There appears to be at least two versions of who St Fiacre really was3'4. In one version, that he was an ordained Irish priest from Co. Kilkenny; in the other version, the first-born son of the King of Scotland, Eugene IV. He settled at the fringe of a forest not far from the village of Breuil-en Brie in a place which is called today 'Saint Fiacre' (Seine et Marne). Many miracles were attributed to St Fiacre. He was once accused of witchcraft and waited for several days outside the church sitting on a big stone waiting for an inquest. Apparently the stone softened while he was sitting on it and an imprint resulted. It was granted the mysterious power of curing haemorrhoids if the patient sat on it and kept faith. The French cab 'fiacre' derived its name from him. The Hotel de St Fiacre in Paris in the middle of the 17th century had these coaches for hire. As the sign of the Inn was an image of the saint, the coaches became called by that name. It was said that Henry V of England died of St Fiacre's disease, at Vincennes. Another royal sufferer was King Louis XIII. F I JACKSON Cross Cancer Institute, 11560 University Ave, Edmonton, AB, Canada T6G 1Z2
References 1 Rains AJH, Capper WM. Bailey & Love's short practice of surgery, 14th edn. London: HK Lewis & Co, 1968:989-96
2 Schwartz Si. Principles of surgery. New York: The Blakiston Division McGraw-Hill Book Company, 1969: 998 3 Racouchot JE, Petouraud CH, Rivoire JO. Saint Fiacre. The Healer of Haemorrhoids and Patron Saint of Proctology. Am J Proctol 1971;22:175-7 4 Thurston H, Attwater D, eds. Alban Butler's Lives of the Saints, complete edition. Westminster, Maryland: Christian Classics, 1981:400-61
Acute myeloid leukaemia complicating sarcoidosLs We read with interest the case report by Murphy et al. in which they report a case of acute myeloid leukaemia (AML) complicating sarcoidosis (June 1991 JRSM, p 368). This case is of interest because of the relationship between sarcoidosis and leukaemia. There have been reports of lung cancer and both Hodgkin's and nonHodgkin's lymphomas occurring in association with sarcoidosis'. Several lines of evidence indicate that this association is not fortuitous. In an epidemiological study of 2544 sarcoidosis patients the number of cases of malignant lymphoma and lung cancer was significantly greater than expected. In this group of sarcoidosis patients, lymphoma occurs 5.5 times more than expected2. The development of AML in sarcoidosis history is extremely rare. Five cases have been previously reported3-7. In two of;them, the sarcoidosis diagnosis must be discussed because the two diseases were diagnosed- at the same time, since granulomata may occur as a reaction of the tumour4. However, in three other cases, sarcoidosis preceded the AML by 3-7 years and Murphy's patient about 10 years5-7. It is difficult to propose one general hypothesis which explains why sarcoidosis may predispose to malignant disease. In -the sarcoidosis-lymphoma syndrome, the increased mitotic activity of the Blymphoid cells in the tissues involved with sarcoidosis lead to; an increased risk of subsequent malignanttransformation, as described in sicca syndrome and rheumatoid arthritis8. We do not know if sarcoidosis may predispose to AML, but it may be suggested that the depletion of circulating T-helper cells seen in sarcoidosis may lead to decreased tumour rejection or perhaps to decreased resistance against putative oncogenic viruses9. Reich suggested that sarcoid-like reactions and sarcoidosis may represent an immunological response to tumour antigen7. It is known that granulomatous inflammation occurs as a response to a large variety of insoluble antigens. Controversially, how can the 3-7 years' interval between sarcoidosis and AML diagnosis be explained? It seems to be interesting to report all cases of sarcoidosis in which AML occurred. A large series might provide an answer about sarcoidosis-AML relationship: causality or coincidence? F MALOISEL Service d'Onco-H6matologie, F OBERLING Hopital de Hautepierre, 67098
Strasbourg cedex, France
References 1 Brincker H. The sarcoidosis-lymphoma syndrome. Br J Cancer 1986;54:467-73 2 Brincker H. Coexistence of sarcoidosis and malignant disease: causality or coincidence? Sarcoidosis 1989; 6:31-43 3 Brandt L, Emanuelsson H, Mitelman F, Stenstam M, Soderstrom N. Pronounced deficiency in T-cells and lymphocyte chromosomal aberrations in a patient with sarcoidosis, myelofibrosis and acute leukemia following thorotrast angiography. Acta Med Scand 1977;201:487-9
Journal of the Royal Society of Medicine Volume 85 January 1992
