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Year : 1970  |  Volume : 18  |  Issue : 3  |  Page : 111-117

Study of red blood cell morphology and abnormal haemoglobin in the causation of Eales' disease and other vitreous haemorrhages

1 Department of Ophthalmology, Postgraduate Institute of Medical Education, & Research, Chandigarh, India
2 Department of Haemotology, Postgraduate Institute of Medical Education, & Research, Chandigarh, India

Correspondence Address:
I S Jain
Department of Ophthalmology, Postgraduate Institute of Medical Education, & Research, Chandigarh
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Source of Support: None, Conflict of Interest: None

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How to cite this article:
Jain I S, Kanwar G S, Das K C. Study of red blood cell morphology and abnormal haemoglobin in the causation of Eales' disease and other vitreous haemorrhages. Indian J Ophthalmol 1970;18:111-7

How to cite this URL:
Jain I S, Kanwar G S, Das K C. Study of red blood cell morphology and abnormal haemoglobin in the causation of Eales' disease and other vitreous haemorrhages. Indian J Ophthalmol [serial online] 1970 [cited 2021 Sep 28];18:111-7. Available from: https://www.ijo.in/text.asp?1970/18/3/111/35074

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Table 1

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Table 1

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Since the time of Henry Eales[8], who first described the condition in 1880 the etiology of so called Eales' disease still remains an enigma. From time to time various theories have been put forward for its causation: Tuberculosis and tuberculo-aller­gic processes - Axenfeld and Stock [5] . Thromboangitis obliterans - Marche­sani [16] , Focal sepsis - Applemann [1] , Werner [22], Puttana [20] , Endocrinal dys­function - Jeandelize [14] , Mawas and Herschberg [17] and Diseases of the hae­mopoetic system - Donner [7] , Ma­was [18] .

Changed red cell morphology, Kahn, Kahan and Benko [15] these authors re­ported thorny red blood cells (acan.­thocytes) in the peripheral blood of a case of Eales disease. The chance finding of acanthocytes by one of us (I. S. Jain) in a case of Eales disease, made us feel to undertake systematic study to evaluate the role of acanthocytes vis-a-vis tubercular allergy, which is the most widely accepted etiological factor.

  Material and Methods Top

Thirty three cases of Eales' disease were studied from January, 1967 to November, 1968; in the eye Depart­ment of the Postgraduate Institute of Medical Education and Research, Chandigarh.

Detailed ocular and systemic exa­mination and routine investigations including Mantoux Test were carried out in every case.

Special investigations for abnormal Red Blood Cells in peripheral blood smear, and abnormal haemoglobin, were also done in every case.

For control, thirty five cases of vitreous haemorrhages due to known causes, were studied for any evidence of acanthocytes in their peripheral blood.

Twenty five cases of Mantoux posi­tive not suffering from vitreous hae­morrhages, and likewise, 25 mantoux negative children suffering from vari­ous diseases in paediatric wards of our Hospital were studied for any evidence of acanthocytes.

Mantoux test results were studied in 25 random samples of normal pi­pulation to compare with Mantoux results in Eales disease cases.

Fifty proved cases of tuberculosis (proved clinically, radiologically and having positive sputum for Acid Fast bacilli), from the chest clinic of the Postgraduate Institute of Medical Edu­cation and Research, Chandigarh were studied for any evidence of acantho­cytes in their peripheral blood.

One hundred normal healthy per­sons, taken from the staff of the eye department and persons reporting for refractive errors, were also screened for any evidence of acanthocytes in their peripheral blood.

Total fat and lipoprotein studies were done in 14 cases, to demonstrate any association with positive and ne­gative acanthocyte cases. Demonstra­tion of Acanthocytes: was done by two methods:

(1) Examination of "Dry blood film" stained by Leishman's Stain.

(2) "Wet blood film study".

One drop of blood, diluted with four drops of saline, covered with a coverslip was examined under the microscope.

5% or greater, incidence of acan­thocytes in the blood film was con­sidered as positive case for acantho­cytosis.

Estimation of foetal Haemoglobin was done in every case by the me­thod of Singer and Chernoff [21] .

  Observations Top

Age and sex incidence

There were 31 males and only two females. No patient was under 10 years of age; two were between 11-20 years and 19 were between 21-30 years while 12 were between 31-40.

Focal sepsis

There was no , evidence of focal sep­sis in thirty patients, while only three patients had evidence of dental caries.

Intestinal Parasites

One case showed giardiasis, another showed cysts of lodomoeba Butschli and the third ova of H. Nana. Rest of the thirty cases showed no intesti­nal parasites.

Systemic examinations.

Only three cases showed evidence of old or active luug involvement; in the rest of the thirty cases respiratory system was normal. No evidence of liver enlargement was found in any of the: 33 cases of Eales disease.

[Table - 1]

This table shows no appreciable difference between the cases of Eale's and that of normal persons.

