Epistaxis from sickle cell disease must not be forgotten
Omar Mulla and colleagues [1] omit mention of sickle cell disease as an important cause of nose bleeding (March 24, p 65). The British Medical Journal, some 46 years ago, published my article entitled “Torrential epistaxis with symmetrical facial-skin ulceration in sickle cell anaemia” [2] in which I said “Epistaxis is common (Hughes et al, 1940; Wintrobe, 1951) even to the extent of one-third of all patients with a sickle cell anaemia and a sickle-cell/haemoglobin-C disease (Hook and Cooper, 1958), and can be very severe (Mabayoje, 1956; Hook and Cooper, 1958)”.[3-6]
THE GREAT MASQUERADER: LESSON FOR CLINICIANS
Drawing attention to Winsor and Burch (1945) who called sickle cell anaemia “a great masquerader” [7] I ventured to make this prediction about the United Kingdom: “The variability of the presenting symptomatology of sickle cell disease needs to be emphasized, especially in a country like Britain, where the incidence of the condition is likely to increase, not decrease” [2] No clinical discipline can keep out sickle cell disease. This 16 year old school girl I described in 1965 was found on admission to Korle Bu Hospital in Accra to have Salmonella typhi osteomyelitis, leg ulcers, generalized lymphadenopathy, and strongyloides infestation [2]. Any African patient who walks into an ENT Department with nose bleeding and a tinge of jaundice has sickle cell disease unless proven otherwise. And if ulcers are found on the medial aspect of the lower leg the phenotype is “SS” ie homozygous sickle cell disease (Sickle Cell Anaemia), again unless proven otherwise.
BRITISH MEDICAL JOURNAL ARCHIVE TO BE CONGRATULATED
To click on a button on BMJ Archive and up pops my article of more than 46 years ago, complete with the facial photograph of this Ghanaian schoolgirl pleases me enormously because that makes the BMJ a far better in-depth Resource material than, say, Wikipedia which doctors and medical students flock to for information. Furthermore, those who rely on ‘MEDSEARCH’ with a cut off point of “30 years back” or “40 years back” are also bound to miss vital clinical information. To find out more about “Genotype epistaxis frequency” (ie ‘SS’ vs ‘SC’ vs ‘SF-Hereditary Persistence’ vs ‘Sbeta-Thalassaemia’), “Sex epistaxis frequency”, and “Age epistaxis incidence” one has to turn to chapter 17 of my “The Sickle Cell Disease Patient” [8]. Suffice it to say that nose bleeding was “twice as common in sickle cell anaemia ‘SS’ (10.2 % of 597 patients) than in Hb ‘SC’ disease (5.3 % of 603 patients)”, and slightly more males than females had epistaxis (8.8 % vs 6.9 %), while in the age distribution “almost unheard of under 5 years of age, epistaxis was noted most often between 10 and 20 years, 41.5 % of all epistaxis cases being in this age range, and the tendency is noted to continue into adulthood. The age group stratification of epistaxis is statistically significant (P < 0.001)”. Incidentally, I noted that the 5.3 % epistaxis incidence in Jamaica of Graham Serjeant’s 300 adult sickle cell anaemia (Hb "SS") patients [9] was “exactly the same as that of the Ghanaian Hb “SC” patients (5.3 % of 602 patients). In my Clinic 4.7 % of 24 patients with Sickle Cell Hereditary Persistence of Fetal Haemoglobin Disease (Hb “SF”) bled from the nose as opposed to 2.8 % of 31 Sickle Cell beta-Thalassaemia Disease patients [8].
SEVERITY OF EPISTAXIS
Mulla and colleagues are right to warn: “do not underestimate the amount of blood that can be lost during epistaxis” [1]. In my patient “an estimate of total blood loss was between 300 and 500 ml, but the haematocrit reading remained at 22%” [2]. And yet “she left hospital 4 months after admission without having had a blood transfusion or any other crises” [2]. See the text [2] for how she was managed without blood transfusion, and how liver and spleen that were huge on admission returned to near-normal on discharge, and how generalised lymphadenopathy abated.
