|Year : 1973 | Volume
| Issue : 2 | Page : 73-77
Orbital deposit in acute myeloid leukaemia
GN Seal, AK Gupta
Department of Ophthalmology, Institute of Post-Graduate Medical Education and Research, and Sukhlal Karnani Hospital, Calcutta, India
G N Seal
Department of Ophthalmology, Institute of Post-Graduate Medical Education and Research, and Sukhlal Karnani Hospital, Calcutta
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Seal G N, Gupta A K. Orbital deposit in acute myeloid leukaemia. Indian J Ophthalmol 1973;21:73-7
Leukaemia is an invariably fatal systemic disease of unknown etiology involving the haemopoietic system and characterized by disorderly proliferation of the leucocytes and their precursors and by the presence of large number of immature leucocytes in the blood stream.
In a fair number of cases, the disease is associated with ocular complications. Thirty-three different ocular complications were listed by GOLDBACH  from among the 242 leukaemias at the Johns Hopkins Hospital. Majority of these were seen in the fundus oculi. Only in 2 per cent of cases of lymphatic leukaemia SORSBY,  orbital infiltration producing bilateral exophthalmos was recorded. Myeloid leukaemia is rarely associated with orbintal infiltration. In India, WRIGHT  recorded a case where there was a hard mass in each orbit beneath the supra-orbital margin in a 4-year-old South Indian child with a typical blood picture of acute myeloid leukaemia. Recently, CHATTERJEE AND SEN  reported a case of acute myeloid leukaemia in a child with leukaemic deposit in the right orbit only. The following is the report of two cases of acute myeloid leukaemia with unilateral orbital deposition of leukaemic cells.
| Case Reports|| |
CASE NO. 1
C. R., a Hindu male child aged 4 years was brought to the ophthalmic department of our hospital on 28th July, 1966, with a history of gradual protrusion of the right eye for the last one and a half months. There were hoarseness of voice and swelling of the neck glands on the right side, of same duration.
About one and a half months ago, the child developed mild fever (100°F), cough and swelling of the lymph glands on the right side of the neck. At that time, the parents also noticed slight swelling of the right upper lid. Fever and cough subsided with treatment in a district hospital, within a week but the lid-swelling went on increasing. The eye ball also started protruding producing widening of the palpebral fissure. Family history did not reveal any significant feature.
The boy was poorly nourished with pallor of the skin and conjuctiva. There was no bleeding from the gums or nose. The temperature was normal. There was enlargement of the neck glands on the right side. The preauricular gland on the affected side was . also palpable. Right axillary nymph nodes were enlarged and firm in consistency. The liver was palpable four fingers' breadth below the costal margin and the spleen just palpable.
Right Eye - There was a swelling under the upper lid extending up to the supra-orbital margin. The eye ball was moderately proptosed with considerable downward displacement. The swelling was firm in consistency and tender to touch. The upper lid was markedly stretched over the swelling and presented prominent veins. The mass appeared to be oval in shape, non-pulsatile, and measured about 2" X 3/4" It extended deep into the orbit. There was chemosis of the bulbar conjunctiva with a patch of subconjunctival haemorrhage near the external canthus. The ocular movements were restricted in all directions particularly in upward direction.
Left .dye - It was apparently normal with full ocular movements.
(a) Urine-and stool ---. N.A.D.
(b) Blood picture -.Hb - 3.5 gm.%.
P.C.V. - 11 ml%
Polymorph - 2%,
Lymphocytes - 2 %
Myeloblasts - 86% ,
Promyelocytes - 10%.
(c) X-rays of the orbits - Bony erosion of the right lesser and greater wings of the sphenoid and of the lateral wall of the right orbit.
(d) X-rays of chest - Nothing abnormal.
(e) Fundoscopy - Fundi oculi in both sides, appeared normal. The retinal vessels did not show any abnormality.
Systemic treatment was immediately started with antimetabolic drug Purinethol (˝ tablet B.D.) and Vitamins. Prednisolone was also started after a week (5 mg. T.D.S.). Since the blood picture did not show any appreciable change and the child was getting pale due to anaemia, a blood transfusion was given on 10-8-66. On 28-9-66, biopsy from the orbital mass was taken under general anaesthesia. The mass was firm in consistency and did not show any green pigmentation. It was relatively avascular. Fundi oculi were again examined and found to be normal.
In the first week of October, 1966 (i.e. 2 months after admission), the patient developed slight proptosis in his left eye. There were dilated veins on the lids and a patch of subconjunctival haemorrhage on the temporal aspect of the globe.
Against medical advice, the child was taken away 10-10-66 with instructions to continue prednisolone and purinethol. Later on, we got the information that the child died after 6 weeks.
CASE No. 2
A Hindu young girl (N.M.) aged I1 years was referred to the eye department, of our hospital from the medical department on 17-6-71 for examination of a gradual swelling in her right eye for the last 3 months. The girl also complained of frequent headache for the same period. About 3 months back, the patient first noticed her right eye slightly prominent than the left. Since then, the eye ball was gradually protruding. It was not associated with any pain or diplopia. A few weeks ago, the girl was taken by her parents to a general practitioner who examined her blood which revealed a high W.B.C. count. So, she was referred to the medical department for further investigation and treatment.