Haemotological Investigations

Abnormal haemoglobin: None was detected in any of the 33 cases of Eales' disease.[Table - 2],[Table - 3],[Table - 4]

Blood lipid studies

Total fat, cholesterol, phaspholipid, total serum proteins, albumin, alpha [1] , alpha, and Beta and gamma globins, lipoproteins - alpha and beta and non-esterified fatty acid - estimations were within normal limits in cases of Eales' disease with acanthocytes and Mantoux positive and other cases of Eales with acanthocyte negative.

Thus no change was noted in lipid studies in acanthocyte positive cases of Eales disease.

  Discussion Top

Most of the authors have blamed tuberculosis as the cause; Axenfeld [5] , found 8 cases of periphlebitis retinae in 284 cases of pulmonary tuberculosis while Elliot [9],[10] found active or heal­ed pulmonary tuberculosis in 35% of his 31 cases of Eales and from India Awasthe, Mehrotra and Shrivastava [4] , reported an incidence 1.3% of Eales, out of 1180 untreated pulmonary tu­berculosis. In the present series, out of a study of 50 cases of proved tuber­culosis none was detected to have Eales disease. Evidence of tuberculo -allergic process has been noted by many authors Appleman [1] , Elliot, [9],[10] Gupta [11] , Ashton [3] , Chanda [6] . The in­cidence of Mantoux positive in the present series of Eales cases is also very high 88% (29/33) cases.

Hagino [13] has recently put forward a positive experimental evidence for tubercular allergy by B. C. G. vacci­nation sensitisation in rabbits. Pahwa [19] also reported seeing one case of vitreous haemorrhage following B. C. G. vaccination.

No significant role of other etiolo­gical factors like, focal sepsis and intestinal parasites could be: establish­ed in the present series.

Role of Acanthocytes

Acanthocytes are small irregularly shaped red blood cells with spur like processes of variable length at irre­gular intervals. [Figure - 1],[Figure - 2],[Figure - 3]. So far, acanthocytosis has been recogni­sed as a congenital abnormality of the R. B. C.'s commonly seen in cases of atypical retinitis pigmentosa, having steatorrhoca with absent or markedly reduced plasma beta lipproteins. Acanthocytosis has also been found in association with hereditary vitreo­retinal degeneration. This abnorm­ality of the red cells is possibly due to a mutant recessive gene, which when inherited from both parents, produces the homozygous state of acanthocy­tosis.

Acquired acanthocytosis has recent­ly been observed in a case of Eales disease by Kahn et al. [15] They lacked confirmation of their observation. From our systematic study to evaluate the role of such abnormalities of the R. B. C. in cases of Eales disease, the following observations and infer­ences are discussed:­

We have taken acanthocytes posi­tive only when the percentage of acanthocytes was 5% or more.

In the present series, we have 9 positive cases (5-50% Acanthocytes) at the time of our examination.

During a random sampling of the general population attending the eye outpatient department we found only 3 positive cases out of 100 normal cases. These cases were not suffering from any intraocular disease. Most of the Eale's disease cases in our series were also Mantoux positive (29/33).

Since tubercular allergy is by far the commonest etiological agent, res­ponsible, for the so called Eales cases, it was thought pertinent to look for this erythrocytic abnormality in cases having frank pulmonary tuberculosis but no ocular involvement. In 50 proved pulmonary tubercular cases 5 patients were found having acantho­cytes. While in 25 cases having only evidence of tubercular allergy name­ly Mantoux positive cases) we found 5 patients (20%) having acanthocytes in their peripheral blood.

Incidentally it is appropriate to emphasise that the first case of acquir­ed acanthocytosis reported by Kahn et al [15] was also found to be in a case having tubercular background.

Evidence of acanthocytes in 25 Mantoux negative cases; was found in only two persons (8%). From this it appears that there is obviously an in­creased incidence of acanthocyte posi­tive cases in individuals who were having tubercular allergy. Out of three cases having focal sepsis, one case was positive for acanthocytes, but this case was also Mantoux positive.

Kahn et al [15] produced transient acanthocytosis within two hours after injecting pyrexal intravenously: (Py­rexal - Wander, is 0.3 g. purified endotoxin prepared from salmonella abortus equi) which disappeared again after two hours. The pyrexal acted as an artificial toxin analogus to the endotoxin liberated by bacterial infec­tion in the body. This experimental evidence by Kahn et al [15] may also explain that increased incidence of acanthocyte positive cases (9/33 - 27.3%) in the present series, could be induced by the tubercular protein toxins in these cases. This pheno­menon was certainly not of lasting duration, as it showed fluctuations on follow up examination and a few cases even became negative later.

It is quite probable that many other cases would also have been acantho­cyte positive, if they had come for examination at that critical stage when this abnormality of the R. B. C. would have been maximum and had preci­pitated the visual catastrophe. It is also possible that various forms of treatment previously tried by these patients before presenting themselves to us e.g. corticosteroids, antituber­cular therapy etc. might have helped in reversing this peculiar abnormality of the R. B. C.