4 Bordelon J, Stone MJ, Frenkel EP. Probable myeloblastic leukemoid reaction with disseminated sarcoidosis. South Med J 1977;70:1378-80 5 Hermann C, Andersen E, Videbaeck AA. Acute myeloblastic leukaemia in sarcoidosis treated with methotrexate. Scand J Haematol 1980;24:234-6 6 Hatake K, Aoyagi N, Takaku F. Acute myelomonocytic leukemia following remission of sarcoidosis. Oncology 1983;40:200-1 7 Reich JM. Acute myeloblastic leukemia and sarcoidosis. Implications for pathogenesis. Cancer 1985;55:366-9 8 Bataille R, Klein B, Luxembourg A, Durie B. Relation entre etats auto-immuns et canc6risation de cellules de la lign6e lymphocytaire B. In: Roux H, Luxembourg A, Roudier J, eds. Immunorhumatologie SOLAL 1990:170-89 9 Hanto DW, Frizzera G, Gajl-Peczalska KJ, Simmons RL. Epstein Barr virus, immunodeficiency and B-cell proliferation. Transplantation 1985;39:461-72
Intestinal tuberculosis We were interested to read the paper by Lingenfelser et al. (July 1991 JRSM, p436) entitled 'Intestinal tuberculosis: an ongoing diagnostic dilemma' but think that some points merit further discussion. The authors state that the rigid criteria for the diagnosis of intestinal tuberculosis have been extended to include an unequivocal response to anti-TB treatment in patients who have 'suggestive' clinical, radiological or operative evidence of intestinal disease and histologically proven TB elsewhere. They also wonder if a therapeutic trial of anti-TB therapy is warranted in patients with suspected intestinal disease alone. In their reported case the diagnosis of pulmonary tuberculosis was eventually made by mediastinal lymph-node biopsy. The idea that intestinal tuberculosis is usually associated with active pulmonary disease is no longer true and the absence of pulmonary involvement should not influence the diagnosis since it has become apparent that greater numbers of patients are presenting with isolated abdominal disease'. In Leicester we have just reviewed 24 cases of intestinal tuberculosis which presented to the General Surgical Department, (unpublished data). In our patient population only two patients (8%) had radiographic evidence.of pulmonary disease and this concurs with the frequency reported by others2. Although as the authors state the diagnosis is ideally made by seeing organisms on microscopy or culture, it is fully justified to instigate a closely monitored trial of chemotherapy in patients in whom the diagnosis is strongly suspected3 and in our series six patients (25%) did not have a histologically proven diagnosis but all made a dramatic improvement on treatment. The authors also state that 'laparotomy remains the definitive investigation in many patients with intestinal tuberculosis'. This statement needs qualification because although laparotomy may eventually be required in many patients other less invasive methods of obtaining tissue for microbiological examination may be employed including laparoscopy and biopsy4 and biopsy of the peritoneum under local anaesthesia5. Consideration should be given to these techniques if appropriate since there is evidence that laparotomy itself may be associated with significant morbidity in these patients6. M J UNDERWOOD M M THOMPSON R D SAYERS
A W HALL
Department of General Surgery, Glenfield General Hospital, Leicester
References 1 National survey of tuberculosis notifications in England and Wales 1978-9. BMJ 1980;281.895-8 2 Kolawole TM, Lewis EA. A radiological study of tuberculosis of the abdomen (gastrointestinal tract). AJR 1975;123:348-58 3 Lambrianides AL, Ackroyd N, Shorey BA. Abdominal tuberculosis. Br J Surg 1980;67:887-9 4 Wolfe JHN, Behn AR, Jackson BT. Tuberculous peritonitis and the role of diagnostic laparoscopy. Lancet 1979,i:852-3 5 Das P, Shulka HS. Clinical diagnosis of abdominal tuberculosis. Br J Surg 1976;63:941-6 6 Bhansali SK. Abdominal tuberculosis: experience with 300 cases. Am J Gastroenterol 1977;67:324-37
'Hysteria', 'functional' or 'psychogenic'? Drs Mace and Trimble (August 1991 JRSM, p 471) raise important though largely unacknowledged problems. The first is that psychiatric diagnosis by exclusion is logically indefensible'. That so many doctors do it doesn't make it right but merely demonstrates how uncritically they accept a practice which expediently places the blame for diagnostic failure upon the