Felix I D Konotey-Ahulu MD(Lond) FRCP(Lond) FRCP(Glasg) DTMH(L’pool)
Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana and Consultant Physician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies, 9 Harley Street Ltd, Phoenix Hospital Group, London W1G 9AL.
2 Konotey-Ahulu FID. Torrential epistaxis with symmetrical facial-skin ulceration in sickle cell anaemia http://www.bmj.com/content/2/5466/859 BMJ Oct 9 1965; 2: 859-860.
3 Hughes JG, Diggs LW, Gillespie CE.The involvement of the nervous system in sickle cell anaemia. Journal of Pediatrics 1940; 17: 166-184.
4 Wintrobe MM. Clinical Hematology, 3rd Edition, Les and Febiger 1951. Philadelphia.
5 Hook EW, Cooper GR. The clinical manifestation of sickle cell haemoglobin C disease and sickle cell anemia. South. Med. Journal (Bgham, Ala) 1958; 51: 610-626.
6 Mabayoje JO. Sickle cell anaemia: a major disease in West Africa. BMJ 1956; 1: 194-196.
7 Winsor T, Burch GE. Sickle cell anaemia: “A great masquerader”. Journal of American Medical Association 1945; 129: 792-976.
8 Konotey-Ahulu FID. The Sickle Cell Disease Patient – Natural History from a clinico-epidemiological study of the first 1550 patients of Korle Bu Hospital Sickle Cell Clinic. The Macmillan Press Ltd London 1991, 1992 and T-A’D Co Watford, England 1996 [Available from tadco@sicklecell.md or Chaplin Multimedia Ltd, P O Box 165, Watford, Herts WD17 3ZH andrew.lalchan@chaplin-multimedia.com ]
9 Serjeant GR. The clinical features in adults with sickle cell anaemia in Jamaica. West Indian Medical Journal 1970; 19: 1-8.
Competing interests:
No competing interests
28 March 2012
FELIX ID KONOTEY-AHULU
Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast, Ghana
Consultant Physician Genetic Counsellor Sickle Cell & Other Haemoglobinopathies
9 Harley Street Ltd, Phoenix Hospital Group, London W1G 9AL England
Rapid Response:
Re: Epistaxis
Epistaxis from sickle cell disease must not be forgotten
Omar Mulla and colleagues [1] omit mention of sickle cell disease as an important cause of nose bleeding (March 24, p 65). The British Medical Journal, some 46 years ago, published my article entitled “Torrential epistaxis with symmetrical facial-skin ulceration in sickle cell anaemia” [2] in which I said “Epistaxis is common (Hughes et al, 1940; Wintrobe, 1951) even to the extent of one-third of all patients with a sickle cell anaemia and a sickle-cell/haemoglobin-C disease (Hook and Cooper, 1958), and can be very severe (Mabayoje, 1956; Hook and Cooper, 1958)”.[3-6]
THE GREAT MASQUERADER: LESSON FOR CLINICIANS
Drawing attention to Winsor and Burch (1945) who called sickle cell anaemia “a great masquerader” [7] I ventured to make this prediction about the United Kingdom: “The variability of the presenting symptomatology of sickle cell disease needs to be emphasized, especially in a country like Britain, where the incidence of the condition is likely to increase, not decrease” [2] No clinical discipline can keep out sickle cell disease. This 16 year old school girl I described in 1965 was found on admission to Korle Bu Hospital in Accra to have Salmonella typhi osteomyelitis, leg ulcers, generalized lymphadenopathy, and strongyloides infestation [2]. Any African patient who walks into an ENT Department with nose bleeding and a tinge of jaundice has sickle cell disease unless proven otherwise. And if ulcers are found on the medial aspect of the lower leg the phenotype is “SS” ie homozygous sickle cell disease (Sickle Cell Anaemia), again unless proven otherwise.