Meehanism of production of vitreous haemorrhage by Acanthocytes

1. These abnormal R. B. C. show increased mechanical fragility.

2. The toxins cause changes in the vessels and hyaloid membrances of vitreous body.

3. The mobility of these abnormal cells is limited to rotating with each other like cogwheels and thus can easily block the vessels because of mechanical hindrance.

This study therefore suggests that both clinical picture of Eales dis­ease and the phenomenon of acquired acanthocytcsis may be due to endo­toxin of tubercular protein or other endotoxins.

  Summary and Conclusions Top

The present study was undertaken to evaluate the role of red cell mor­phology (Acanthocytosis) in the pa­thogenesis of Eales disease.

Abnormal haemoglobin was not found to play any role in its causa­tion. Acquired acanthocytosis was observed in 27.3% of cases of Eales disease, which was fluctuating in du­ration and it is suggested that this phenomenon may be induced by endogenous toxins principally tuber­cular proteins.

Intestinal worm infestation and focal sepsis were not considered to play a significant role in the causation of Eales disease.

  References Top

Applemans, M.: Bull. Soc. Franc. Ophthal. 572-574 (1947).  Back to cited text no. 1
Idem: Treatment of recurrent retinal haemorrhages in young subjects by Neo-Antergan (in French). Arch. Ophthal. (Paris) 8, 398 (1948).  Back to cited text no. 2
Ashton, N.: Pathogenesis and etiology of Eales' disease: Acta XIX concilium Ophthalmologicum Vol. II, 828-838 (1962).  Back to cited text no. 3
Awasthi, P.. Mehrotra. M. L. and Srivastava, S. N.: Ocular conditions amongst pulmonary tuberculosis pa­tients in India : Proceedings of the XX International Congress of Oph. 1025, (1966).  Back to cited text no. 4
Axenfeld, Th. and Stock, W.: On residual vitrcoush aemorrhaje and reti­nitis proliferans and tubercular diathesis (In German) Cong. Intern. D'Opht. XI, 367-368 (1909).  Back to cited text no. 5
Chanda, N. N.: Eales' disease: Gene­ral Observations Acta XX Concilium Ophthalmologica Vol: 11, 880-884 (1962).  Back to cited text no. 6
Donner, K. F. A.: Klin. Mbl. Augen­heilk, 123, 112 (1953).  Back to cited text no. 7
Eales. H.: Cases of Retinal Haemorr­hage associated with epistaxis and constipation. Birmingham Med. Res. 9, 262-273 (1880).  Back to cited text no. 8
Elliot, A. J.: Recurrent intraocular haemorrhage in young adults (Eales disease) J. Am. Oph. Soc. 521, 811 (1954).  Back to cited text no. 9
Idem: Recurrent intraocular haemorr­hage in young adults (Eales Disease) A. M. A. Arch. Oph. (Chicago) 61, 745 (1959).  Back to cited text no. 10
Gupta, S. P.: Clinical study of non­traumatic intraocular haemorrhage with particular reference to Eales' disease - Proceedings of all India Oph. Society Vol: XV 158 (1955).  Back to cited text no. 11
Idem: Eales disease its etiology and prognosis Arch. XIX conch. Ophthal­mologicum Vol. 11 868-871 (1962).  Back to cited text no. 12
Hagino, R.: Experimental aspects of Eales disease Acta XIX cong. Ophthal­mology Vol. 2, 841-853 (1962).  Back to cited text no. 13
Jeanedelize, P. & Drouet, P. L.: Endo­crine troubles, particularly of the hy­pophysis in recurrent vitreous haemorr­hages (in French). Bull. Soc. Oph­thal. Paris (260-266), (1936).  Back to cited text no. 14
Kahn, A., Kahan, I. L. and Benko, A.: Acquired acanthocytosis and my­elophthosis in, a case of Eales disease: Brit. J. Ophth. 47, 632 (1963).  Back to cited text no. 15
Marchesani, O.: Min Wschr. 13, 993 (1934).  Back to cited text no. 16
Mawas, J. and Herschberg, A. D.: Treatment of recurrent retinel haemor­rhages in the young by testosterone implants (in French). Bull. Soc. Franc. Ophthal. 66, 388-391 (1953).  Back to cited text no. 17
Mawas, J.: Ophthalmologica: (Basel) 109, 274 (1945).  Back to cited text no. 18
Pahwa. J. M.: Ealcs Disease and Photocoagulation. Trans. of 25th All India Oph. Conference Vol. XXII- 157, (1965).  Back to cited text no. 19
Puttana, S. T.: Retinal vasculitis and focal sepsis. Proceedings of All In­dia Oph. Society Vol: XXI. P. 108 (1964).  Back to cited text no. 20
Singer, K., Chernoff, A. 1. and Singer. L.: Studies on abnormal haemoglo­bin. Blood 6, 413 (1951).  Back to cited text no. 21
Werner, L. E.: Trans. Ophthal. Soc. U.K., 66, 676 (1946).  Back to cited text no. 22


  [Figure - 1], [Figure - 2], [Figure - 3]

  [Table - 1], [Table - 2], [Table - 3], [Table - 4]


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