victim2. Second, the terms used in ascribing the putative mental illness supposedly responsible for the patient's symptoms - 'hysteria', 'neurotic', and the like - are not merely diagnostic dustbins for disposing of patients with normal test results; they have specific technical meanings. People to whom they validly apply must display certain positive diagnostic features; to use them when these features are lacking or when their presence has not been ascertained is to use them figuratively rather than in their technical sense. The figurative use oftechnical terms, or metaphor, poses no intrinsic problems; they only arise when metaphor is passed off as if it represented technical usage, which is effectively what happens when a patient's speculatively ascribed 'neurosis' is accorded the undeserved status of a properly reached diagnosis. Third, although such pseudodiagnoses cannot long survive critical scrutiny3, they possess considerable face validity. As the authors note, 'however arrived at, the neurologist's opinions in these cases [are] likely to be of lasting influence'. It is worrying that as many as 36 000 British neurological patients per year may be relegated to the disadvantaged status of ascribed mental illness because of systematic abuse of the doctor's diagnostic authority. Finally, there is the problem that psychogenic dismissal4 may cause lasting psychological damage to patients traumatized by the experience. This dismissal injury appears to be an iatrogenic variant of post-traumatic stress disorder6. If doctors admitted inability to diagnose a patient, these problems wouldn't arise. V RIPPERE
61 Queen Alexandra Mansions, Hastings Street, London
References 1 Rippere V. Anatomy of a nonsense, or the common medical practice of dismissing the patient.as neurotic., Newsletter of the Society for Environmental Therapy 1989,9:160-7 2 Rippere V. Blaming the patient: the Flat Earth Society guide to successful medical practice. Newsletter of the Society for Environmental Therapy, 1990;10:1f61-9'3 Slater E. The diagnosis of hysteria. BMJ 1965;i:1395-9
59
Journal of the Royal Society of Medicine Volume 85 January 1992
Letters to the Editor Preference is given to letters commenting on contributions recently published in the JRSM. They should not-exceed 300 words and should be typed double-spaced.
Alfred the Great: a diagnosis I read the article on Alfred the Great by Craig with interest (May 1991 -JRSM, p 303). The argument for Crohn's disease is very ingenious. However, haemorrhoidal disease and complications are much more common. Bouts ofthrombosis and prolapse, with a probably psychologic overlay, occur frequently enough to account for the intermittency of Alfred's symptoms, rather than Crohn's disease. His many pursuits (wars, Viking attacks, governing, etc) would tend to rule out an affliction with a chronic debilitating disease. According to Asser, Alfred contracted the malady in the 'first flowering of youth'. On one occasion, the attack had been cured after Alfred lay prostrate in silent prayer for a long while. This suggests a simple reduction of haemorrhoids as Asser goes on 'to substitute for the pangs of the present and agonizing infirmity some less severe illness, on the understanding that the new illness would not be outwardly visible on his body'. His attack of pain during the Great Feast after he married Ealhswith may well have been due to prolapse or thrombosis precipitated by his nuptial exertions. Certainly, even a king would have been embarrassed at such a time to have displayed the cause of his pain! The term used by Asser, 'ficus' (a fig') is generally translated as 'piles', 'haemorrhoids', or St Fiacre's disease2. There appears to be at least two versions of who St Fiacre really was3'4. In one version, that he was an ordained Irish priest from Co. Kilkenny; in the other version, the first-born son of the King of Scotland, Eugene IV. He settled at the fringe of a forest not far from the village of Breuil-en Brie in a place which is called today 'Saint Fiacre' (Seine et Marne). Many miracles were attributed to St Fiacre. He was once accused of witchcraft and waited for several days outside the church sitting on a big stone waiting for an inquest. Apparently the stone softened while he was sitting on it and an imprint resulted. It was granted the mysterious power of curing haemorrhoids if the patient sat on it and kept faith. The French cab 'fiacre' derived its name from him. The Hotel de St Fiacre in Paris in the middle of the 17th century had these coaches for hire. As the sign of the Inn was an image of the saint, the coaches became called by that name. It was said that Henry V of England died of St Fiacre's disease, at Vincennes. Another royal sufferer was King Louis XIII. F I JACKSON Cross Cancer Institute, 11560 University Ave, Edmonton, AB, Canada T6G 1Z2
References 1 Rains AJH, Capper WM. Bailey & Love's short practice of surgery, 14th edn. London: HK Lewis & Co, 1968:989-96
2 Schwartz Si. Principles of surgery. New York: The Blakiston Division McGraw-Hill Book Company, 1969: 998 3 Racouchot JE, Petouraud CH, Rivoire JO. Saint Fiacre. The Healer of Haemorrhoids and Patron Saint of Proctology. Am J Proctol 1971;22:175-7 4 Thurston H, Attwater D, eds. Alban Butler's Lives of the Saints, complete edition. Westminster, Maryland: Christian Classics, 1981:400-61