BRITISH MEDICAL JOURNAL ARCHIVE TO BE CONGRATULATED
To click on a button on BMJ Archive and up pops my article of more than 46 years ago, complete with the facial photograph of this Ghanaian schoolgirl pleases me enormously because that makes the BMJ a far better in-depth Resource material than, say, Wikipedia which doctors and medical students flock to for information. Furthermore, those who rely on ‘MEDSEARCH’ with a cut off point of “30 years back” or “40 years back” are also bound to miss vital clinical information. To find out more about “Genotype epistaxis frequency” (ie ‘SS’ vs ‘SC’ vs ‘SF-Hereditary Persistence’ vs ‘Sbeta-Thalassaemia’), “Sex epistaxis frequency”, and “Age epistaxis incidence” one has to turn to chapter 17 of my “The Sickle Cell Disease Patient” [8]. Suffice it to say that nose bleeding was “twice as common in sickle cell anaemia ‘SS’ (10.2 % of 597 patients) than in Hb ‘SC’ disease (5.3 % of 603 patients)”, and slightly more males than females had epistaxis (8.8 % vs 6.9 %), while in the age distribution “almost unheard of under 5 years of age, epistaxis was noted most often between 10 and 20 years, 41.5 % of all epistaxis cases being in this age range, and the tendency is noted to continue into adulthood. The age group stratification of epistaxis is statistically significant (P < 0.001)”. Incidentally, I noted that the 5.3 % epistaxis incidence in Jamaica of Graham Serjeant’s 300 adult sickle cell anaemia (Hb "SS") patients [9] was “exactly the same as that of the Ghanaian Hb “SC” patients (5.3 % of 602 patients). In my Clinic 4.7 % of 24 patients with Sickle Cell Hereditary Persistence of Fetal Haemoglobin Disease (Hb “SF”) bled from the nose as opposed to 2.8 % of 31 Sickle Cell beta-Thalassaemia Disease patients [8].
SEVERITY OF EPISTAXIS
Mulla and colleagues are right to warn: “do not underestimate the amount of blood that can be lost during epistaxis” [1]. In my patient “an estimate of total blood loss was between 300 and 500 ml, but the haematocrit reading remained at 22%” [2]. And yet “she left hospital 4 months after admission without having had a blood transfusion or any other crises” [2]. See the text [2] for how she was managed without blood transfusion, and how liver and spleen that were huge on admission returned to near-normal on discharge, and how generalised lymphadenopathy abated.
Felix I D Konotey-Ahulu MD(Lond) FRCP(Lond) FRCP(Glasg) DTMH(L’pool)
Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana and Consultant Physician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies, 9 Harley Street Ltd, Phoenix Hospital Group, London W1G 9AL.
Email: felix@konotey-ahulu.com Website: www.sicklecell.md
Competing interests: None declared
1 Mulla Omar, Prowse Simon, Sanders Tim, Nix Paul. Epistaxis (Ten-Minute Consultation). BMJ 2012; 344: e1097 [March 24 2012, Volume 344, page 65]
2 Konotey-Ahulu FID. Torrential epistaxis with symmetrical facial-skin ulceration in sickle cell anaemia http://www.bmj.com/content/2/5466/859 BMJ Oct 9 1965; 2: 859-860.
3 Hughes JG, Diggs LW, Gillespie CE.The involvement of the nervous system in sickle cell anaemia. Journal of Pediatrics 1940; 17: 166-184.
4 Wintrobe MM. Clinical Hematology, 3rd Edition, Les and Febiger 1951. Philadelphia.
5 Hook EW, Cooper GR. The clinical manifestation of sickle cell haemoglobin C disease and sickle cell anemia. South. Med. Journal (Bgham, Ala) 1958; 51: 610-626.
6 Mabayoje JO. Sickle cell anaemia: a major disease in West Africa. BMJ 1956; 1: 194-196.
7 Winsor T, Burch GE. Sickle cell anaemia: “A great masquerader”. Journal of American Medical Association 1945; 129: 792-976.
8 Konotey-Ahulu FID. The Sickle Cell Disease Patient – Natural History from a clinico-epidemiological study of the first 1550 patients of Korle Bu Hospital Sickle Cell Clinic. The Macmillan Press Ltd London 1991, 1992 and T-A’D Co Watford, England 1996 [Available from tadco@sicklecell.md or Chaplin Multimedia Ltd, P O Box 165, Watford, Herts WD17 3ZH andrew.lalchan@chaplin-multimedia.com ]
9 Serjeant GR. The clinical features in adults with sickle cell anaemia in Jamaica. West Indian Medical Journal 1970; 19: 1-8.
Competing interests: No competing interests