The girl appeared healthy with moderate pallor of the conjunctiva. There was no fever, jaundice or cyanosis. Pulse rate was 70 per minute and regular. There was no enlargement of the lymph glands anywhere in the body. Liver, spleen and thyroid glands were not palpable.
Right Eye - The eyeball was moderately proptosed and slightly pushed downwards and medially. Upward movement of the eyeball was slightly restricted. There was neither congestion nor chemosis of the conjenctiva. A moderate fullness was noticed in the lateral part of the upper lid. On palpation, a firm crescentic mass was felt above and lateral to the eyeball. The mass was lobulated and extended backwards into the orbit.
Fundus examination did not reveal any abnormality. The visual acuity was 6/12.
Left Eye - It was normal with 6/9 visual acuity. Fundus oculus did not show any abnormality.
(a) Urine and stool - Nothing abnormal.
(b) Blood picture -
Hb. - 8.2 gm%. P.C.V. - 25 ml%.
W.B.C. - 31,000/c.mm.
Blast cells - 24%
Promyelocytes - 34%.
Myelocytes - 20%,
Metamyelocytes - 8%.
Neutrophil - 10%,
Lymphocytes - 4%.
(c) X-rays of the orbits and chest: Nothing abnormal.
At the beginning, the patient was treated with Prednisolone tablets (Deltacotril 10 mg. T.D.S.) for two weeks but there was no reduction of the W.B.C. count (increased to 120,000/ cmm.) . So Purinethol (6 mercaptopurine) tablets were started (50 mg T.D.S.) in addition to Prednisolone. The blood picture gradually improved and W.B.C. count came down to 16,800/c.mm in 4 weeks' time. The orbital mass did not get reduced in size. On 2-7-71, biopsy from the mass was taken under local anaesthesia. The mass was found to be pale white in colour and cartilagenous in consistency. There was not much bleeding on section of the mass. Histologically the biopsy tissue showed a collection of leukaemic cells in a fairly dense fibrous stroma.
| Dicussion|| |
Orbital infiltration in myelogenous leukaemia is very rare as compared to lymphatic leukaemia (DUKE-ELDER,  ). In both the cases presented here, the mass in the orbit was proved to be deposition of leukaemic cells by histological examination of the biopsy material. Like CHATTERJEE & SEN'S  case, our cases also presented unilateral deposition, although in Case No. 1 the opposite orbit was beginning to get involved. Another interesting feature in our second case was that the orbital findings was the only clinical evidence of the disease apart from the blood picture. These tumour-like deposits have to be differentiated from a condition called chloroma.
Chioroma, a variant form of acute myeloid leukaemia, is a rare pathological condition in which, there are multiple deposits of greenish tumourlike masses in the skeleton, the lymph glands and the viscera (STURGIS,  ) . It is of particular ophthalmological interest because, in about 50% of cases, there is tumour-like deposition of leucocytes within the periosteum of the orbits producing exophthalmos (SORSBY,  ). The deposited mass is locally invasive and greenish in colour due to presence of choleglobin, possibly derived from the breakdown products of haemoglobin. Ross  suggested that the term chloroma should be reserved for those cases where the tumour masses show green coloration. Since neither of our cases showed any greenish coloration, we cannot class them as chloroma. HAMEED, DAS AND AGARWAL  reported two cases of acute meyloblastic leukaemia with orbital deposits and diagnosed them as chloroma although they did not take the biopsy to note the colour of the neoplasm.
Fundus examination in both our cases did not show any abnormality whatsoever. The second case is still under our observation. Although the blood picture has considerably improved with treatment, the orbital mass did not show any sign of regression.
So it is not understood why, with the same pathological condition of the haemopoietic system, the deposited mass is white in colour in same cases while it is coloured with greenish pigments in others. Again, in the latter cases, the incidence is much higher (about 50 per cent) as compared to the former ones.
| Summary|| |
Two cases of acute myeloid leukaemia with unilateral orbital deposit are reported. The differentiating features from chloroma of the orbit are discussed.
| Acknowledgement|| |
Our thanks are due to Dr. N. Pal, Deptt. of Pathology & Bacteriology for the histological reports and microphotographs and to Dr. S. Sen, Deptt. of Medicine for allowing us to publish the second case which was under his treatment.
| References|| |
Chatterjee, B. M. and Sen, N. N.: Brit. J. Ophthal., 44: 440 (1960).
Duke-Elder, S.: "Textbook -- of Ophthalmology", Vol. 5, p. 5552. Kimpton, London, (1952).
Goldbach, L. J.: "Leukaemic Retinitis", Arch. Ophthal. Chicago, 10: 808 (1933).
Hameed, S., Das, T., and Agarwal, K. C.: Brit. J. Ophthal., 43: 107 (1959).
Ross, R. R.: Amer. J. Med., 18: 671 (1955).
Sorsby, A.: "Systemic Ophthalmology", p. 574 Butterworth, London. (1951).
Sturgis, C. C.: "Hematology", 2nd ed., p. 862. Thomas, Springfield, Ill (1955).
Wright: "Extra-ocular Affections". Madras. (1938). (Quoted by Duke Elder, in 2).
[Figure - 1], [Figure - 2]