Acute myeloid leukaemia complicating sarcoidosLs We read with interest the case report by Murphy et al. in which they report a case of acute myeloid leukaemia (AML) complicating sarcoidosis (June 1991 JRSM, p 368). This case is of interest because of the relationship between sarcoidosis and leukaemia. There have been reports of lung cancer and both Hodgkin's and nonHodgkin's lymphomas occurring in association with sarcoidosis'. Several lines of evidence indicate that this association is not fortuitous. In an epidemiological study of 2544 sarcoidosis patients the number of cases of malignant lymphoma and lung cancer was significantly greater than expected. In this group of sarcoidosis patients, lymphoma occurs 5.5 times more than expected2. The development of AML in sarcoidosis history is extremely rare. Five cases have been previously reported3-7. In two of;them, the sarcoidosis diagnosis must be discussed because the two diseases were diagnosed- at the same time, since granulomata may occur as a reaction of the tumour4. However, in three other cases, sarcoidosis preceded the AML by 3-7 years and Murphy's patient about 10 years5-7. It is difficult to propose one general hypothesis which explains why sarcoidosis may predispose to malignant disease. In -the sarcoidosis-lymphoma syndrome, the increased mitotic activity of the Blymphoid cells in the tissues involved with sarcoidosis lead to; an increased risk of subsequent malignanttransformation, as described in sicca syndrome and rheumatoid arthritis8. We do not know if sarcoidosis may predispose to AML, but it may be suggested that the depletion of circulating T-helper cells seen in sarcoidosis may lead to decreased tumour rejection or perhaps to decreased resistance against putative oncogenic viruses9. Reich suggested that sarcoid-like reactions and sarcoidosis may represent an immunological response to tumour antigen7. It is known that granulomatous inflammation occurs as a response to a large variety of insoluble antigens. Controversially, how can the 3-7 years' interval between sarcoidosis and AML diagnosis be explained? It seems to be interesting to report all cases of sarcoidosis in which AML occurred. A large series might provide an answer about sarcoidosis-AML relationship: causality or coincidence? F MALOISEL Service d'Onco-H6matologie, F OBERLING Hopital de Hautepierre, 67098
Strasbourg cedex, France
References 1 Brincker H. The sarcoidosis-lymphoma syndrome. Br J Cancer 1986;54:467-73 2 Brincker H. Coexistence of sarcoidosis and malignant disease: causality or coincidence? Sarcoidosis 1989; 6:31-43 3 Brandt L, Emanuelsson H, Mitelman F, Stenstam M, Soderstrom N. Pronounced deficiency in T-cells and lymphocyte chromosomal aberrations in a patient with sarcoidosis, myelofibrosis and acute leukemia following thorotrast angiography. Acta Med Scand 1977;201:487-9
Journal of the Royal Society of Medicine Volume 85 January 1992
4 Bordelon J, Stone MJ, Frenkel EP. Probable myeloblastic leukemoid reaction with disseminated sarcoidosis. South Med J 1977;70:1378-80 5 Hermann C, Andersen E, Videbaeck AA. Acute myeloblastic leukaemia in sarcoidosis treated with methotrexate. Scand J Haematol 1980;24:234-6 6 Hatake K, Aoyagi N, Takaku F. Acute myelomonocytic leukemia following remission of sarcoidosis. Oncology 1983;40:200-1 7 Reich JM. Acute myeloblastic leukemia and sarcoidosis. Implications for pathogenesis. Cancer 1985;55:366-9 8 Bataille R, Klein B, Luxembourg A, Durie B. Relation entre etats auto-immuns et canc6risation de cellules de la lign6e lymphocytaire B. In: Roux H, Luxembourg A, Roudier J, eds. Immunorhumatologie SOLAL 1990:170-89 9 Hanto DW, Frizzera G, Gajl-Peczalska KJ, Simmons RL. Epstein Barr virus, immunodeficiency and B-cell proliferation. Transplantation 1985;39:461-72
Intestinal tuberculosis We were interested to read the paper by Lingenfelser et al. (July 1991 JRSM, p436) entitled 'Intestinal tuberculosis: an ongoing diagnostic dilemma' but think that some points merit further discussion. The authors state that the rigid criteria for the diagnosis of intestinal tuberculosis have been extended to include an unequivocal response to anti-TB treatment in patients who have 'suggestive' clinical, radiological or operative evidence of intestinal disease and histologically proven TB elsewhere. They also wonder if a therapeutic trial of anti-TB therapy is warranted in patients with suspected intestinal disease alone. In their reported case the diagnosis of pulmonary tuberculosis was eventually made by mediastinal lymph-node biopsy. The idea that intestinal tuberculosis is usually associated with active pulmonary disease is no longer true and the absence of pulmonary involvement should not influence the diagnosis since it has become apparent that greater numbers of patients are presenting with isolated abdominal disease'. In Leicester we have just reviewed 24 cases of intestinal tuberculosis which presented to the General Surgical Department, (unpublished data). In our patient population only two patients (8%) had radiographic evidence.of pulmonary disease and this concurs with the frequency reported by others2. Although as the authors state the diagnosis is ideally made by seeing organisms on microscopy or culture, it is fully justified to instigate a closely monitored trial of chemotherapy in patients in whom the diagnosis is strongly suspected3 and in our series six patients (25%) did not have a histologically proven diagnosis but all made a dramatic improvement on treatment. The authors also state that 'laparotomy remains the definitive investigation in many patients with intestinal tuberculosis'. This statement needs qualification because although laparotomy may eventually be required in many patients other less invasive methods of obtaining tissue for microbiological examination may be employed including laparoscopy and biopsy4 and biopsy of the peritoneum under local anaesthesia5. Consideration should be given to these techniques if appropriate since there is evidence that laparotomy itself may be associated with significant morbidity in these patients6. M J UNDERWOOD M M THOMPSON R D SAYERS
A W HALL
Department of General Surgery, Glenfield General Hospital, Leicester
References 1 National survey of tuberculosis notifications in England and Wales 1978-9. BMJ 1980;281.895-8 2 Kolawole TM, Lewis EA. A radiological study of tuberculosis of the abdomen (gastrointestinal tract). AJR 1975;123:348-58 3 Lambrianides AL, Ackroyd N, Shorey BA. Abdominal tuberculosis. Br J Surg 1980;67:887-9 4 Wolfe JHN, Behn AR, Jackson BT. Tuberculous peritonitis and the role of diagnostic laparoscopy. Lancet 1979,i:852-3 5 Das P, Shulka HS. Clinical diagnosis of abdominal tuberculosis. Br J Surg 1976;63:941-6 6 Bhansali SK. Abdominal tuberculosis: experience with 300 cases. Am J Gastroenterol 1977;67:324-37
'Hysteria', 'functional' or 'psychogenic'? Drs Mace and Trimble (August 1991 JRSM, p 471) raise important though largely unacknowledged problems. The first is that psychiatric diagnosis by exclusion is logically indefensible'. That so many doctors do it doesn't make it right but merely demonstrates how uncritically they accept a practice which expediently places the blame for diagnostic failure upon the
victim2. Second, the terms used in ascribing the putative mental illness supposedly responsible for the patient's symptoms - 'hysteria', 'neurotic', and the like - are not merely diagnostic dustbins for disposing of patients with normal test results; they have specific technical meanings. People to whom they validly apply must display certain positive diagnostic features; to use them when these features are lacking or when their presence has not been ascertained is to use them figuratively rather than in their technical sense. The figurative use oftechnical terms, or metaphor, poses no intrinsic problems; they only arise when metaphor is passed off as if it represented technical usage, which is effectively what happens when a patient's speculatively ascribed 'neurosis' is accorded the undeserved status of a properly reached diagnosis. Third, although such pseudodiagnoses cannot long survive critical scrutiny3, they possess considerable face validity. As the authors note, 'however arrived at, the neurologist's opinions in these cases [are] likely to be of lasting influence'. It is worrying that as many as 36 000 British neurological patients per year may be relegated to the disadvantaged status of ascribed mental illness because of systematic abuse of the doctor's diagnostic authority. Finally, there is the problem that psychogenic dismissal4 may cause lasting psychological damage to patients traumatized by the experience. This dismissal injury appears to be an iatrogenic variant of post-traumatic stress disorder6. If doctors admitted inability to diagnose a patient, these problems wouldn't arise. V RIPPERE
61 Queen Alexandra Mansions, Hastings Street, London
References 1 Rippere V. Anatomy of a nonsense, or the common medical practice of dismissing the patient.as neurotic., Newsletter of the Society for Environmental Therapy 1989,9:160-7 2 Rippere V. Blaming the patient: the Flat Earth Society guide to successful medical practice. Newsletter of the Society for Environmental Therapy, 1990;10:1f61-9'3 Slater E. The diagnosis of hysteria. BMJ 1965;i:1395-9
59
